Self Assessment Cases

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Self assessment courseSelf assessment course

Aleodor Andea, MD Aleodor Andea, MD

Associate Associate Professor of Pathology andProfessor of Pathology and Dermatology Dermatology Director of Director of Dermatopathology Dermatopathology

University of Alabama at BirminghamUniversity of Alabama at Birmingham

Birmingham,Birmingham, AL AL

[email protected]



Cazul 1

� Un pacient de 42 de ani se prezinta cu un

nodul facial asimptomatic. A. Tricofoliculom

B. Tricoadenom

C. Tricoblastom

D. Tricoepiteliom

E.Ch

ist eruptiv cu par velos



Cazul 1


nodul facial asimptomatic.A. Tricofoliculom

B. Tricoadenom

C. Tricoblastom

D. Tricoepiteliom

E.Ch

ist eruptiv cu par velos



TrichofolliculomaTrichofolliculoma

�� Clinical:Clinical:

± ± Flesh colored papules with central pore with Flesh colored papules with central pore with

protruding hairsprotruding hairs

± ± Adults Adults

± ± Face,Face, scalp,neckscalp,neck









Alte optiuni

� Tricoadenom

� Tricoepiteliom

� Tricoblastom



TrichoadenomaTrichoadenoma





TrichoepitheliomaTrichoepithelioma











TrichoblastomaTrichoblastoma

�� Variant of trichoepitheliomaVariant of trichoepithelioma

�� Constituted largely of follicular Constituted largely of follicular

germinative cellsgerminative cells







Vellus hair cyst� Multiple small (eruptive), papules on face, neck, chest, limbs,

axillae� young adults/ children



Eruptive vellus hair cyst



Cazul 2

� Un pacient se prezinta cu un nodul de

culoare rosiatica localizat la baza

incheieturii miini. A. Keratosa seboreica

B. Hidroacanthom

C. Spiradenom ecrin

D. Porom ecrin



Cazul 2

� Un pacient se prezinta cu un nodul de

culoare rosiatica localizat la baza

incheieturii miini. A. Keratosa seboreica

B. Hidroacanthom

C. Spiradenom ecrin

D. Porom ecrin



Eccrine PoromaEccrine Poroma


± ± Adults Adults

± ± solitary, sessilesolitary, sessile ± ± skinskin--colored to red papule / nodulecolored to red papule / nodule

± ± sole or side of the foot; rare in other sitessole or side of the foot; rare in other sites

± ± up to 3cm in diameter up to 3cm in diameter



Eccrine poroma













Alte optiuni

� Hidroacantom

� Keratosa seboreica

� Spiradenom ecrin



Hidroacanthoma SimplexHidroacanthoma Simplex


± ± skin colored to red papule or plaqueskin colored to red papule or plaque

± ± trunk or extremitiestrunk or extremities

± ± elderlyelderly

± ± Often confused with SK, BCC, or SCC Often confused with SK, BCC, or SCC



Hydroacanthoma simplex



Seborr heic Keratosis

� Among the most

common benign

epidermal

proliferationsdeveloping in the

middle-aged and

elderly.



Seborr heic Keratosis

� Hyperkeratosis,papillomatosis

� Pseudohorn cysts



SpiradenomaSpiradenoma

�� Clinical:Clinical: ± ± 0.30.3--5.0 cm.5.0 cm.

± ± dermal painful noduledermal painful nodule ± ± blue colorationblue coloration

± ± young adultsyoung adults

± ± head, neck, upper trunk or upper extremitieshead, neck, upper trunk or upper extremities



spiradenoma







Cazul 3

� O femeie de 67 de ani se prezinta cu o

leziune pe picior de culoare rosiatica, in

relief, cu scuame prezenta de citeva luni. A. Pheohifomicoza

B. Necrobioza lipoidica

C. Xanthogranulom necrobiotic

D.C

romoblastomicozaE. Tinea nigra



Cazul 3


leziune pe picior de culoare rosiatica, in

relief, cu scuame prezenta de citeva luni. A. Pheohifomicoza


C. Xanthogranulom necrobiotic

D. Cromoblastomicoza

E. Tinea nigra



Chromoblastomycosis

� Disease of tropics and subtropics

� Caused by saprophitic pigmented fungi

from plants debris and soil

� Organisms

± Fonseca pedrosoi (Phialospora pedrosoi)

± Phialospora compacta

± Phialospora verucossa

± Cladosporium carrionii



Verrucous nodule or plaque











Alte optiuni

� Pheohifomicoza

� Necrobioza lipoidica

� Xanthogranulom necrobiotic� Tinea nigra



Mycoses� Superficial fillamentous

infections

± Dermatophytoses

� Yeast infections

± Candidosis

± Cryptococcosis

± Pityriasis versicolor

± Pityrosporum folliculitis

� Systemic mycoses

± Blastomycosis

± Coccidioidomycosis

± Paracoccidioidomycosi

± Histoplasmosis

� Dematiaceous fungi

± Chromomycosis

± Phaehyphomycosis

± Sporotrichosis

± Tinea nigra

� Zygomycoses

� Hyalohyphomycoses

± Fusariosis

± Penicilliosis

± Aspergilosis

� Algal infections

± Protothecosis



Phehyphomycosis

� Verrucous, nodular or cystic lesions

� Organisms

± Exophiala jeanselmei

± Wangiella dermatidis









Tinea nigra

� Exophiala werneckii

� Slowly enlarging brown to black macule

� Palms, plantar surface� Mimicks melanocytic lesions







Necrobiotic xanthogranuloma

� Chronic disease

� Sharply demarcated nodules and plaques

� Violaceous to red� Periorbital area









Cazul 4

� O femeie de 30 de ani se prezinta cu un

nodul intradermic dureros pe antebratul

sting. A. Neurofibrom

B. Neurilemom

C. Neurom

D. Leiomiom

E. Leiomiosarcom



Cazul 4


nodul intradermic dureros pe antebratul

sting. A. Neurofibrom

B. Neurilemom

C. Neurom

D. Leiomiom

E. Leiomiosarcom



SCHWANNOMA

(neurilemmoma)� Clinical:

± single, sporadic tumors;

± 20-50 yrs;

± M=F;

± head, neck, limbs (i.e. usually named nerves)



SCHWANNOMA











CM









S-100



Alte optiuni

� Neurofibrom

� Neurom

�Leiomiom

� Leiomiosarcom



� PERIPHERAL NERVE SHEATH TUMORS ± Neuroma

± Palisaded encapsulated neuroma

± Neurofibroma

± Schwannoma ± Granular cell tumor

NEUROMA



NEUROMA

(traumatic neuroma)� Clinical:

± history of injury

± nerve severing event

± a firm, painful nodule



TRAUMATIC NEUROMA



NEUROFIBROMA

� Clinical:

± 90% are solitary, not associated with NF;

± diffuse & plexiform closely associated with NF-1



Neurofibroma vs. Schwannoma



NEUROFIBROMA



NEUROFIBROMA

PLEXIFORM



PLEXIFORM

NEUROFIBROMA

PLEXIFORM



PLEXIFORM

NEUROFIBROMA



Cazul 5


placa dureroasa, eritematoasa si indurata

in zona posterioara a gambei. A. Eritem nodos

B. Erithem indurat

C. Necroza grasa diseminata (Paniculita

pancreatica)

D. Deficienta de alfa-1-antitripsina

E. Paniculita lipomembranoasa



Cazul 5


placa dureroasa, eritematoasa si indurata

in zona posterioara a gambei.A. Eritem nodos

B. Erithem indurat

C. Necroza grasa diseminata (Paniculita

pancreatica)

D. Deficienta de alfa-1-antitripsina

E. Paniculita lipomembranoasa



Erythema nodosum

� Septal panniculitis



Erythema Nodosum

acute painful nodules, anterior lower legs













Classification of panniculitis

� Septal

� Lobular

S t l



Septal

� Erythema Nodosum� Subcutaneous morphea/scleroderma

� Necrobiosis Lipoidica



Lobular

� Erythema Induratum

� Pancreatic Panniculitis

� Alpha 1 anti-trypsin deficiency

� Lupus panniculitis

� Subcutaneous panniculitis-like T cellLymphoma

� Subcutaneous fat necrosis of the newborn &Sclerema neonatorum

� Lipodermatosclerosis



Alte optiuni

� Erithem indurat

� Paniculita pancreatica

� Deficienta de alfa-1-antitripsina� Paniculita lipomembranoasa



Alpha-1-antitrypsin deficiency

� Tender nodules on

trunk or extremities,

� Precipitated by

trauma

� Ulceration and oily

discharge.





Erythema induratum



Tender nodules on calves

y





Lipomembraneous Panniculitis or



Lipomembraneous Panniculitis or

Membranous Lipodystrophy� Venous insufficiency: =Lipodermatosclerosis.

� End result of multiple disease processes:

± EN, Morphea profunda, Lupus, traumatic fat necrosis,insulin lipoatrophy, encapsulated fat necrosis,dermatomyosistis and Bechet¶s disease.

Lipodermatosclerosis



Lipodermatosclerosis

�indurated inverted

champagne bottleappearance







Pancreatic panniculitis



� Painful subcutaneousnodules / plaques

� Thighs, buttocks,

lower trunk & distal

extremities.

� Acute pancreatitis

� Acinic cell pancreatic

carcinoma� Etiology: fat digestion

by of pancreatic

enzymes





C l 6



Cazul 6


nodul polipoid de 3 cm crescut pe o

leziune in placa indurata pe partea

superioara a spatelui. A. Neurilemom

B. Dermatofibrom

C. Dermatofibrosarcom protuberant

D. Fibroxantom atipic

C l 6



Cazul 6


nodul polipoid de 3 cm crescut pe o

leziune in placa indurata pe partea

superioara a spatelui. A. Neurilemom

B. Dermatofibrom

C. Dermatofibrosarcom protuberant


DFSPDFSP



DFSPDFSP

�� FibrohistiocyticFibrohistiocytic tumor tumor

�� Intermediate malignant potential:Intermediate malignant potential:

± ± locally aggressive (11locally aggressive (11--73% local recurrence)73% local recurrence)

± ± but a low rate of metastasis (1but a low rate of metastasis (1--3%) to lung.3%) to lung.

