cpc kurban i

8/8/2019 CPC Kurban I

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CPC I

1) Dermatomyositis

2)Erythema Multifome

3) Psuedoxanthoma

Elasticum 4) Amyloidosis

5) Gestational Pemphigus

6) Grover,s Disease

7) Pediculosis capitis

8) Steatocystoma

Multiplex

9)Lupus Rythematosis

10) Keratoacanthoma

11) Leukocytoclastic

Vasculitis 12) Bullous Pemphigoid

13) Porokeratosis

14) Hailey Hailey Disease



Case 1



Dermatomyositis: Clinical Proximal extensor myopathy

Adult and Juvenille

Females more common in adult

25% of adult with malignancy

Poikiloderma (violaceous) Heliotrope sign

Photodistribution, nailfoldtelangiectasia

Grotton¶s papules R/o CTD overlap

Anti-Jo-1 20%



Dermatomyositis: Histology

CPC! Non-diagnostic

Similar to SLE

Vacuolar degeneration of basallayer

Epidermal atrophy withhyperkeratosis and blunting of rete ridges

Thickening of the basementmembrane

Edema and increased dermalmucin

Sparse dermal/perivascular mononuclear cell infiltrate



Epidermal atrophy

Basal vacuolar

degeneration



Edema

Dermal mucin

Sparse perivascular infiltrate



Case 2



Erythema Multiforme: Clinical Erythematous, symmetrical, fixed

papules -> targets

HSV, Orf, Histoplasma, ?EBV

50% with clinical herpes labialis(genital rare)

Appear within 24-72h; ~100 lesions Itch or burning of lesions

Dorsal hands, forearms > palms,neck, face, trunk

Oral lesions (lips buccal tongue)

³Target´ lesions have dusky center and erythematous halo (2 or 3rings)

Resolves 2 weeks



Erythema Multiforme: Histology

Vacuolar degeneration of basal layer with dermaledema and sub-epidermalblister

Scattered or groupednecrotic keratinocytes

³Satellitosis´: necrotickeratinocytes surrounded bylymphocytes

Superficial perivascular lymphocytic infiltrate

Extravasated dermal RBCs



Vacuolization, Demal edemaVacuolization, Demal edema



Grouped necrotic keratinocytesGrouped necrotic keratinocytes



SatellitosisSatellitosis



Case 3



Pseudoxanthoma Elasticum: Clinical

Skin, eyes, cardiovascular

Yellowish ( pseudoxanthomatous)

papules -> plaques Flexural; lax and inelastic skin

ABCC6 ± transmembrane transport

protein (present in skin, eyes, arteries)



Inner lip lesions are mostcommon mucosal finding

Angiod Streaks

breaks in the calcified elastic

lamina of Bruch's membrane Subsequent hemorrhage

Association with B-

thalassemia Early cardiovascular disease

Hematopoietic mases



PXE: Histology

Distorted and fragmentedelastic fibers in the reticular dermis

Calcium deposits on elasticfibers may be visible as³purple clumps´

Silver stains for elastin(Verhoeff van Giesson) and

calcium (von Kossa) tovisualize alterations in theelastic fibers



Case 4



Macular Amyloidosis: Clinical

Cutaneous Amyloidosis:macular, lichen, nodular

Brown stippled macules;³salt and pepper´

Moderately pruritic

Interscapular region(classic); thighs, shins,arms, breasts, buttocks

Chronic Result of itching notalgia

paresthetica?



Primary Cutaneous Amyloidosis: ClinicalMacular

Brown stippled macules; ³salt andpepper´; moderate pruritus

Interscapular region (classic); thighs,shins, arms, breasts, buttocks

Result of itching of notalgia paresthetica?

Lichen

persistent pruritic papules/plaquesextensor extremities; shins, thighs,forearms

skin-colored to hyperpigmented papules

Moderately pruritic

Unilateral; can become bilateral

Nodular Very rare

Can progress to systemic (7%)

Paraproteinemia (40%)



Macular Amyloidosis: Histology

Amyloid deposits inthe papillary (andupper) dermis

Melanophages(pigment incontinence

Sparse perivascular lymphistiocytic

infiltrate Stains with congo red,

crystal violet

+birefringence

Nodular AmyloidNodular Amyloid



Primary Cutaneous Amyloidosis: Histology

Amyloid deposits in thepapillary (and upper)dermis

Melanophages (pigment

incontinence) Sparse perivascular

lymphistiocytic infiltrate

Stains with congo red,crystal violet

+birefringence

Nodular AmyloidNodular Amyloid



+ Birefringence+ Birefringence

Congo RedCongo Red

Crystal VioletCrystal Violet



Case 5



C3



Gestational Pemphigoid(= Herpes Gestationis)

(BP180; collagen XVII)

