dermatopathology q - a

1-Leukocytoclastic vasculitis can be seen in which condition?

A. Rheumatoid neutrophilic dermatosis

B. Erythema elevatum diutinum

C. Urticaria

D. Granuloma inguinale

E. Sweet's Syndrome

Rheumatoid neutrophilic dermatitis is seen in the setting of severe rheumatoid arthritis. The pathogenesis is not understood, but in the few reports of this rare dermatosis, a true vasculitis has not been seen. Sweet's syndrome also does not show evidence of a true vasculitis. Erythema elevatum diutinum histologically shows a leukocytoclastic vaculitis, but with prominent interstitial neutrophils. Other conditions with evidence of LCV include Henoch-Schonlein purpura, granuloma faciale, urticarial vasculitis, and occasionally serum sickness. (B)

2-Mantle cell lymphoma is characteristically positive for which of the following?

A. CD10

B. CD23

C. CD138

D. bcl-1

E. bcl-6

Bcl-1 (Cyclin D1) is a marker for mantle cell lymphoma. CD10, bcl-6, and bcl-2 are markers for follicular cell lymphoma. Bcl-2 also stains normal T cells. CD23 is a marker for CLL/SLL and is negative in mantle cell lymphoma. CD138 is a marker for plasma cells. (D)

3-Clinically, this lesion often has a blue hue and usually occurs on the face:

A. Apocrine hidrocystoma

B. Epidermoid inclusion cyst

C. Bronchogenic cyst

D. Eccrine hidradenoma

E. Myxoid cyst

apocrine hidrocystoma are usually solitary, tranlucent nodules which may have a bluish hue due to the Tyndall effect. Histologically, they have one or several large cystic spaces with decapitation secretion. (A)

4-This keratin is associated with hair and nail:

A. Keratin 6

B. Keratin 8

C. Keratin 10

D. Keratin 16

E. Keratin 17

Keratins 6 and 16 are found in the nail. Keratin 17 is seen in the nail as well as in the hair follicle, and patients with pachyonychia congenital type 2, in which there is a mutation of keratins 6b and 17, have nail findings as well as steatocysts. (E)

5-Caterpillar bodies are seen in:

A. Lipoid proteinosis

B. Amyloidosis

C. Porphyria cutanea tarda

D. Mucocele

E. Dyskeratosis congenital

Caterpillar bodies are thought to be type IV collagen. (C)

6-The promontory sign is seen in:

A. Tufted angioma

B. Glomeruloid hemangioma

C. Spindle cell hemangioendothelioma

D. Acroangiodermatitis of Mali

E. Kaposi?s sarcoma

The promontory sign refers to the formation of new vessels around existing vessels and adnexal structures. This is seen in Kaposi?s. (E)

7-You examine a biopsy that is square on low power. The clinical history reports an IgG paraproteinemia and the presence of a "doughnut sign". Which of the following conditions would you think of without looking on higher power?

A. Scleromyxedema

B. Scleroderma

C. Scleredema

D. Localized Morphea

E. Pretibial myxedema

The doughnut sign is seen on proximal interphalangeal joints where a central depression surrounded by an elevated rim can be seen (Bolognia p649). This is a feature of the skin thickening seen in Scleromyxedema. The other options will have a square appearance on low power microscopic examination and scleredema can have an IgG paraprotein. Other microscopic findings in scleromyxedema include incrased fibroblasts with fibrosis/increased collagen and thickening of the dermis. Mucin may be scant or absent. This bx is more cellular than pretibial myxedema. (A)

8-Multiple dermatofibromas are seen in:

A. Cowden's

B. Lobomycosis

C. Incontinentia pigmenti

D. Lupus erythematosus

E. Reticulohistiocytosis

Multiple dermatofibromas are seen in lupus erythematosus and immunosuppression/HIV. (D)

9-All of the following are true regarding smooth muscle hamartomas except:

A. Hyperpigmentation

B. Transient piloerection

C. Hypertrichosis

D. Association with ?Michelin tire baby? in females

E. Becker?s nevus is an example

Smooth muscle hamartomas of the skin may develop from three locations: the arrector pili muscles, blood vessel walls, and genital/areolar skin. The have been associated with hyperpigmentation, tranisent piloerection ("pseudo-Darier's sign") and hypertrichosis. It is thought to be the underlying lesion in "Michelin tire baby" syndrome in boys, not girls.(D)

10-Multiple clear cell acanthomas are associated with:

A. Ichthyosis

B. Cowden's

C. Immunosuppression

D. Gastrointestinal polyps

E. Breast cancer

Clear cell acanthoma is associated with ichthyosis. (A)

11-Which of the following can be seen in dermatofibromas?

A. Hypopigmentation of the basal layer

B. Infiltration into the fat with a honeycomb pattern

C. Hypoplasia of the epidermis

D. S-100 positivity

E. Vimentin positivity

Dermatofibromas have characteristic features that can include collagen trapping, hyperplasia of epidermis, hyperpigmentation of basal layer, dermal spindle cells, and whorls of fibrous tissue with keloidal collagen. It stains with factor XIIIa(+) but not MAC 387 (-), S-100(-), or CD34 (-). DFSP infiltrates the fat with a honeycomb pattern. (E)

12-Which disease process best describes Texier's disease?

A. Neutrophilic dermatosis

B. Deposition disorder

C. Infectious process

D. Panniculitis

E. Granulomatous disease

Texier's disease is a panniculitis secondary to vitamin K injections causing sclerotic lesions with lilac borders on the buttocks and thighs resembling a cowboy belt and holster. (D)

13-Which fixative would best preserve the histologic features of a gout tophus?

A. Saline

B. Absolute ethanol

C. Formaldehyde

D. Michel's fixative

E. Tissue culture media

Gout tophi are caused by deposition of monosodium urate monohydrate crystals. Using absolute ethanol to fix the biopsy specimen will preserve the crystalline architechture. In formaldehyde fixed tissure, the crystals are less obvious. (B)

14-Immunohistochemical staining with neuron-specific enolase is positive in:

A. Anaplastic large cell lymphoma

B. Malignant firbroushistiocytoma

C. Cutaneous T cell lymphoma

D. Merkel cell carcinoma

E. Sebaceous carcinoma

Neuron-specific enolase is a cytoplasmic product produced by Schwann cells and neurons. This enzyme is present in neuroendocrine cells, neurons and tumors derived from them. Positive staining for neuron-specific enolase is found in Merkel cell carcinomas, carcinoid tumors, and malignant melanoma. (D)

15-Dorf balls are seen in which tumor?

A. Kaposi's sarcoma

B. Angiosarcoma

C. Tufted angioma

D. Kaposiform hemangioendothelioma

E. Dermatofibroma sarcoma protuberans

Dorf balls are pink amorphous globules seen in vessels in Kaposi's sarcoma. Typical histologic findings include proliferation of spindle cells, prominent slitlike vascular spaces, and extravasated red blood cells. (A)

16-Turk cells are found in what infection?

A. Roseola

B. Mumps

C. Rubella

D. Syphilis

E. Rubeola

Turk cells are atypical lymphocytes found in rubella. (C)

17-Granular cell tumors are derived from:

A. Connective tissue

B. Smooth muscle

C. Vascular tissue

D. Neural tissue

E. Adipose tissue

40% of granular cell tumors occur on the tongue. They appear well-circumscribed, raised, firm nodules. Histologically, the cells are plump and polygonal arranged in nests and cords. Cells are filled with fine granules representing lysozymes. The tumors are neurally derived and stain with S-100 and PAS. (D)

18-Which of the following drugs has been known to cause pyogenic granuloma?

