cpc kurban iv

8/8/2019 CPC Kurban IV

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CPC IV

1) Minocycline Pigmentation

2) Kyrle Disease

3) Molloscum contagiosum

4) Acrokeratoelastoidosis ofCosta

5) Tungiasis

6) Trichomycosis axilaris

7) Pseudocyst of the auricle

8) Subcorneal PustularDermatosis

9) Acrokeratosis Verruciformisof Hopf

10) NecrobioticXanthogranuloma

11) Elastosis PerforansSerpiginosum

12) Angiokeratoma

13) Syringoma

14) Urticaria

15) Poikiloderma

16) Keratosis LichenoidesChronica

17) Hyperkeratosis LenticularisPerstans (Flegels)

18) Infantile myofibromatosis

19) Papular acrodermatitis ofchildhood (Gianotti-Crosti)


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Case 1


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Minocycline pigmentation


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Clinical-minocycline

pigmentation Blue-black discoloration in areas of priorinflammation-acne scars (type I).

Blue-black on shins (type II).

Generalized muddy brown hyperpigmentation,accentuated in sun-exposed areas (type III)-uncommon.

Teeth-grey or grey green on midportion of tooth

(different than tetracycline). Can also affect sclera, ears,bone, thyroid,

nailbed.


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Path: Minocycline pigmentation

Brown dermal pigment

Positive with iron and melanin stains

Pigment granules within dermalmacrophages.


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Case 2


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Kyrle Disease

(Acquired perforatingdermatoses)


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Clinical-Kyrle

Associated with renal failure and/or diabetes

4-10% of dialysis patients-usu legs

Variable itchiness.

Felt to be a response to trauma-the scratching in

reponse to pruritis of renal failure.

Tx:PUVA, UVB, Hydration, retinoids, renal

transplantation.


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Path-Kyrles

Hyperkeratotic plug, sometimesassociated with follicular orifices.

Parakeratosis and dyskeratosis.

Epidermal hyperplasia

No elastic fibers or collagen fibers withinplug.

Foreign body giant cells in the dermis atperforation site.


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Case 3


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Molloscumcontagiosum


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Case 4


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Acrokeratoelastoidosis of Costa

Inheritance

Clinical

Path: Focal acral hyperkeratosis

Acrokeratoelastoidosis of Costa

Treatment


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Case 5


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Case 6


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Trichomycosis axilaris


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Path

Cavity within the auricular cartilage (without an epithelial lining) that

contains clear fluid.

Fibrous tissue and granulation tissue may be found in the cavity aswell.

The cartilage lining the cavity may show degenerative changes.

However, no inflammation is seen in the cartilage; this is helpful inexcluding ___________, which is frequently in the clinical differential

diagnosis.


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Pseudocyst of the auricle

Endochondral pseudocyst; cystic chondromalacia; intracartilaginous cyst

Clinical

Etiology

Treatment

DDX: Relapsing polychondritis


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Case 8


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Inframammary Fold


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Inframammary Fold


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Pathology

search

Subcorneal

vesiculation filled with

neutrophils and

eosinophils


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Subcorneal Pustular Dermatosis

(Sneddon-Wilkinson) A benign, chronic-relapsing, sterile pustular eruption, usually

involving flexural sites and proximal extremities

Idiopathic. Some cases are thought to be a variant of pustular

psoriasis. Some cases are associated with IgA pemphigus.

Known association with paraproteinemia, monoclonal gammopathy,and lymphoproliferative disorders, such as multiple myeloma

Usually occur in people >40 years of age

Classically, pt are found to have flaccid bullae on the intertriginous

area, especially axilla, inframammary folds, and groin

Studies: SPEP, bacterial culture (usually negative) Tx: dapsone, acitretin, phototherapy, infliximab


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Case 9


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Pathology

Hyperkeratosis,

regular acanthosis,

and papillomatosis

Regular undulatingappearance of the

surface

No parakeratosis

Very little

inflammatory infiltrate


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Acrokeratosis Verruciformis of Hopf

Genodermatosis characterized by multiple warty lesions foundtypically on the dorsal hands and feet

Autosomal dominant

Clinically very similar to keratosis follicularis of Dariers disease.However, no dyskeratosis on the pathology.

Usually present at birth or since childhood Clinically present as multiple skin-colored to red/brown flat, warty

papules on the dorsal hands and feet.