FIBROHISTIOCYTIC TUMORSFIBROHISTIOCYTIC TUMORS



FIBROHISTIOCYTIC TUMORSFIBROHISTIOCYTIC TUMORS

�� BenignBenign ± ± DermatofibromaDermatofibroma

�� IntermediateIntermediate

± ± Atypical Atypical fibroxanthomafibroxanthoma

± ± DermatofibrosaromaDermatofibrosaroma protuberansprotuberans

�� MalignantMalignant

± ± Malignant fibrousMalignant fibrous histiocytomahistiocytoma

Enlarging plaque, sometime with a nodule.Enlarging plaque, sometime with a nodule.

















Alte optiuni



Alte optiuni

� Neurilemom

� Dermatofibrom

� Fibroxantom atipic

� Histiocitom fibros malign

DERMATOFIBROMADERMATOFIBROMA



DERMATOFIBROMADERMATOFIBROMA


± ± 0.50.5 ± ± 1 cm1 cm

± ± LegLeg

± ± puckers when pinc

hedpuckers w

hen pinc

hed

± ± may be pigmented (due to melanin or hemorr hage)may be pigmented (due to melanin or hemorr hage)







Cazul 7



Cazul 7

� Un barbat de 30 de ani a remarcat aparitia

recenta a unui nodul rosu-purpuriu de

bratul drept. Biopsia confirma un

diagnostic de: A. Hemangiom capilar

B. Angiokeratom

C. Sarcom Kaposi

D. Nev intradermic

E. Carcinom cu celule squamouase, tip cu celule

fusiforme

Cazul 7



Cazul 7

� Un barbat de 30 de ani a remarcat aparitia

recenta a unui nodul rosu-purpuriu de

bratul drept. Biopsia confirma un

diagnostic de: A. Hemangiom capilar

B. Angiokeratom

C. Sarcom Kaposi

D. Nev intradermic

E. Carcinom cu celule squamouase, tip cu celule

fusiforme

Kaposi Sarcoma



� Low-grade clonal endothelial proliferation� Variants:

± Classic KS:

� Elderly patients

� Distal extremities

± African KS

� Likely related to chronic immunosuppression

± AIDS-related/ immunosuppression:

� Disseminate multi-organ disease, predilection for mucocutaneous sites, aggressive course

Kaposi Sarcoma











PAS



PAS

CD 31 CD 34



HHV8

Alte optiuni



Alte optiuni

� Hemangiom capilar

� Angiokeratom

� Nev intradermic

� C arcinom cu celule squamouase, tip cu

celule fusiforme

Lobular capillary hemangioma

(Pyogenic granuloma)



(Pyogenic granuloma)



Angiokeratoma





Intradermal nevus



Intradermal nevus





Cazul 8



Ca u 8

� O femeie de 60 de ani se prezinta cu

leziuni eritematoase dureroase pe frunte.

Biopsia confirma un diagnostic de:

A. Foliculita bacterianaB. Zona zoster

C. Lupus eritematos bulos

D. Reactie la intepatura de insecta

E. Pemfigus vulgar

Cazul 8



� O femeie de 60 de ani se prezinta cu

leziuni eritematoase dureroase pe frunte.

Biopsia confirma un diagnostic de:

A. Foliculita bacterianaB. Zona zoster

C. Lupus eritematos bulos

D. Reactie la intepatura de insecta

E. Pemfigus vulgar

Zona Zoster



� Clinical:

± epidemic, acute vesicular eruption with a

febrile illness

± rash develops in successive stages ± pneumonia in immunocompromised









Alte optiuni



p

� Foliculita bacteriana

� Lupus eritematos bulos

� Reactie la intepatura de insecta

� P emfigus vulgar

Staphyloccal folliculitis



Bullous lupus





Insect bite



Insect bite





Case 9



� Care este cel mai probabil diagnostic

bazat pe examenul histopatologic? A. Carcinoma bazocelular

B. TricoblastomC. Porom ecrin

D. Carcinom cu celule Merkel

E. Limfom cutanat cu celule B mari, difuz

Case 9



� Care este cel mai probabil diagnostic

bazat pe examenul histopatologic? A. Carcinoma bazocelular

B. TricoblastomC. Porom ecrin

D. Carcinom cu celule Merkel

E. Limfom cutanat cu celule B mari, difuz

Merkel cell carcinoma



� First reported by Toker in 1972

� Merkel cell origin is not clearly

demonstrated

� Neuroendocrine tumor of the skin

� Clinical:

± Sun-exposed areas of elderly patients

± Nodules 2 cm average diameter

�3 cm ulcerated and crusted nodule

�Clinical Dx: BCC, Amelanotic MM, Metastatic cancer











CK 20 Chromogranin



Synaptophysin

Alte optiuni



� Carcinoma bazocelular

� T ricoblastom

� P orom ecrin

� Limfom cutanat cu celule B mari, difuz

Basal Cell Carcinoma



� Most common cutaneous malignant tumor (70%)

� Head and neck areas

� Males

� Age related

Microscopic



� Basaloid cells with peripheral palisading



Primary cutaneous diffuse large B-

cell lymphoma, leg type



cell lymphoma, leg type

� Definition: is a primary cutaneous large

cell lymphoma with confluent sheets of

centroblasts and immunoblasts wh

ich occurs on the legs



Willemze et al. 2005 105:3768-85





Case 10



� Care este cel mai probabil diagnosticbazat pe examenul histopatologic?