A. Daunorubicin

B. Mithramycin

C. Isosfamide

D. Capecitabine

E. Paclitaxel

Systemic retinoids, indinavir and capecitabine have all been describe to cause pyogenic granulomas. (D)

19-The pigment deposits in ochronosis are accentuated with:

A. Cresyl violet

B. Methyl-green pyronin

C. Silver nitrate

D. Bodian

E. Cresyl violet and silver nitrate

Cresyl violet stains the pigment deposits in ochronosis black. Methylene blue will also stain the pigment black. This pigment does not stain with silver nitrate. The Bodian stain is for nerves. Methyl-green pyronin stains RNA and DNA. (A)

20-Multiple spiradenomas are seen in this syndrome:

A. Alagille

B. Brooke-Spiegler

C. Nicolau-Balus?

D. Schopf?s

E. Lhermitte-Duclos

Brooke-Spiegler syndrome combines multiple cylindromas and multiple trichoepitheliomas, and sometimes multiple spiradenomas can be seen as well. Alagille syndrome associates arteriohepatic dysplasia and nevus comedonicus. Nicolau-Balus? syndrome describes the constellation of multiple eruptive syringomas, milia, and atrophoderma vermiculata. Schopf?s syndrome combines multiple apocrine hidrocystomas, palmar-plantar keratoderma, teeth abnormalities (hypodontia), and onychodystrophy. Lhermitte-Duclos disease may be a manifestation of Cowden?s. In Lhermitte-Duclos, there is a proliferation in the cerebellum (dysplastic gangliocytoma) with macrocephaly.

(B)

21-Which of the following can be used to stain amyloid?

A. PAS

B. Eosin

C. Giemsa

D. Aldehyde fuchsin

E. Crystal violet

Stains for amyloidosis: Congo Red, Thioflavin T, Crystal Violet, Methyl violet, Pagoda red no. 9, PAS + diastase, amyloid P-component antibody (E)

22-Subcutaneous panniculitis-like T-cell lymphoma with an indolent course is positive for which of the following?

A. CD4

B. CD8

C. CD10

D. CD45

E. CD57

There seems to be two subsets of subcutaneous panniculitis-like T-cell lymphoma. One has an indolent course, and is often CD8-positive and is positive for the alpha-beta T-cell receptor. The other has an aggressive course, sometimes with evidence of systemic hemophagocytosis and high mortality. This subset is CD56-positive and positive for the gamma-delta T-cell receptor. (B)

23-The predominant location of the cleft in cicatricial pemphigoid is:

A. Dermal

B. Basment membrane zone

C. Basal keratinocytes

D. Supra basal

E. Subcorneal/granular

Cicatricial pemphigoid is an autoimmune blistering disease that presents with ulcers, blisters and erosions of mucosal surfaces, especially the eyes and mouth. The cleft in cicatricial pemphigoid is found in the basement membrane zone/subepidermal as the antigens are usually BPAg2,laminin 5 and alpha-6-beta-4 integrin. Direct immunoflourescence is identical to that of bullous pemphigoid showing linear IgG and complement deposits in the basement membrane zone. (B)

24-Positive staining with Gross cystic disease fluid protein 15 (GCDFP-15) suggests:

A. An apocrine origin

B. An eccrine origin

C. A sebaceous origin

D. A follicular origin

E. None of these answers are correct

Gross cystic disease fluid protein-15 (GCDFP-15) is a commonly used apocrine marker. (A)

25-Goblet cells are seen in:

A. Cutaneous ciliated cyst

B. Endometriosis

C. Dermoid cyst

D. Bronchogenic cyst

E. Steatocystoma

Bronchogenic cysts have a pseudostratified cuboidal or columnar lining that is ciliated; goblet cells are found in the lining as well. (D)

26-A healthy 6 month old girl has a subcutaneous nodule above her right eyebrow. A skin biopsy demonstrates a cystic lesion with adnexal structures in the wall. Your diagnosis is:

A. Steatocystoma

B. Pilar cyst

C. Nevus sebaceous

D. Epidermal inclusion cyst

E. Dermoid cyst

Dermoid cysts present along lines of embryonic closure. The are most commonly found on the head (around the eyes) and the neck. They are lined by an epidermis that contains various epidermal appendages that are usually fully matured. (E)

27-Which of the following is characteristic of pleomorphic lipoma?

A. Foreign body giant cells

B. Floret giant cells

C. Frequent mitoses

D. Exocytosis of lymphocytes

E. Virchow bodies

Pleomorphic lipomas characteristically have a mixture of variably sized fat cells with a varying number of pleomorphic enlarged cells. These cells contain nuclei arranged in a circumferential pattern that has been termed floret cells. Rare lipoblasts are found. Focal collections of lymphocytes and plasma cells within the tumor are seen. (B)

28-The inclusions in infantile digital fibromatosis stain for trichrome and:

A. Phosphotungstic acid hematoxylin

B. Osmium tetroxide

C. Thioflavin T

D. Bodian

E. Pentahydroxy flavanol

Osmium tetroxide stains fat. Thioflavin T stains amyloid. The Bodian stain is for nerves. Pentahydroxy flavanol is a fluorescent stain for calcium. (A)

29-Biopsy of a mucosal neuroma from a patient with MEN IIb looks histologically like a:

A. Neurilemmoma

B. Neurofibroma

C. Palisaded encapsulated neuroma

D. Traumatic neuroma

E. Neurothekeoma

Mucosal neuromas in MEN IIb often look histologically like PENs. Occasionally mucosal neuromas display thickened nerves/perineurium. (C)

30-Which of the following is commonly seen histopathologically in pityriasis rosea?

A. Absent spongiosis

B. Full thickness necrosis

C. Exravasated erythrocytes in the subcutaneous fat

D. Lichenoid lymphocytic infiltrate

E. Focal parakeratosis

Histopathological features of Pityriasis Rosea: Mild subacute spongiotic dermatitis, focal parakeratosis (corresponds to cigarette paper-like scale), perviascular lymphocytic infiltrate, hemorrhage in papillary dermis, and sometimes a few necrotic keratinocytes. (E)

31-This woman should have a workup for:

A. Lymphoma

B. Nephrolithiasis

C. Pancreatic cancer

D. Hemochromatosis

E. Thalassemia

Porphyria cutanea tarda has been shown to be associated with hemochromatosis. Patients with porphyria cutanea tarda have mutations in the HFE gene, and early detection of mutations can improve life expectancy for these patients. (D)

32-Osteoclast-like giant cells are characteristic of:

A. Giant cell fibroblastoma

B. Reticulohistiocytoma

C. Necrobiosis lipoidica diabeticorum

D. Giant cell tumor of the tendon sheath

E. Necrobiotic xanthogranuloma

Osteoclast-like giant cells are seen in giant cell tumor of the tendon sheath. Osteoclast-like giant cells have eosinophilic cytoplasm with haphazardly arranged nuclei. Reticulohistiocytoma is characterized by giant cells with an oncocytic glassy pink cytoplasm. (D)

33-Multiple pilomatricomas are seen in:

A. Myotonic dystrophy

B. Cowden's

C. Turner's

D. Gorlin's

E. Myotonic dystrophy and Turner's

Multiple pilomatricomas are seen in Rubinstein-Taybi, Gardner's (cyst-like pilomatricomas), myotonic dystrophy, Turner's, sarcoidosis, sternal cleft and coagulation defects. (E)

34-Which of the following stains is specific for melanin?