Direct IF demonstrating deposits of C3 along the dermo-epidermal junction

Papillary dermal edema andperivascular lymphocytic and

eosinophilic infiltrate



Gestational Pemphigoid



Gestational Pemphigoid

(= Herpes Gestationis) Sudden onset of intensely pruritic vesiculo-bullous eruption

developing during 2nd or 3rd trimester or the immediatepostpartum period

starts on abdomen in half the cases

Lesions rapidly progress to generalized bullous eruption sparingthe face, mucous membranes, palms and soles

Spontaneous resolution following delivery



Case 6



T i t th l ti d t i



Transient acantholytic dermatosis(Grover's Disease)

four histologic patterns, resembling Darier's disease, Hailey-Hailey

disease, pemphigus and a spongiotic pattern

T i t th l ti d t i



Transient acantholytic dermatosis(Grover's Disease)

intensely pruritic, polymorphic, papulovesicular

dermatitis primarily of the trunk

chiefly affectsCaucasian men after the age of 40 years

transient version lasts for weeks to months

chronic variant follows a chronic, relapsing course over

years

exacerbating factors include friction, heat, sweating andsunlight exposure



Case 7



di l i i i



Pediculosis capitis(Head lice)

Skin findings (excoriations, erythema, pyoderma, and

scaliness) most commonly behind the ears and posterior

neck

Classic symptom is intense pruritus

Diagnosis is made by the identification of nits and/oradult lice in the scalp hair

Viable eggs are usually tan to brown in color. Hatchedeggs are clear to white and have to be differentiatedfrom hair casts, dandruff, hair gel, and white piedra



Case 8



Steatocystoma: Histology




Thin walled dermal cyst

Stratified squamousepithelium

Fine scant keratinousdebris

Sebaceous glandsadjacent to or arising

from cyst wall Irregular eosinophiliccuticle lines the cyst




Thin walled cyst

Sebaceous

glands adjacent

to cyst wall





stratified squamousepithelium

irregular eosinophiliccuticle lines the cyst

Fine scant keratinousdebris



Steatocystoma-path



Steatocystoma-path

Cyst with sparse keratin or hair shafts, but

frequently appears empty.

Wall of ruggated squamous epithelium

with a wrinkled eosinophilic refractile

cuticle of keratin-no granular layer.

Sebaceous glands within or adacent to the

cyst wall opening into cyst.

Steatocystoma: Clinical



Steatocystoma: Clinical

Steatocystoma Simplex

Steatocystoma Multiplex ± Autosomal Dominant; Keratin

17

± Association with Eruptive Vellus

Hair Cysts; PachyonychiaCongenita (also K17)

Dermal cysts

Drain oily fluid when

punctured Chest, axilla, groin

Asymptomatic/Cosmeticconcerns



Case 9



³Lupus Band´



p

granular Ig + C3 at the DEJ

Photo: Bhawan, Byers, & Sau. Dermatopathology Interactive Atlas.



Summary: Histology of LE



Summary: Histology of LE

Epidermal atrophy and hyperkeratosis,

follicular plugging

Vacuolar interface dermatitis

Superficial and deep perivascular

lymphocytic infiltrate

Periadnexal infiltrate

Thickened basement membrane

Dermal mucin

Andrews Classification



Andrews Classification

Chronic LE ± DLE ± localized

± DLE ± generalized

± Hypertrophic LE

± LE-LP overlap

± Tumid LE

± Lupus Panniculitis

± Chilblain LE

Subacute LE ± Annular

± Papulosquamous

± Drug-induced

± Neonatal

± Complement deficiency

Acute LE ± Malar rash & related

± Bullous LE

DLE Clinical Features



DLE ± Clinical Features

Most common form of chronic cutaneouslupus

Erythematouspapules & plaques

Scale

Atrophy

Scarring Dyspigmentation

Follicular Plugging

S d DLE



Sun and DLE

DLE is photosensitive, but not limited to a

photodistribution

± Experimentally reproduced by UVB and UVA

± Not as strongly photosensitive as other forms of

cutaneous lupus

± May occur on photoprotected skin (including sites of

prediliction ± scalp and conchal bowls)

DLE Scalp involvement



DLE ± Scalp involvement

Involved in 60%

Scarring alopecia

may result

Mucosal DLE



Mucosal DLE

Up to 24% in one

study

Mouth (buccal

mucosa-mostcommon)

Nose

Eye

Genitals

DLE Prognosis



DLE - Prognosis

Occurs in 20% of

patients with SLE

< 5-10% patients

presenting with DLEwill progress to SLE

*Caution: children

with DLE may have a

higher chance of progression

DLE



all patients

localized

Fewer manifestations of SLE

Less often ANA+

Greater chance of spontaneous

remission (~50%)