A. Porfirie cutanata tardiva

B. Eritem multiformC. Pemfigoid bulos

D. Pemfigus vulgar

E. Lupus eritematos bulos

Case 10




A. Porfirie cutanata tardiva

B. Eritem multiformC. Pemfigoid bulos

D. Pemfigus vulgar

E. Lupus eritematos bulos

Porphyria cutanea tarda



� Blisters develop

in sun-exposed

areas� Dorsum of the

hands









Alte optiuni



� E ritem multiform

� Pemfigoid bulos

� P emfigus vulgar

� Lupus eritematos bulos

Bullous PemphigoidBullous Pemphigoid



�� Most commonMost common subepidermalsubepidermal blisteringblisteringdisease.disease.

�� Abdomen, groin, flexor surface of arms and Abdomen, groin, flexor surface of arms and

legslegs

�� ElderlyElderly









Case 11



� Cel mai probabil diagnostic bazat exclusivpe examenul histopatologic este:

A. Scleromixedem

B. Fasciita nodularaC. Morfea profunda


E. Histiocitom fibros malign

Case 11




A. Scleromixedem

B. Fasciita nodularaC. Morfea profunda



Nodular FasciitisNodular Fasciitis



�� Upper extremities, trunkUpper extremities, trunk

�� Rapid growthRapid growth

�� Easily confused with a sarcomaEasily confused with a sarcoma

















Alte optiuni



� Scleromixedem

� Morfea profunda



Scleromyxedema



± M-protein Paraproteinemia (85%): IgG

± Onset: Middle aged adults

Scleromyxedema

± Papules plaques &



Papules, plaques &

infiltrative lesions

± Face; Neck upper

Arms, legs.





Deep morphea







Case 12




A. Lichen plan

B. Lichen striat

C. Lichen nitidus

D. Lichen planopilar

Case 12




A. Lichen plan

B. Lichen striat

C. Lichen nitidus


Lichen Planus



� Papules

� Pruritic

� Poligonal

� Purple

� Flexor surface or

wrists, trunk, thighs,

genitalia

Band-like infiltrateEpidermal acanthosis

Compact Hyperkeratosis

Wedge-shaped hypergranulosis



Saw-tooth rete



Vacuolar -interface changes = liquefactive

degeneration



Subepidermal clefting ±Caspary-JosephSpace



Dyskeratotic cells = Civatte bodiesMelanophages



Colloid bodies



Fibrin IgM

Direct immunofluorescence



Case 13




A. Lichen plan

B. Lichen striat

C. Lichen nitidus


Case 13




A. Lichen plan

B. Lichen striat

C. Lichen nitidus


LichenLichen StriatusStriatus



�� Unknown etiologyUnknown etiology�� Linear Linear papular papular

eruption along oneeruption along one

side of body, usuallyside of body, usuallythe length of anthe length of an

extremityextremity





Lichenoid infiltrate on 3-4 dermal papillae

Less dense than in LP

Acanthosis

Spongiosis with exocytosis of lymphocytes

Hyperorthokeratosis and focal parakeratosis



Vacuolar interface changesDyskeratotic cells at all levels

Spongiosis with exocytosis of lymphocytes





Alte optiuni



� Lichen nitidus� Lichen planopilar

Lichen Nitidus



� Multiple small flesh colored papules

� 1-2 mm

� Upper extremities,chest, abdomen,

genitalia

� Children and youngadults

Claw-like rete (ball and claw)Lichenoid infiltrate

Thinned epidermis with dyskeratosis and vacuolar changes





Granulomatous infiltrate



Perieccrine infiltrate

LichenLichen PlanopilarisPlanopilaris



�� A common cause of scarring alopecia A common cause of scarring alopecia�� Keratotic follicular lesions and erythema atKeratotic follicular lesions and erythema at

marginsmargins



Case 14



� Cel mai probabil diagnostic bazat exclusivpe examenul histopatologis este:

A. Lupus eritematos cronic cutanat (discoid)

B. Lichen plan

C. Keratoza lichenoida

D. Eritem multiform

Case 14




A. Lupus eritematos cronic cutanat (discoid)

B. Lichen plan

C. Keratoza lichenoida

D. Eritem multiform

Erythema multiforme



� Pleomorphic eruption� Individual lesions: papule -> vesicle-

>target

� Herpes, drugs, mycoplasma� Minor/ major forms (Stevens-Johnson)





Erythema multiforme/Stevens-Johnson











Alte optiuni

Li h l



� Lichen plan� Lupus eritematos cronic cutanat (discoid)

� Keratoza lichenoida

Lichen Planus-Like Keratosis� Solitary, 3-10 mm



� Scaly, violaceous or

pink

� Arms and chest

� Middle age and

elderly women

� DDX: BCC, SCCIS





Case 15

C l i b bil di ti hi t l i t



� Cel mai probabil diagnostic histologic este: A. Psoriasis

B. Lichen simplex cronicus

C. Lichen sclerotic si atrofic

D. Morfea

E. Fasciita eosinofilica

Case 15

Cel mai probabil diagnostic histologic este:



� Cel mai probabil diagnostic histologic este: A. Psoriasis

B. Lichen simplex cronicus

C. Lichen sclerotic si atrofic

D. Morfea

E. Fasciita eosinofilica









Alte optiuni

� Psoriasis



� P soriasis� Lichen simplex cronicus

� Morfea

� Fasciita eosinofilica

Lichen simplex chronicus

� Scaly thickened



� Scaly t ickenedplaques develop in

response to persistent

rubbing

� Predilection for: ± Nape of neck

± Ulnar border of forearms

± Wrists

± Pretibial region

± Dorsa of feet

± Perianal and genital

region





Eosinophilic Fasciitis (Shulman¶s

Syndrome)

� Sudden onset

� 50% occur after strenuous activity



� 50% occur after strenuous activity.