A. S-100

B. HMB-45

C. Fontana-Masson

D. MART-1

E. Melan-A

Fontana-Masson is a silver stain and stains melanin black. S-100, HMB 45, MART-1 (Melan-A) are melanocyte stains. S-100 also decorates Langerhans cells, acrosyringium, and neural crest-derived cells. (C)

35-The mutated product in Darier�s disease is:

A. SPINK5

B. SERCA2

C. ATP2A2

D. ATP2C1

E. SPINK5 and ATP2A2

In Darier�s disease, the ATP2A2 gene encoding the SERCA2 Ca(2+)-ATPase is mutated in some

patients. SPINK5 is the gene that is mutated in some patients with Netherton�s syndrome, and this

gene the serine protease inhibitor LEKTI. ATP2C1 is the gene mutated in some patients with Hailey-Hailey disease. (B)

36-What infectious agent is most likely responsible for a reaction of fibrin and antibodies which help to prevent phagocytosis?

A. Nocardia

B. Ricketsii species

C. Actinomycosis

D. Candida albicans

E. Anthrax

Hoeppli-Splendore reaction is characterized histologically by intensely eosinophilic material consisting of fibrin and antibodies. Causes of the phenomenon include Actinomycosis israelii, Staph aureus, Proteus, Pseudomonas and E. coli. (C)

37-Using the salt-split skin technique with direct immunofluorescence, epidermolysis bullosa acquisita will show linear deposition of complement in what position?

A. Roof of the split

B. Roof and floor of the split

C. Floor of the split

D. Neither the roof or floor of the split since IgA is the most common reactant

E. None of the above since the pattern is not linear

Epidermolysis bullosa acquisita (EBA) is a bullous disease of adults in which minor trauma (usually on the hands and feet) leads to blisters that heal with scaring. On histology there is classically a noninflammatory subepidermal split. The blister will immunostain with IgG on the floor of salt-split skin, as the antigen is type VII collagen. (C)

38-The diagnosis is:

A. Dermatofibrosarcoma

B. Nodular fasciitis

C. Angiolipoma

D. Epithelioid sarcoma

E. Liposarcoma

Angiolipomas are tumors of fat that are characteristically painful. Histopathologically, mature adipocytes are seen with numerous vessels. Microthrombi are often present. (C)

39-A lichenoid infiltrate that surrounds eccrine glands is seen in:

A. Lichenoid drug rection

B. Lichen striatus

C. Lichen planus

D. Lichenoid purpura

E. Lichen planopilaris

Lichen striatus is an uncommon inflammatory dermatitis seen most commonly in children aged 5 to 15. It presents unilaterally along Blaschko's lines as raised, slightly scaly, erythematous papules, which are often pruritic. These lesions typically regress spontaneously within a year. The histopathologic features of lichen striatus include a superficial perivascular inflammatory lymphohistiocytic infiltrate with rare plasma cells and eosinophils. There is a focal lichenoid infiltrate in the papillary dermis with basilar vacuolar alteration and necrotic keratinocytes. Spongiosis with exocytosis of lymphocytes can be seen in the epidermis. A specific and distinctive feature of lichen striatus is the presence of an inflammatory infiltrate that surrounds hair follicles and eccrine glands. (B)

40-Verruciform xanthoma is seen most commonly on:

A. Head and neck

B. Distal extremities

C. Oral mucosae and genital areas

D. Mucosal surfaces and trunk/proximal extremities

E. Nail bed and periungual areas

Verruciform xanthoma is an uncommon lesion that usually occurs on the oral mucosa of middle-aged persons or on the scrotum of middle-aged to elderly Japanese men. The most common site for verruciform xanthoma is the oral mucosa. (C)

41-Subcutaneous fat necrosis of the newborn has been associated with:

A. Hypocalcemia

B. Hypercalcemia

C. Hypokalemia

D. Hyperkalemia

E. Hyponatremia

Hypercalcemia has been noted in some cases of subcutaneous fat necrosis of the newborn. (B)

42-The histologic finding of "shoulder parakaratosis", parakeratosis with prediliection for the follicular ostia, is characteristic of pityriasis rubra pilaris as well as:

A. Stasis dermatitis

B. Atopic dermatitis

C. Seborrheic dermatitis

D. Nummular dermatitis

E. Allergic contact dermatitis

Parakeratosis refers to pyknotic keratinocyte nuclei in the stratum corneum, where nuclei are not usually present. It is common in diseases with changes in the epidermis. Histologically seborrheic dermatitis can shows "shoulder parakeratosis" with epidermal spongiosis. Histologically atopic, nummular and contact dermatitis present with spongiosis with or without vesicles. Stasis dermatitis presents with more dilated papillary dermal small blood vessels and hemosiderin. (C)

43-The predominant location of the cleft in erythema toxicorum neonatorum is:

A. Dermal

B. Basement membrane zone


D. Suprabasal


Erythema toxicorum neonatorum is an idiopathic vesiculopustular eruption on the face and trunk of neonates that often resolves in a few weeks. Histologically it presents with subcorneal eosinophil rich pustules.(E)

44-Cicatricial pemphigoid antibodies directed against this are associated with high frequency of malignancy:

A. Laminin 5

B. Laminin 6

C. Beta4-integrin

D. BPAg2

E. All of these answers are correct

Anti-laminin 5 cicatricial pemphigoid (CP) is also known as anti-epiligrin CP. Anti-epiligrin CP is associated with an increased frequency of internal adenocarcinomas. Laminin 5 is composed of three chains (heterotrimer), alpha3, beta3, gamma2. Antibodies are frequently directed against the alpha3 chain, and so cross-reactivity can be observed with laminin 6, as laminin 6 (alpha3beta1gamma1) has the alpha3 chain as well. Beta4-integrin antibodies have been associated with ocular CP. BPAg2 antibodies are seen in CP patients that have mucosal as well as skin disease. (A)

45-Which of the following shows granular deposition of IgA in the dermal papillae and along the basement membrane zone on direct immunoflourescence:

A. IgA pemphigus

B. Bullous pemphigoid

C. Linear IgA dermatosis

D. Dematitis herpetiformis

E. Herpes gestationalis

Dermatitis herpetiformis or Duhring's disease, presents with very pruritic vesicles symmetrically on extensor surfaces. On histology it presents as suprapapillary vesicles with mostly neutrophils and inflammatory destruction of the basement membrane zone. Direct immunoflourescence shows granular deposition of IgA in the dermal papillae and along the basement membrane zone. (D)

46-This patient developed an acute vesicular rash after eating a mango. She has returned for a routine follow-up. She needs to be careful of exposure to:

A. Ginkgo fruit

B. Croton

C. Ragweed

D. Tea tree oil


Patients allergic to the peel of a mango can also be allergic to other plants/products of the Anacardiaceae family. Cross-reactions can occur with exposure to any plants of the genus Toxicodendron, to the oil from the cashew nut shell, to the Brazilian pepper tree, to lacquer from the Japanese lacquer tree, to ink from the Indian marking nut, and to the fruit pulp of the ginkgo tree, and others. (A)

47-Which of the following histologic features is seen in aging skin?