Higher chance of SLE

More often ANA+, leukopenia, ESR

Less chance of remission (<10%)

generalized

Lab Workup: Other serologies



Lab Workup: Other serologies

ANA

dsDNA, Sm

± Markers for SLE

Ro (SSA), La (SSB)

± Markers for SCLE, photosenstivity, Sjogren¶s

U1RNP

± MCTD, SLE

Treatment of Dermatologic LE



Treatment of Dermatologic LE

Foundation:

Sunscreen & Smoking Cessation,

Eliminate drug precipitants

Topicals

Antimalarials

Other

Treatment Topicals



Treatment - Topicals

Topical steroids as determined by lesions

thickness and site

ILK

Immunomodulators

± Tacrolimus, pimecrolimus

Treatment First Line Systemics



Treatment ± First Line Systemics

Hydroxychloroquine

± 200mg po qd to bid

Chloroquine ± 250mg po qd

Quinacrine

± 100mg po qd in addition to HCQ or CQ

± Must be compounded

Antimalarial Resistant Disease



Antimalarial-Resistant Disease

Review treatment foundations: ± sun protection

± smoking cessation

Immunosuppressive agents ± Methotrexate

± Azathioprine

± Mycophenolate mofetil

Oral retinoids ± Hypertrophic or palmoplantar disease

Thalidomide

Path: LE



Path: LE

Follicular plugging

Epidermal atrophy/hyperplasia

Colloid bodies

Liquefaction degeneration of basal layer Thickened basement membrane

Increased Mucin

Perivascular and peradnexal lymphocitic

infiltrate Immunofluorescence-granular IgM, IgG and

complement at the DEJ



Case 10



keratoacanthoma

KA-clinical



KA clinical

Common volcano nodule or plaque

erupting over several weeks.

Capable of spontaneous resolution.

Subtype of SCC-rarely metastasizes.

SCC-ka type, the usual designation by

pathologists.

Path: KA



Path: KA

Keratin filled crater

Pale, eosinophilic, glassy well

differentiated epithelial proliferation with

squamous eddies or keraitn pearls

Cytologic atypia no more than mild

Occasional perivascular or lichenoid

infiltrate



Case 11



Leukocytoclastic vasculitis

Clinical LCV



Clinical LCV

Majority follow acute infection or exposure tonew medication.

Palpable purpura is the hallmark of the disease.

Annular, vesicular, bullous.

Affect mainly dependent areas-legs.

Ck-CBC, ASO, HBV, HCV, ANA. Consider SPEP, UPEP, complement, ANCA¶s, andcryoglobulins.

Path-LCV



Path LCV

Epidermis-normal to necrotic

Vasculitis of small venules, predominantly

neutrophils

Nuclear dust often.

RBC extravasation.

Occ. Thrombi.

Fibrin deposits may be seen.



Case 12



Bullous pemphigoid

Clinical-BP



Large tense blisters-groin, axilla, trunk, thighs,flexor surfaces

Bullae often occur on an urticarial base.

Age of onset-65-75 years.

Circulating antibodies to BPAg1 (230kda) andBPAg2 (180kda).

Tx: topical steroid, systemic steroids, other immunesuppression, tetracyclines plusniacinamide.

Path-BP



Eosinophilic spongiosis

Subepidermal blister

Viable roof over blisters-non necrotic

Immunofluorescence-linear IgG (IgG4) in

the basement membrane zone



Case 13



Porokeratosis

Clinical: porokeratosis



Types: ± Mibelli-usually solitary.

± DSAP-sun-exposed areas.

± Linear porokeratosis-highest risk of developing cutaneous malignancies.

± Palmaris, plantaris et disseminata-first onhands and feet, then rest of body.

± Punctate porokeratosis-limited to hands andfeet.

Path-porokeratosis



Coronoid lamella-a column of

parakeratosis under which there is

hypogranulosis.

Epidermis centrally may be normal. Perivascular or lichenoid lymphocytes

occasionally under coronoid lamella.



Case 14



Benign Familial Pemphigus

(Hailey-Hailey): Clinical



Autosomal Dominant, rare

ATP2C1 ± Mutation in Calcium ATPase, Golgi apparatus

Loss of cellular adhesion, stratumspinosum

Onset ages 10-50 years

Flaccid blisters, erosive plaques

Intertriginous and Neck

± Axillae, groin

Painful; Malodorous vegetations

Flares and remissions

Heals with scarring

SCCSCC

HSVHSV

Kaposi¶sKaposi¶s

varicelliformvaricelliform

eruptionseruptions

Benign Familial Pemphigus:

Histology



Generalizedacantholysis leads to

"dilapidated brick wall³

³Villi´: dermal papillaelined by basal cells

Rare ³corps ronds´

Negative directimmunofluorescence



Benign Familial Pemphigus:

Histology



³Dilapidated brick

wall"

Acanthosis

+/- parakeratosis

(later lesions)

cpc kurban i

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