� Symmetric induration of skin of limbs > trunk

� Characteristic orange-peel appearance.Spares hands.

� Peripheral eosinophilia.

� Malaise, weakness, fever and weight loss

OrangeOrange--peelpeel

appearanceappearance







Layering effect of Layering effect of hyalinizedhyalinized sclerotic collagensclerotic collagenalternating with trapped fat and inflammatoryalternating with trapped fat and inflammatorycells.cells.





Case 16

� Cel mai probabil diagnostic bazat exclusiv




A. Dermatita spongiotica

B. Psoriasis

C. Pitiriasis rubra pilaris

D. Micoza fungoida

Case 16




Cel mai probabil diagnostic bazat exclusivpe examenul histopatologis este:


B. Psoriasis


D. Micoza fungoida

Psoriasis

� Well-circumscribed



Well circumscribederythematous

patches with a

silvery scale

� Extensor surfaces

of the extremities,

sacral region,

scalp, nails















Cazul 17

� Care este cel mai probabil diagnosticb t l hi t t l i ?



Care este cel mai probabil diagnosticbazat pe examenul histopatologic?


B. Psoriasis


D. Micoza fungoida

Cazul 17

� Care este cel mai probabil diagnosticb t l hi t t l i ?



Care este cel mai probabil diagnosticbazat pe examenul histopatologic?


B. Psoriasis


D. Micoza fungoida

Mycosis fungoides

� Small to medium sized T lymphocytes withb if l i



Small to medium sized T lymphocytes with cerebriform nuclei

� Older adults

� Indolent clinical course ± Patch

± Plaque

± Tumor









Pautrier microabscesses



CD

3 + CD

4 +



CD

8

-

CD

7-

Alte optiuni

± Dermatita spongioticaPsoriasis



± P soriasis

± Pitiriasis rubra pilaris

Spongiotic dermatitis

� Allergic contact dermatitis� Dyshidrotic eczema



g� Dyshidrotic eczema

� Nummular dermatitis

� Atopic dermatitis� Seborr heic dermatitis

� Phototoxic dermatitis

� Pityriasis rosea

� Stasis dermatitis

Almost identical histology









Pityriasis rubra pilaris

� Small follicular papules with a central plugP if lli l th



g� Perifollicular erythema

� Islands of sparing

� Palmoplantar keratoderma









Cazul 18




pe examenul histopatologic este: A. Psoriasis

B. Pitiriasis rubra pilar

C. Dermatita spongiotica

D. Scabie

E. Micoza fungoida

Cazul 18




pe examenul histopatologic este: A. Psoriasis

B. Pitiriasis rubra pilar

C. Dermatita spongiotica

D. Scabie

E. Micoza fungoida









Cazul 19

� Alegeti cel mai probabil diagnostic dinoptiunile de mai jos, bazat pe examenul



optiunile de mai jos, bazat pe examenul

histopatologic. A. Pemfigoid bulos

B. Pustuloz exantematic acut generalizat

C. Pemfigus vulgar

D. Boala IgA lineara

E. Pseudoporfirie

Cazul 19

� Alegeti cel mai probabil diagnostic dinoptiunile de mai jos, bazat pe examenul




histopatologic. A. Pemfigoid bulos

B. Pustuloz exantematic acut generalizat

C. Pemfigus vulgar

D. Boala IgA liniara

E. Pseudoporfirie

Linear IgA Bullous DermatosisLinear IgA Bullous Dermatosis

��Ch

ronic Bullous Disease of Ch

ildhood:

Chronic Bullous Disease of

Childhood: ± ± Large, tense bullae arising during the 1Large, tense bullae arising during the 1stst decadedecade



± ± Predilection for perioral and genital regionsPredilection for perioral and genital regions

± ± Usually benign courseUsually benign course

�� Adult linear IgA Bullous Dermatosis Adult linear IgA Bullous Dermatosis ± ± Varied presentations, sometimes annular with lesionsVaried presentations, sometimes annular with lesions

usually involving trunk and limbsusually involving trunk and limbs

± ± Common association with drug Rx (vancomycin)Common association with drug Rx (vancomycin)



Chronic BullousChronic Bullous DermatosisDermatosis of Childhoodof Childhood











IgA



SubepidermalSubepidermal BlistersBlisters

�� Cell Poor Cell Poor ::

± ± EB, EBA, PCTEB, EBA, PCT



�� Cell Rich:Cell Rich:

± ± Lymphocytes:Lymphocytes: EM, Fixed drugEM, Fixed drug

± ± Neutrophils:Neutrophils: DH,DH, Linear IgALinear IgA,, BullousBullous SLESLE

± ± EosinophilsEosinophils:: BP, HG, IP, arthropod, drugBP, HG, IP, arthropod, drug