A. Thickened dermal-epidermal junction

B. Increased mast cells

C. Increased number of terminal hairs

D. Fewer Langerhans cells

E. Increased sebum production

Histologic features of aging epidermis include flattened dermo-epidermal junction, occasional nuclear atypia, decrease in the number of melanocytes and Langerhans cells. Changes that are present in the dermis include atrophy, decrease in fibroblasts, mast cells and blood vessels.(D)

48-Which of the following stains with Ulex europeus agglutinin I?

A. Smooth muscle

B. Eccrine glands

C. Endothelial cells

D. Macrophages

E. Melanocytes

Ulex europeus agglutinin I is a stain which identifies endothelial cells, keratinocytes, angiosarcomes and Kaposi's sarcoma. (C)

49-The predominant location of the cleft in acropustulosis of infancy is:

A. Dermal

B. Basment mebrane zone


D. Suprabasal


Acropustulosis of infancy presents as idiopathic pustules on acral skin. Diagnosis is made only after other causes of pustules have been ruled out, and it usually resolves in a few years. The cleft in acropustulosis of infancy is subcorneal/granular with neutrophils. (E)

50-Langerhans cells express or are characterized by all of the following except:

A. Chromagranin

B. HLA-DR

C. CD1a

D. Birbeck granules

E. S-100

Chromagranin stain neuroendocrine cells, Merkel cellcarcinomas and eccrine glands. They do not stain Langerhans cells.(A)

51-All have been associated with increased risk of breast cancer except:

A. Cowden?s

B. Multicentric reticulohistiocytosis

C. Peutz-Jeghers

D. Birt-Hogg-Dube

E. ataxia telangiectasia

Birt-Hogg-Dube is associated with renal cancer and thyroid cancer. Female carriers of a mutated ATM (homozygous mutations ATM cause ataxia telangiectasia) have an increased risk of breast cancer. (D)

52-Eosinophilia-Myalgia syndrome is caused by:

A. Norwegian salt-petter

B. Unadultered Spanish grapeseed oil

C. Pb intoxication

D. L-Tryptophan

E. Excessive anaerobic exercise

The eosinophilia myalgia syndrome is characterized by marked peripheral eosinophilia with a clinical spectrum of signs and symptoms, including generalized myalgias, pneumonitis, myocarditis, neuropathy, encephalopathy and fibrosis. Many patients progress to a clinical picture clinically indistinguishable from eosinophilic fasciitis. The disease is caused by the ingestion of certain lots of L-tryptophan. (D)

53-Eccrine glands are found on the:

A. Labia majora

B. Labia minora

C. Glans

D. Prepuce

E. Vermilion

Eccrine glands are present all over the body except on the vermilion of lips, glans, labia minora, nail beds, and inner prepuce. (A)

54-Which of the following is characteristic of lichen planus histopathologically?

A. Wedge-shaped parakeratosis

B. Irregular acanthosis

C. Absent interface changes

D. Parakeratosis

E. Absent granular layer

Histopathology of lichen planus: Acanthosis with wedge-shaped hypergranulosis, irregular (sawtooth acanthosis), hyperkeratosis without significant parakeratosis, and basal vacuolization (interface changes).(B)

55-In addition to psoriasis, all of the following dermatoses demonstrate regular psoriasiform hyperplasia except:

A. Lichen simplex chronicus

B. Pityriasis rubra pilaris

C. Acanthosis nigricans

D. Reiter?s syndrome

E. Inflammatory linear verrucous epidermal nevus

Lichen simplex chronicus develops in areas of chronically rubbed skin. On histopathology, there is hyperkeratosis with areas of parakeratosis, hypergranulosis, slight spongiosis, and a sparse superficial perivascular infiltrate. The acanthosis in LSC is irregular. The other choices all have regular psoriasiform hyperplasia. (A)

56-A patient with a blue-red discoloration of the nail plate reports that the same finger becomes very tender when exposed to the cold. You suspect a:

A. Periungual verruca

B. Glomus tumor

C. Pterygium

D. Mucous cyst

E. Pyogenic granuloma

Glomus tumors are tumors of the arterio-venous anastamosis of the digital dermis. They occur most frequently in the nail bed. The commonly have a bluish-red discoloration and may be tender or painful with exposure to heat or cold. (B)

57-Acantholysis is not a prominent histopathologic feature of which disease?

A. Dermatitis herpetiformis

B. Darier's disease

C. Grover's disease

D. Pemphigus vulgaris

E. Hailey-Hailey diesease

Dermatitis herpetiformis is a bullous disease that on histology shows neutrophils in the dermal papillae. On direct immunoflourescence IgA is seen in a granular depostion pattern. Acantholysis is seen histologicaly in Darier's disease, Grover's disease, Pemphigus vulgaris, Hailey-Hailey disease and warty dyskeratoma.(A)

58-The predominant cleft in dermatitis herpetiformis is:

A. Dermal



D. Suprabasal


Dermatitis herpetiformis or Duhring's disease, presents with very pruritic vesicles symmetrically on extensor surfaces. On histology it presents as suprapapillary vesicles with mostly neutrophils and inflammatory destruction of the basement membrane zone. Direct immunoflourescence shows granular deposition of IgA in the dermal papillae and along the basement membrane zone. The cleft in dermatitis herpetiformis is most commonly found in the basement membrane zone/subepidermal. The antigen is transglutaminase.(B)

59-All are sebaceous glands except:

A. Tyson

B. Moll

C. Wolf

D. Fordyce

E. Meibomian

Moll?s glands are modified apocrine glands located on the eyelid. Sebaceous glands in certain locations have an associated name (Tyson?s on the penis, Wolf?s or Meibomian on the eyelid, and Fordyce on the vermilion/oral mucosa). (B)

60-In which of the following would you expect to find Dutcher bodies?

A. Molluscum contagiosum

B. Sarcoidosis

C. Chromoblastomycosis

D. Schwannoma

E. Chronic lymphocytic leukemia

Dutcher bodies may be found in chronic lymphocytic leukemia and multiple myeloma. They represent intranuclear inclusions of immunoglobulin, which may be mistaken for nucleoli.(E)

61-Psoriasis has been shown to be genetically linked to:

A. Pityriasis rubra pilaris

B. Clear cell acanthomas

C. Epidermodysplasia verruciformis

D. Verruciform xanthomas

E. Poromas

Mutations have been found in the EVER1 and EVER2 genes in epidermodysplasia verruciformis; these genes are on 17q25, the location of the PSORS2 gene (one of the genes implicated in the inheritance of psoriasis). (C)

62-This body contains calcium:

A. Negri body

B. Lipschutz body

C. Michaelis-Gutman body

D. Guarnieri body

E. Negri body and Michaelis-Gutman body

The Michaelis-Gutman body is a concentrically laminated spherical inclusion that contains calcium that is seen within macrophages in malakoplakia. Other inclusion bodies that contain calcium are Schaumann bodies and psammoma bodies. The Negri body is seen in rabies. The Lipschutz body is an intranuclear inclusion seen in herpes. It is considered synonymous with the Cowdry A body. The Guarnieri body is seen in smallpox. (C)

63-Clear cell syringomas are associated with:

A. Malignancy

B. Sarcoidosis

C. Argyria

D. Diabetes

E. Lichen myxedematosis

Clear cell syringomas are associated with diabetes. Syringomas are associated with Down�s

syndrome. (D)