One family found with mutation in ATP2A2 (possibly an allelicmutation to Dariers)

Tx with superficial ablation, e.g. Nd:YAG laser


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Case 10


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Pathology

Duffuse predominantly

granulomatous infiltrate of

foamy and multinucleated

histiocytes.

Touton giant cells

Also can see numerous

neutrophils with focal abscess

formation

Necrobiosis in the center with

degradation of collagen.


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Necrobiotic Xanthogranuloma

Rare, progressive histiocytosis, with close association withparaproteinemia.

Adult onset, with average in the 6th decade.

May begin as a papule/nodule and slowly enlarge.

Clinically with red/orange or yllow color.

Most common site of involvement is the face, particularly periorbitalregion (85%). Can have ophthalmologic complication in 50% ofcases

May involve extracutaneous sites, e.g. heart, lungs, kidney.

Associate with paraproteinemi. 80% with monoclonal gammopathy

on SPEP. Also, multiple myeloma and Hodgkins lymphoma. Anecdotal treatment with chlorambucil, melphalan, localized

radiotherapy, interferon, and plasmapheresis withhydroxychloroquine.


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Case11


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Post. Cervical


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Pathology

Increased elastic fiber in the

papillary dermis

Elastic fiber can be seen as

clumped and disorganized.

Abnormal elastic fiber, alongwith collagen fiber,

inflammatory cells, and cellular

debris are extruded through

the epidermis


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Case12


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Angiokeratoma


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Case13


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Syringoma


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Case14


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Urticaria


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Urticaria


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Case15


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Case16


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P th l


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Pathology

Orthokeratotichyerperkeratosis

Irregular acanthosis

Superficial lymphocytic

infiltrate with a feweosinophils

Melanin pigment isobserved

Follicular plugging and

thinning of stratumspinosum is locallyobserved

search

K t i Li h id Ch i


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Keratosis Lichenoides Chronica

A rare disorder consists of progressive development ofasymptomatic verrucous papules on the limbs and trunk, with lineardistribution. A small reticulated network can be seen

In 75% of cases, an erythematous eruptions can occur on themediofacial area.

Pt may have ulceration, infiltration, and inflammation of the mucous

membrane. Histologically may mimick hypertrophic lichen planus. However,

KLC has linear/reticulated pattern and pruritus is usually absent.

Several reports associate KLC with visceral pathology, such ashepatitis, chronic lymphoid leukemia, or nephropathy.

Topical therapy is ineffective. So far, some cases report

improvement with oral etretinate or isotretinoin, PUVA therapy, orphotodynamic therapy.


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Case17


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Pathology


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Pathology

Abrupt and discreteareas of compactlamellarhyerperkeratosis

Epidermal atrophy

Band-like infiltrate

Hyperkeratosis Lenticularis


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Perstans (Flegels) Originally described as red-brown papules with scales of irregular

outlines measuring 1-5mm in idameter and up to 1mm in depth.

Lesions are primarily located on dorsal feet and lower legs.

Unclear etiology

Ulstrastructurally, there is a loss or decrease of membrane-coatinggranules (Odland bodies), resulting in decreased desquamation ofthe stratum corneum, with subsequent retention hyperkeratosis.

Treatment with Calcipotriol is reported to be effective.

Other successful treatments include topical fluorouracil cream, localexcision, and dermabrasion.


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Case18


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Pathology


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Pathology

Path shows whorls and fascicles of smooth muscle and

spindle-shaped myofibroblastic cells in a fibrous stroma.

The leison is surrounded by vascular channels, which is

characteristic of these tumors, resembling

hemangiopericytoma.

A trichome stain confirms the presence of smooth muscle.

Infantile myofibromatosis


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Infantile myofibromatosis

Benigh fibrous spindle cell tumor This is the most common juvenile fibrous tumor, classified into three forms:

Solitary (>50%)

Multicentric (


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Case19


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Papular acrodermatitis of childhood


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(Gianotti-Crosti) A self-limited childhood exanthem that manifests characteristically in an

acral distribution

In the US, most commonly associated with EBV. The original report waswith HBV.

Affect mostly children from 3months to 15 years of age, with average at 2years of age

In children, both genders are equally affected. However, in adults, reportedcases have been mostly women.

Monomorphous skin-colored papules or papulo-vesicles localizedsymmetrically and acrally over the extensor surfaces of extremities,buttocks and face.

Eruption lasts from 10 days to 6 weeks. Complete resolution often takes

more than 2 months. May have associated constitutional symptoms such as fever,

lymphadenopathy.

cpc kurban iv

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