± ± Mast Cells:Mast Cells: BullousBullous MastocytosisMastocytosis

Alte optiuni

�P

emfigoid bulos� Pustuloz exantematic acut



Pustuloz exantematic acut

generalizat

�P

emfigus vulgar � P seudoporfirie

Acute generalizedexanthematous pustulosis

� Sterile, miliary



pustules on an

erythematous

background� Fever, blood

leukocytosis

� Occurs within hoursafter ingestion of

drugs







DDX of subcorneal pustules

± ± BullousBullous impetigoimpetigo

± ± StaphylococcalStaphylococcalscalded skinscalded skin



± ± PemphigusPemphigus foliaceusfoliaceus

± ± IgAIgA PemphigusPemphigus

± ± Acute generalized Acute generalized

exanthematousexanthematous

pustulosispustulosis

± ± Pustular Pustular psoriasispsoriasis ± ± SubcornealSubcorneal pustular pustular

dermatosisdermatosis

Cazul 20

� Alegeti cel mai probabil diagnostic bazatpe examenul histopatologic din optiunile



de mai jos. A. Pemphigoid bulos

B. Lupus eritematos bulos

C. Pemfigus vulgar

D. Boala Hailey-Hailey

E. Pseudoporfirie

Cazul 20




de mai jos. A. Pemphigoid bulos


C. Pemfigus vulgar

D. Boala Hailey-Hailey

E. Pseudoporfirie

Pemphigus vulgaris

� 80% of pemphigus

cases� Oral blisters then



cutaneous lesions

� Flacid blisters onnormal base

� Trunk, groin, axillae,

scalp, face, presure

points

Pemphigus VulgarisPemphigus Vulgaris

�� Ab Ab directed againstdirected against desmogleindesmoglein 33�� About half of patients also haveAbout half of patients also have AbAb toto



About half of patients also have About half of patients also have Ab Ab toto

desmogleindesmoglein 11









DIF

� IgG in the intercellular

regions of theepidermis lesional



epidermis, lesional

and perilesional

Suprabasilar Blisters

� Pemphigus Vulgaris� Paraneoplastic Pemphigus



� Paraneoplastic Pemphigus

� Acantholytic dyskeratosis

± Hailey-Hailey Disease

± Darier¶s Disease

± Grover¶s Disease

Alte optiuni

A. P emphigoid bulos




C. Boala Hailey-Hailey

D. P seudoporfirie

Hailey-Hailey disease

� Familial benign chronic pemp

higus

� Genodermatosis, AD



� Onset in teens

� ATP2C1 (calcium pump)

Hailey Hailey Disease

Axillary Axillary skin showingskin showing

erythematouserythematous eroded anderoded andcrusted lesionscrusted lesions



c us ed es o sc us ed es o s



�� PartialPartial acantholyisacantholyis

(dilapidated brick wall)(dilapidated brick wall)

�� AcantholyticAcantholytic cellscells

�� No follicular involvement No follicular involvement



Cazul 21


d i j



de mai jos. A. Granulom anular

B. Necrobioza lipoidicaC. Nodul reumatic

D. Infectie cu micobacteria

E. Sarcoidoza

Cazul 21


de mai jos



de mai jos. A. Granulom anular



E. Sarcoidoza

Sarcoid- Clinical Features

� Blacks > Wh

ites� Women > Men



� Cutaneous lesions in <25% of patients

with systemic disease

� If confined to skin, runs an indolent course











Asteroid body

Cazul 22

�C

el mai probabil diagnostic bazat exclusivpe examenul histopatologic este:A Granulom anular



A. Granulom anular


C. Nodul reumaticD. Infectie cu micobacteria

E. Sarcoidoza

Cazul 22


pe examenul histopatologic este:A Granulom anular



A. Granulom anular


C. Nodul reumaticD. Infectie cu micobacteria

E. Sarcoidoza

Granuloma Annulare

� Papules

� Annular or



arciform plaques.

� Commonly onextremities.











Cazul 23

� Alegeti cel mai probabil diagnostic din


histopatologic:



histopatologic: A. Granulom anular



E. Sarcoidoza

Cazul 23



histopatologic:



histopatologic: A. Granulom anular



E. Sarcoidoza

Rheumatoid Nodules

� Subcutaneous nodules, usually in the

vicinity of joints.

� Can persist for years



� Can persist for years







Alte optiuni

� Granulom anular

� Necrobioza lipoidica

Infectie cu micobacteria



� Infectie cu micobacteria

� Sarcoidoza

Necrobiosis Lipoidica

� Predilection for legs

(shins), usually bilateral.

� Red papules, enlarging to

become plaques which



become plaques which

develop atrophic,

depressed centers







Mycobacterial infection

� Less discrete than sarcoidal granulomas

with tendency toward confluence

� More giant cells and often accompanied



More giant cells and often accompanied

by a rim of lymphocytes and plasma cells

� Caseous necrosis sometimes









Cazul 24

� Cel mai probabil diagnostic in acest caz

este: A. Vasculita leucocitoclazica



B. Angiodermita purpurica si pigmentara

C. Purpura actinicaD. Coagulare intravasculara diseminata

Cazul 24


este:A. Vasculita leucocitoclazica



B. Angiodermita purpurica si pigmentara

C. Purpura actinicaD. Coagulare intravasculara diseminata

Leukocytoclastic vasculitis

� Most common vasculitis

� Not a disease but a manifestation of

circulating IC caused by an underlying



c cu a g C caused by a u de y g

disorder

Causes of allergic vascultitis

� Infection: bacteria, mycobacteria, viral

� Drugs: phenacetin, sulfonamies, penicilin,iodides

� Chemicals: insecticides



� Associated diseases:

± SLE, other CTD ± Lymphoma, carcinoma

± RA

± Sjogren sd

± HS purpura

± Cryoglobulinemia













Alte optiuni

� Angiodermita purpurica si pigmentara

� Purpura actinica

� Coagulare intravasculara diseminata



Coagulare intravasculara diseminata

Pigmented purpuric dermatosis

� Group of purpuric

diseases

� Variable



pigmentation

� Lower extremities

� Young adults







Solar purpura







DIC/purpura fulminans

� Fibrin, platelets or mixed thrombi in the

capillaries and venules









Cazul 25


nodul verucos pe fata si o biopsie este

efectuata. Care este pasul urmator in

tratament?



tratament?