64-All of the following are true of reticulohistiocytoma except:

A. Rare occurrence in children

B. Giant cells with ?ground-glass? cytoplasm

C. Association with arthritis

D. Immunostaining is positive for OKM1

E. Trauma is precipitating factor

Reticulohistiocytomas, also called giant cell reticulohistiocytomas, occur almost exclusively in adults. They are generally solitary, and unlike the multicentric type, are not associated with mutilating arthritis or predisposition for malignancy. (C)

65-This is associated with MEN IIa:

A. Neurothekeoma

B. Macular amyloidosis

C. Malignant peripheral nerve sheath tumor

D. Chondroid syringoma

E. Mucocele

Macular amyloidosis is associated with MEN IIa. (B)

66-In this patient, this test will be helpful in making the diagnosis:

A. Fluorescent antibody test for herpes

B. Patch test

C. Tissue culture

D. Indirect immunofluorescence

E. KOH exam

This patient has herpes zoster. A direct fluorescent antibody test for the varicella zoster virus can be performed to confirm the diagnosis.(A)

67-This patient says the rash is spreading and not controlled with topical therapy. You give him a course of oral treatment that lasts:

A. 1 week

B. 2 weeks

C. 3 weeks

D. 4 weeks

E. 5 weeks

Generally, for poison ivy dermatitis, if patients are given a course of oral steroids, the course should be at least 3 weeks long, as if the duration is shorter, patients may develop a rapid rebound.(C)

68-The deficiency in familial multiple cutaneous leiomyomatosis is:

A. Arginase

B. Adenosine deaminase

C. Endoglin

D. MC1R

E. Fumarate hydratase

Fumarate hydratase is deficient in familial multiple cutaneous leiomyomatosis. This same enzyme is deficient in familial uterine leiomyomatosis associated with renal cell cancer. Arginase is deficient in arginemia. Adenosine deaminase is deficient in autosomal recessive severe combined immunodeficiency disease (SCID). Endoglin is deficient in some patients with Osler-Weber-Rendu. MC1R (melanocortin 1 receptor) shows sequence variation in people with red hair. (E)

69-An increased number of miniaturized hairs are seen in:

A. Lichen planopilaris

B. Alopecia areata

C. Trichotillomania

D. Telogen effluvium

E. Lichen planopilaris and alopecia areata

In alopecia areata, especially early stages, increased miniaturized catagen hairs can be seen in

addition to the finding of peribulbar lymphocytes resembling a �swarm of bees.� Lichen

planopilaris is a scarring alopecia in which vertical tracts of fibrosis are seen in place of follicles. Trichotillomania displays follicular plugging, trichomalacia, pigmented casts, hemorrhage, and increased catagen hairs on biopsy. Telogen effluvium is characterized by an increased number of telogen hairs. (B)

70-This tumor is vimentin+ and cytokeratin+:

A. Angiosarcoma

B. Neurothekeoma

C. Nodular fasciitis

D. Dermatofibroma

E. Epithelioid sarcoma

Characteristic immunohistochemistry of epithelioid sarcoma is vimentin- and cytokeratin- positivity. (E)

71-On biopsy of this patient?s skin lesion, a split would be expected to be seen at the following level:

A. Right below the stratum corneum

B. Within the stratum spinosum

C. Within the basal layer

D. At varying levels in the epidermis

E. Nowhere; there is only a dense lichenoid infiltrate

This infant has epidermolysis bullosa simplex, a disorder in which there is defective keratin 5 and 14. Because of this defect, there is abnormal attachment of the basal cells at the dermoepidermal junction, and a split forms ?within? the basal layer. (C)

72-Elastosis perforans serpiginosa is associated with all except:

A. Rothmund-Thompson

B. Scleredema

C. Ehlers-Danlos

D. Acrogeria

E. Down?s

Elastosis perforans serpiginosa is associated with Down?s syndrome, Ehlers-Danlos type IV, Osteogenesis imperfecta, Rothmund Thompson, Marfan?s, Werner?s, acrogeria, and penicillamine therapy. (B)

73-A skin biopsy shows numerous fibroblasts with fibrosis and thickening of the dermis. There is sparse mucin deposition and on low power the biopsy appears square. Which of the following paraproteins would you expect to find in this patient?

A. IgG lambda

B. IgA

C. IgM

D. IgG kappa

E. IgA gamma

The description of the biopsy above is that of scleromyxedema. These patients have an associated IgG lambda paraprotein. Other IgG subtypes may occur but lambda is most common. Scleromyxedema is a subset of lichen myxedematosus (papular mucinosis). It will present with coalescent erythematous to yellow papules and plaques. The most common location is the face, but will occur in many other locations. The surrounding skin is usually sclerodermoid in appearance. (Bolognia, p648-9) (A)

74-The predominant location of the cleft in transient neonatal pustular melanosis is:

A. Dermal



D. Suprabasal


Transient neonatal pustular melanosis is an idiopathic pustular eruption of newborns, mostly on the chest, that heals with hyperpigmentation. It is most common on pigmented individuals. Histologically it presents as subcorneal pustules with eosinophils and neutrophils. (E)

75-Methyl-green pyronin stains RNA what color?

A. Pink

B. Green

C. Blue

D. Purple

E. Black

Methyl-green pyronin stains RNA pink and stain DNA green. (A)

76-Blue-gray pigmentation on the legs secondary to minocycline on biopsy stains with:

A. Fontana Masson

B. Perls

C. Sudan black

D. Fontana Masson and Perls


There are three types of pigmentary change that are caused by minocycline. The blue-gray pigmentation on the legs and the blue pigment in scars is thought to be secondary to a drug-protein complex deposited in the dermis. The blue-gray pigment on the legs stains with Perls and Fontana-Masson. The blue in scars (often on the face) stains with Perls. The muddy-brown discoloration on sun-exposed areas shows increased basilar pigment and melanin incontinence on biopsy. It is likely secondary to phototoxicity. (D)

77-Which of the following hitologic features would be most helpful in differentiating lichenoid drug eruption from lichen planus?

A. Civatte bodies

B. Parakeratosis and eosinophils

C. Squamatization of the basal layer

D. Presence of pruritus

E. Band-like infiltrate with ?Saw-tooth? rete ridges

Lichenoid drug eruptions share clinical and histopathologic features with lichen planus. Sometimes differentiation is not possible; however, eosinophil, parakeratosis, and a deeper perivascular infiltrate is more suggestive of lichenoid drug. Implicated medications include captopril, penicillamine, and chloroquine. (B)

78-CK20 is useful in the diagnosis of:

A. Dermatofibrosarcoma

B. Dermatofibroma

C. Epitheloid sarcoma

D. Merkel cell carcinoma

E. Plasmacytoma

CK20 is useful in diagnosis of Merkel Cell Ca. Dermatofibrosarcoma would stain CD34+.

Dermatofibroma would stain CD68 +. Plasmacytoma would stain CD138 + and CEDa�. Epithelioid

sarcoma would stain CD34+. (D)

79-Weibel-Palade bodies are seen in:

A. Endothelial cells

B. Spitz Nevi

C. Cells infected with MCV

D. Plasmacytoid Cells

E. Malakoplakia

Weibel-Palade bodies are seen in endothelial cells and are therefore found in vascular lesions. Kamino bodies are found in Spitz nevi. Henderson Patterson bodies are seen in molluscum. Dutcher bodies are intranuclear inclusions seen in plasmacytoid cells. Michaelis Gutmann bodies are partially digested bacteria seen in malakoplakia. (A)

80-The cytoplasmic granules seen in granular cell tumor are:

A. Phagolysosomes

B. Ribosomes

C. Mitochondria

D. Intermediate filaments

E. Vacuoles

The granules in granular cell tumor are phagolysosomes. The granularity of the granular cell layer in epidermodysplasia verruciformis may be secondary to increased ribosomes. Mitochondria fill the cells in hibernoma. (A)

81-This patient has a lichenified plaque in the lower mid abdomen as well as these two exczematous plaques. This patient needs:

A. patch testing

B. a steroid

C. an antiviral

D. a KOH scraping

E. an antifungal

This patient is likely allergic to the nickel in his belt buckle and possibly to metals that he places in his pocket. He needs patch testing. While a topical steroid might help resolve pruritus, the patient ultimately needs to be diagnosed with his allergy and avoid nickel. Dimethylglyoxime can be used as an indicator to detect nickel in metals. (A)

82-Which of the following is true regarding pilomatricomas?

A. Calcification is rarely seen

B. Poorly demarcated tumor

C. Comprised of shadow cells and basophilic cells

D. Mutations found in the APC gene

E. Frequent malignant degeneration

Pilomatricomas are well-defined lobular tumor in dermis or subcutis comprised of germinative-matrical cells that are basaloid and transition to "ghost" or "shadow" cells. Calcifiation or ossification are frequently seen. Mutations are found in beta catenin. (C)

83-Which of the following is characteristic of PLEVA?

A. Alternating orthokeratosis and parakeratosis

B. Red cell extravasation

C. Dermal mucin deposition

D. Fibrinoid necrosis of medium sized vessels

E. Numerous eosinophils and neutrophils

Pityriasis lichenoides et varioliformis acuta has characteristic findings. Parakeratosis, spongiosis with vacuolar alteration, papillary dermal edema, wedge shaped infiltrate, and extravasated RBC's can all be seen. Occasionally, the infiltrate can include atypical lymphoid cells. Eosinophils and neutrophils are more frequently seen in LyP. (B)

84-Steatocystoma multiplex is associated with:

A. Jadassohn-Lewandowsky

B. Jackson-Lawler

C. Schaufer-Brunauer

D. Zinsser-Engman-Cole

E. Touraine-Solente-Gole

Jackson-Lawler (Jackson-Sertoli) is known as pachyonychia congenital type 2. Multiple steatocysts can be seen in this condition. (B)

85-What is the diagnosis?

A. Rosacea

B. Angiofibroma

C. Palisaded encapsulated neuroma

D. Kaposi's sarcoma

E. Radiodermatitis

This is an angiofibroma, in which there are slightly dilated superficial vessels, stellate fibroblasts, and incrased collagen. Sometimes the collagen surround vessels and adnexal structures in an ?onion-skin? pattern. (B)

86-Histologically, adenoma sebaceum represent which of the following lesions?

A. Neurofibromas

B. Angiofibromas

C. Collagenomas

D. Angiokeratomas

E. Smooth muscle hamartomas

Adenoma sebaceum, fibrous papules and pearly penile papules all have similar features histologically, presenting as angiofibromas. Features include atrophic epidermis with patchy melanocytic hyperplasia and hyperkeratosis, vertically oriented collagen, increased fibroblasts and blood vessels. (B)

87-Lipomembranous change is seen in:

A. Hibernoma

B. Cystic sebaceous adenoma

C. Sebaceous carcinoma

D. Mucocele

E. Sclerosing panniculitis

Lipomembranous change is a non-specific histologic pattern that is most commonly seen in lipodermatosclerosis, which is also known as sclerosing panniculitis; this condition may be secondary to venous stasis. (E)

88-Multiple trichoepitheliomas are seen in all except:

A. Bazex's syndrome

B. Brooke-Fordyce syndrome

C. Brooke-Spiegler syndrome

D. Gorlin's syndrome

E. Rombo syndrome

Gorlin's syndrome is nevoid basal cell carcinoma syndrome; multiple trichoepitheliomas are not seen. Several syndromes have been associated with multiple trichoepitheliomas: Basex, Brooke-Fordyce, Brooke-Spiegler, Rombo, and possibly Rasmussen. (Rasmussen described one family in 1975 with autosomal dominant inheritance of multiple trichoepitheliomas, milia, and cylindromas.) Basex syndrome (follicular atrophoderma, hypotrichosis, occasional trichoepitheliomas, basal cell carcinomas, and localized or generalized hypohidrosis) is inherited in an X-linked dominant manner. Brooke and Fordyce both described multiple trichoepitheliomas concurrently in 1892, and therefore multiple familial trichoepitheliomas are sometimes called ?Brooke-Fordyce? sydrome. Spiegler described patients with multiple cylindromas in 1899 and also noted that many of these patients had mutiple trichoepitheliomas; more recently it has been noted that multiple spiradenomas may be seen in patients with multiple trichoepitheliomas and cylinidromas; this co-occurrence of tumors has been referred to as ?Brooke-Spiegler? syndrome. (Brooke-Fordyce and Brooke-Spiegler are likely the same syndrome.) Rombo syndrome is characterized by vermiculate atrophoderma, multiple BCCs, multiple trichoepitheliomas, cyanosis and peripheral vasodilation. (D)

89-The green color in chloroma is secondary to:

A. Stromelysin

B. Chloracetate

C. Fumarase

D. Myeloperoxidase

E. Alkaline phosphatase

Chloromas are greenish tumor grossly secondary to involvement of the skin in acute granulocytic leukemia. The green color is secondary to myeloperoxidase. (D)

90-Diffuse staining with this marker is suggestive of BCC rather than trichoepithelioma:

A. CAM 5.2

B. Bcl-2

C. PTAH

D. CEA

E. Cytokeratin 8

Trichoepitheliomas stain with bcl-2 on the periphery of individual tumor islands. (B)

91-Birt-Hogg-Dube has been associated with increased risk of renal cancer and:

A. Spontaneous pneumothorax

B. Hepatic cysts

C. Adrenal hemorrhage

D. Cirrhosis

E. Gastrointestinal polyps

The gene defect in Birt-Hogg-Dube is folliculin. (A)

92-Paraproteinemia is associated with all except:

A. Scleromyxedema

B. Necrobiotic xanthogranuloma

C. Scleredema

D. Sclerosing panniculitis

E. Plane xanthoma

Sclerosing panniculitis (lipodermatosclerosis) displays characteristic changes in the fat (lipomembranous change); it is not associated with paraproteinemia. Generalized plane xanthomas, scleromyxedema, necrobiotic xanthogranuloma, scleredema, erythema elevatum diutinum, xanthoma disseminatum, and pyoderma gangrenosum have all been associated with a paraproteinemia. (D)

93-Histopathologically, a swiss cheese type appearance is characteristic to which of the following?

A. Silica

B. Tattoos

C. Liquid silicone

D. Sea urchin spines

E. Zirconium

Parafinoma and silicone granuloma both have a swiss cheese type appearance in the dermis to subcutaneous fat. The remaining options typically appear sarcoidal. (C)

94-Which of the following would suggest a diagnosis of malignant lymphoma, B-cell type over pseudolymphoma, B-cell type?