A. Revine pentru control din 6 in 6 luni.B. Programat pentru excizie cu margini largi de

siguranta (1-2 cm)

C. Programat pentru excizie conservative a leziunii

D. Revine pentru control din 3 in 3 luni

Cazul 25


nodul verucos pe fata si o biopsie este

efectuata. Care este pasul urmator in

tratament?



tratament?

A. Revine pentru control din 6 in 6 luni.B. Programat pentru excizie cu margini largi de

siguranta (1-2 cm)

C. Programat pentru excizie conservative a leziunii

D. Revine pentru control din 3 in 3 luni

Nevoid melanoma

� Variant of melanoma that is easily

mistaken for a common nevus



Nevoid melanoma

� Definition from McKee: ³A melanoma that

a pathologist diagnosed as nevus and

wished that she/he had not´



� Borderline histology but not borderline

prognosis!

Nevoid melanoma

� Wide age range

� No gender predilection

� Two types



± Verrucous

± Dome shaped

� Clinical:

± Verrucous ±resemble a verruca or SK, if

pigmented a warty nevus

± Dome shaped -more non-specific













Histology ±confusing features

� Dome shaped or

verrucous� Small size

� Symmetric of slightly

asymmetric



asymmetric

� Circumscribed� Limited intraepidermal

component

� Pseudomaturation

Histology -clues

� Confluent or sheetlike

growth pattern



Histology -clues

� Multiple mitotic figures

� Mitoses at the base of the lesion

� Atypical mitoses



� Cytologic atypia

Size

Symmetry

Circumscription

Confluent growth at DEJ

NEVI MELANOMA

Small Large

Symmetric Asymmetric

Well-circumscribed Poorly-circumscribed

No Present

Histologic features



Pagetoid spread

Maturation

Dermal pattern

Cytologic atypia

Mitoses in dermis

Atypical mitoses

Nested Sheetlike

No Prominent

Present Absent

Absent Severe

Absent

AbsentPresentPresent

Size

Symmetry

Circumscription

Confluent growth at DEJ

NEVINEVOID

MELANOMASmall Small

Symmetric Symmetric

Well-circumscribed Well-circumscribed

No No

Histologic features



Pagetoid spread

Maturation

Dermal pattern

Cytologic atypia

Mitoses in dermis

Atypical mitoses

Nested Sheetlike

No No

Present Present

Absent Mild

Absent

AbsentPresentPresent

Cazul 26

� Aceasta leziune este negativa pentru

coloratiile imunohistochimice S100,keratina, p63 si desmina si pozitiva pentru

CD10 Care este diagnosicul cel mai



CD10. Care este diagnosicul cel mai

probabil? A. Melanom desmoplastic

B. Leiomiosarcom

C. Carcinom squamocelular cu celule fuziforme



Cazul 26

� Aceasta leziune este negativa pentru

coloratiile imunohistochimice S100,keratina, p63 si desmina si pozitiva pentru

CD10 Care este diagnosicul cel mai



CD10. Care este diagnosicul cel mai

probabil? A. Melanom desmoplastic

B. Leiomiosarcom

C. Carcinom squamocelular cu celule fuziforme



ATYPIC AL FIBROXANTHOMA ATYPIC AL FIBROXANTHOMA

�� Def: identical toDef: identical to pleomorphicpleomorphic malignant fibrousmalignant fibrous

histiocytomahistiocytoma (i.e. undifferentiated(i.e. undifferentiated pleomorphicpleomorphicsarcoma)sarcoma)

�� Clinical: nodule on sunClinical: nodule on sun--exposed head/neckexposed head/neck











Leziuni dermice pleomorfice cucelule fusiforme



� Carcinom squamocelular cu celule

fuziforme



fuziforme

� Melanom desmoplastic

� Leiomiosarcom

Maligant fibrous histiocytoma

� Aceasi histologie cu fibroxantomul atipic

� Tumora este centrata in tesutul subcutanat

sau muschi



� Prognosticul este foarte diferit:

± AFX -100% supravietuire la 5 ani

± MFH - ~20% supravietuire la 5 ani









Spindle cell SCC









Keratin (CK 903)



Desmoplastic melanoma

� Predilection for elderly males

� Sun-damaged areas� 4% of invasive melanomas

� Infiltrated plaque or amelanotic nevus



p q

� Often unsuspected clinically

± Spindle cells dispersed

into a dense stroma



± Nodular lymphoid

infiltrates

± Associated MIS lentigo

maligna type (only about



g yp ( y

half of cases) Carlson J. C ancer

1995: 76:478-94

± Oval to fusiform nuclei



± Perineural invasion

± Deep infiltration into

dermis and subcutis

± Mitoses (sometimes

scant)