A. Mixed-cell infiltrate

B. Mitotic figures only in germinal centers

C. Histoicytic giant cells episodically

D. Larger lymphocytes predominate

E. Prominant vasculature with thick-walled blood vessels lined by plump endothelial cells

Histological findings in pseudolymphoma, B-cell type include a mixed-cell infiltrate (important diagnostic criterion), small lymphocytes (that predominate), the presence of histiocytes and giant cells, mitotic figures limited to the germinal centers and prominant vasculature. (D)

95-This patient recently developed this rash. You decide to patch test her, but in the meanwhile you tell her to avoid:

A. Chamomile

B. Primin

C. Abietic acid

D. Benzocaine

E. Cinnamon

This patient likely has a fragrance allergy. Patients allergic to fragrances also need to avoid certain spices like cinnamon.(E)

96-Clinically, a nondescript hyperkeratotic papule on the ulnar side of the base of the fifth finger is most likely:

A. Acquired digital fibrokeratoma

B. Accessory digit

C. Cutaneous horn

D. Digital fibromatosis

E. Glomus tumor

Accessory digits (supernumerary digits) are usually found at the base of the fifth finger, often bilaterally. (B)

97-Which of the following is more commonly seen in lichen sclerosus et atrophicus compared to morphea?

A. Inflammation and fibrosis in the subcutis

B. Marked edema in the papillary dermis

C. Minimal hydropic degeneration noted at the dermaldermal-epidermal junction

D. Epidermis relatively normal with no thinning of the rete ridges

E. No follicular plugging

Morphea vs. Lichen schlerosus et atrophicus: Morphea has a relatively normal epidermis without follicular plugging. Interface changes are rare. In the dermis, the collagen fibers appear homogenized. Elastic fibers are preserved. The inflammation and sclerosis can extend into the subcutaneous fat. LS&A has an atrophic epidermis with follicular plugging. There is often prominent hydropic changes with clefting. There is often edema of the papillary dermis and elastic fibers are often absent. The subcutis is uninvolved.(B)

98-Treatment of postmenopausal women with systemic conjugated estrogens has demonstrated:

A. Increased cellular atypia

B. Decreased mitotic activity of keratinocytes

C. Increased dermal collagen content

D. Decreased sebaceous gland activity

E. Increased skin laxity

Systemic use of conjugated estrogens in postmenopausal women increases total skin thickness, dermal collagen content and mitotic activity of keratinocytes. In addition, there is a reduction of dry skin, slackness and increased hydration.(C)

99-Which immunohistochemical stain would be positive in eosinophilic granuloma?

A. HMB-45

B. Cytokeratin 20

C. Congo red

D. Mucin

E. CD1a

Eosinophilic granuloma is a form of Langerhans Cell Histocytosis (LCH), previously called Histiocytosis X. Eosinophilic granuloma is a localized, benign form which is more common in males and generally affects the bones. All forms of LCH are characterized by the infiltration of Langerhans cells on pathology, which staing for S-100, CD1a and contain cytoplasmic birbeck granules.(E)

100-Silver preferentially deposits in:

A. Eccrine glands

B. Apocrine glands

C. Hair follicles

D. Fat

E. Eccrine glands and apocrine glands

Argyria can look like normal skin if you do not notice the silver (black) deposits in the eccrine glands in the deep dermis. (A)

101-Psoriasis vulgaris may demonstrate all except one of the following histopathologic changes.

A. Munro microabscesses

B. Clubbing of the rete ridges

C. Spongiform pustules of Kogoj

D. Hypergranulosis

E. Suprapapillary thinning

Histopathological features of psoriasis: Hyperkeratosis with confluent parakertosis associated with a thin to absent granular layer, Uniform elongation of rete ridges and they are expanded at their tips (Clubbing of rete ridges), papillary dermal edema with dilated toruous capillaries, thinned suprapapillary plates, neutrophils within stratum corneum (Munro's micorabscesses), neutrophils within the spinous layer (spongiform pustule of Kogoj), and superficial perivascular lymphocytic infiltrate.(D)

102-Mulberry cells contain increased:

A. Phagolysosomes

B. Mitochondria

C. Golgi

D. Ribosomes

E. Phagolysosomes and mitochondria

Hibernomas commonly arise in the neck, axillae, and posterior shoulder. The cells are multivacuolated and resemble mulberries; the cells are filled with mitochondria, as are the cells in normal brown fat. (B)

103-This is considered to be the juvenile counterpart of DFSP:

A. Juvenile hyaline fibromatosis

B. Giant cell fibroblastoma

C. Myxofibrosarcoma

D. Myofibromatosis

E. Plexiform fibrous histiocytoma

An entity called giant cell fibroblastoma is CD34-positive, is mostly seen in male children on the neck/trunk, and is thought to be a juvenile counterpart of DFSP. (B)

104-Which of the following lesions demonstrates a pseudo-Darier?s sign?

A. Mastocytoma

B. Spitz nevus

C. Smooth muscle hamartoma

D. Pilomatricoma

E. Bullous pemphigoid

Smooth muscle hamartomas are benign tumors which arise from smooth muscle of the dermis. Pseudo-Darier's sign may be elicited due to transient piloerection after rubbing. Histologically, red-orange bundles and fascicles are present with blunt-ended nuclei. (C)

105-Which of the following regarding stains is true?

A. A Verhoeff-van Gieson stain is used to stain elastic fibers red.

B. A methanamine silver stain is used to identify bacteria.

C. A fite stain is used to identify spirochetes.

D. A Von Kossa stain is used to identify calcium.

E. A giesma stain is used to identify eosinophil granules.

Stains/Application/Result Van Gieson - Elastic fibers - Black Methanamine-silver - Fungi, parasites - Black AFB/Fite - Acid-fast bacilli - Red Von Kossa - Calcium - Black Giesma - Mast cells - Metachromatically purple (D)

106-The predominant location of the cleft in porphyria cutanea tarda is:

A. Dermal



D. Subrabasal


Porphyria cutanea tarda (PCT) is the most common porhyria. It is due to a deficiency of uroprophyrin decarboxylase. Adults present with sun induced vesicles, papules, crusts and milia in areas of scaring. On histology the cleft in porphyria cutanea tarda is subepidermal or in the basement membrane zone with festooning of dermal papillae into the blister. Hyalinized material around blood vessels in the papillary dermis may be seen, as well as caterpillar bodies (eosinophilic segemented basement membrane) in the roof of the blister. PCT is associated with hepatitis C, alcohol abuse and liver disease. (B)

107-Comma-shaped bodies are seen in:

A. Benign cephalic histiocytosis

B. Malakoplakia

C. Sarcoidosis

D. Lipoid proteinosis

E. Gaucher�s

Comma-shaped bodies and worm-shaped bodies can be seen in a variety of histiocytoses and are non-specific. The most common association is with benign cephalic histiocytosis. (A)

108-Which of the following is true regarding S-100 Protein?

A. S-100 protein is an basic protein that binds Ca2+ and Zn2+

B. It is not soluble in 100% ammonium sulfate at neutral pH

C. Can be detected in melanocytes and in Schwann cells

D. It is not useful in diagnosing of spindle cell melanoma & desmoplastic melanoma

E. It is not useful in diagnosing poorly differentiated cutaneou

S-100 protein is an acidic protein that binds Ca2+ and Zn2+. At a neutral pH, it is soluble in 100% ammonium sulfate. It is useful in diagnosing both spindle cell melanomas, desmoplastic melanomas, as well as poorly differentiated cutaneous metastases. It also stains positively in neurofibromas and schwannomas. (C)

109-Which of the following is characteristic of Birt-Hogg-Dube Syndrome?

A. Autosomal recessive mode of inheritance

B. Multiple trichoepitheliomas

C. Caused by mutation in hamartin

D. Multiple trichodiscomas

E. Colon cancer common

Birt-Hogg-Dube Syndrome (BHD) characterized by multiple small harartomas of mesodermal component of hair discs, which were indentified as trichodiscromas. It is an autosomal dominant disease caused by mutations in folliculin. Patients with multiple fibrofolliculomas may also have acrochordons, collagenomas, lipomas, and/or oral fibromas. BHDS recently has been reported in association with various types of renal tumors, such as oncocytoma and a variant of papillary renal cell carcinoma. There are no trichoepitheliomas in BHD syndrome.(D)

110-Both mesenchymal and epithelial elements may be found in this tumor:

A. Cylindroma

B. Microcystic adnexal carcinoma

C. Chondroid syringoma

D. Bednar tumor

E. Folliculosebaceous cystic hamartoma

Cutaneous mixed tumor, also known as Chondroid syringoma, represents an acquired hamartoma with folliculosebaceous-apocrine differentiation that has been generally interpreted as a form of adnexal adenoma (neoplasm). It has both a mesenchymal and epithelial component.(C)

111-Which of the following stains would be helpful in the diagnosis of cryptococcus?

A. Colloidal iron

B. Oil red O

C. Mucicarmine

D. Giemsa

E. Verhoeff von Gieson

The yeast cytoplasm of Crytococcosis stains with PAS and methanamine silver while the the capsule stains with Alcain blue and mucicarmine. Colloidal iron stains mucin; Oil red O stains fat; Giemsa stains mast cells and leishmaniasis; Verhoeff von Gieson stains elastic tissue. (C)

112-Cellular neurothekeoma stains with:

A. Stromelysin-3

B. Desmin

C. S-100

D. PGP-9.5

E. Low molecular weight keratin

PGP-9.5 and S100-a6 stains cellular neurothekeoma. Stromelysin-3 is positive in dermatofibromas and negative in dermatofibrosarcoma protuberans. Desmin stains rhabdomyosarcoma. S-100 stains neural tumors and melanocytic tumors among other things, but cellular neurothekeomas are generally S100-negative. (D)

113-The PDGF gene has been shown to be translocated into the collagen I gene in:

A. Angiofibroma

B. Dermatofibroma

C. Dermatofibrosarcoma protuberans

D. Fibrous hamartoma of infancy

E. Solitary fibrous tumor

A translocation involving the PDGF gene and collagen I gene is found in dermatofibrosarcoma protuberans. (C)

114-Stromelysin 3 is a negative marker for which of the following?

A. Dermatofibroma

B. Basal cell carcinoma

C. Dermatofibromasarcoma protuberans

D. Squamous cell carcinoma

E. Breast carcinoma

Stromelysin 3 is a negative marker which helps to distinguish dermatofibrosarcoma protuberans from dermatofibromas. Stromelysin 3 is a metalloproteinase which is expressed tissue remodeling. In a study performed by Cribier et.al. 100% of dermatofibromas stained positive Stromelysin 3 (ST3) is a member of the metalloproteinase family, which is expressed in tissue remodeling processes such as scarring, embryogenesis, or tumoral invasion. (C)

115-The most common cause of a longitudinal groove of the nail plate is:

A. Myxoid cyst

B. Verruca vulgaris

C. Psoriasis

D. Onychomycosis

E. Paronychia

A myxoid cyst is not a true cyst as it is devoid of cyst lining. They usually occur in an acral location and are the most common cause of a longitudinal groove of a nail plate.(A)

116-Histologic examination of cutaneous metastases from breast cancer is likely to demonstrate:

A. Dense lymphocytic infiltration

B. Tumors cells perivascularly

C. Eosinophilia

D. Tumor cells in the lymphatics

E. Band-like dermal infiltrate

Histologic appearance of metastatic breast carcinoma to the skin typically shows tumor cells in

linear arrangement in so-called �Indian-filing� pattern. These tend to occur in the dermis and

subcutaneous lymphatics. Extensive lymphatic dissemination may be caused by retrograde lymphatic spread. The tumor cells are large, pleomorphic with hyperchromatic nuclei.(D)

117-Supporting evidence for the diagnosis of mycosis fungoides is CD4+ lymphocytes with loss of CD7 as well as loss of

A. CD2

B. CD3

C. CD5

D. CD20

E. CD30

CD5 as well as CD7 are sometimes lost on the surface of epidermotropic T cells in mycosis fungoides. CD2, CD3, and CD5 are T cell markers. CD20 is a B cell marker. CD30 is positive in

anaplastic large cell lymphoma cells, Hodgkin�s lymphoma, and lymphomatoid papulosis. Reactive

infiltrates can also have some CD30-positive cells. (C)

118-For this patient, you request that the lab perform indirect immunofluorescence using what substrate?

A. Monkey esophagus

B. Rat bladder

C. Guinea pig esophagus

D. Mouse epithelium

E. Hep-2 cells

This patient has pemphigus folicaceus, and indirect immunofluorescence works best on guinea pig esophagus.(C)

119-Which of the following stains Natural Killer Cells?

A. CD1a

B. CD11

C. CD30

D. CD56

E. CD68

CD56 stain Natural Killer Cells and angiocentric lymphomas.(D)

120-Degeneration of cartilage in chondrodermatitis nodularis helices affects which type of collagen?

A. Type I collagen

B. Type II collagen

C. Type III collagen

D. Type IV collagen

E. Type VII collagen

Chondrodermatitis nodularis helices presents as a tender nodule usually on the helix or antihelix of the ear. It is thought to be a result of vascular insufficiency. Degeneration of the cartilage is seen on histopathology which is primarily composed of type II collagen.(B)

121-This is a desmoplakin:

A. BPAg1

B. Plakoglobin

C. Plakophilin

D. Desmocollin

E. Beta-catenin

Desmoplakins include desmoplakin 1, BPAg1, envoplakin, and periplakin. (A)

122-62-year old female with history of acute myeloid leukemia presents with multiple edematous, erythematous papules after starting G-CSF.

A. Sweet's syndrome

B. Leukocytoclastic vasculitis

C. Bowel bypass dermatosis

D. Polymorphous light eruption

E. Erythema multiforme

Sweet's syndrome, or acute febrile neutrophilic dermatoses, is often associated with AML and G-CSF. Histologically, there is marked dermal edema with a prominent infiltrate composed of neutrophils with leukocytoclasia. There is an absence of extensive vascular damage.(A)

123-The predominant location of the cleft in linear IgA is:

A. Dermal



D. Suprabasal


Linear IgA bullous dermatosis is an autoimmune disorder that presents with tense blisters along red annular rings (like a string of pearls). Antibodies are found in the lamina lucida against LAD-1 antigen in anchoring filaments. The cleft in linear IgA would be found in the basement membrane zone/subepidermal with direct immunostaining IgA in a linear pattern at the dermal-epidermal junction.(B)

dermatopathology q - a

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