Variable pleomorphism



S100 Mart-1

HMB45



HMB45

LeiomyosarcomaLeiomyosarcoma

�� CutaneousCutaneous leiomyosarcomaleiomyosarcoma

�� DeepDeep leiomyosarcomaleiomyosarcoma

�� Prognosis depends on locationPrognosis depends on location±± Dermal: benignDermal: benign



Dermal: benignDermal: benign

± ± Deeper: more aggressiveDeeper: more aggressive











Desmin



Cazul 27


optiunile de mai jos, bazat pe examenulhistopatologic:

A. Cicatrice

B Ci t i hi t fi



B. Cicatrice hipertrofica

C. Dermatofibrom

D. Morfea

E. Keloid

Cazul 27


optiunile de mai jos, bazat pe examenulhistopatologic:

A. Cicatrice

B Cicatrice hipertrofica



B. Cicatrice hipertrofica

C. Dermatofibrom

D. Morfea

E. Keloid

Morphea

� trunk or extremities

� ivory plaque with violaceous border.





Linear Scleroderma:Linear Scleroderma:

FrontalparietalFrontalparietal involvementinvolvementcalled Coup de Sabrecalled Coup de Sabre

Systemic SclerodermaMulti-system disorder affecting the

subcutaneous tissue, internal organs

and walls of blood vessels.













Scleroderma/morphea Histololgy

Early









Scleroderma/morphea

Histology Late





Alte optiuni

� Cicatrice

� Cicatrice hipertrofica

� Keloid

� Dermatofibrom



Hypertrophic Scars and Keloids

� Exuberant wound healing that either

± remains within the boundaries of original wound =hypetrophic scar

± extends outside the site of injury = keloid.

� More common in blacks





Scar









Cazul 28


este: A. Lupus eritematos bulos

B. Lupus eritematos cronic cutanat (discoid)

C. Lupus eritematos cutanat subacut



D. Lupus eritematos tumid

E. Dermatomiozita

Cazul 28


este: A. Lupus eritematos bulos

B. Lupus eritematos cronic cutanat (discoid)

C. Lupus eritematos cutanat subacut



D. Lupus eritematos tumid

E. Dermatomiozita

Discoid lupus erythematosus

� Female preponderance

� Sharply demarcated, erythematous scalypatches

� Follicular plugging



� Face, neck, scalp, eyelids, lips, oralmucosa, hands

� Atrophy and scaling



Lupus ErythematosusHistology

� Vacuolar interface dermatitis

� Superficial and deep perivascular andperiadnexal dermatitis

� Increased dermal mucin















Direct immunofluorescence

� IgG, IgM, IgA, C3,

fibrin� 50%-90% positivity in

involved skin



� Less positivity on thechest

Alte optiuni

Lupus eritematos bulos

Lupus eritematos cutanat subacut

Lupus eritematos tumid

Dermatomiozita



Subacute cutaneous lupuserythematosus� Annular or

papulosquamous lesions

� Photosensitive areas: face,

neck, upper trunk, extensor

surface of arms



� Non-scarring lesions

� May be associated with

drug ingestion

� In annular formh

isto differs only in degreefrom DLE

± More basal vacuolar changes

± More Chivatte bodies

± More epidermal atrophy

± More superficial dermal edema

± More superficial dermal mucin

L h k t i



± Less hyperkeratosis ± Less follicular plugging

± Less basement membrane thickening

± Less dense dermal infiltrate, sometimesconfined to upper dermis

� Papulosquamous form: identical with DLE







DIF

� DIF from lesional skin positive in 60%

� Band is not as thick an in DLE

� IgG dust like particles described in the

cytoplasm of basal cells



Tumid lupus

� Only dermal changes









Dermatomyositis

� Polymyositis

� Skin involvement ± Violaceous, erythematous, scaly

± Face, shoulders, extensor surface of the forearmsand thighs

± Poikiloderma



± Pruritus

± Heliotrope rash

± Gottron¶s papules

� 10-20% with underlying malignancy

Histology

� Mild vacuolar-interface dermatitis

� Rare apoptotic cells

� Sparse dermal infiltrate, often superficial

� Abundant dermal mucin









DIF

� Usually negative

� C5b is positive perivascular



Connective tissue diseaseDLE SCLE Tumid lupus Dermatomyositis



� Prominent epidermalchanges

� No follicular plugging� No basement

membrane thickening� Sparse dermal infiltrate,

confined to upper dermis

� No epidermal changes� Superficial and deep

dermal infiltrate

� Prominent dermal

mucin

� Follicular plugging� Basement membrane

thickening

� Superficial and deep

dermal infiltrate� Dermal mucin

� Very subtle epidermalchanges

� Very sparse dermal

infiltrate

� Prominent dermalmucin

Cazul 29


nodul pe fata si o biopsie este efectuata.Care este pasul urmator in tratament? A. Informeaza pacientul si parintii despre natura benigna a acestei

leziuni si programeaza pacientul la controala regulate.

B Programeaza pentru excizie cu margini largi de siguranta



B. Programeaza pentru excizie cu margini largi de siguranta.

C. Trimite specimenul pentru teste de "fluorescence in situ

hybridization" pentru a stabili natura maligna sau benigna a

tumorii.

D. Trimite specimenul pentru teste de "comparative genomic


tumorii.

Cazul 29


nodul pe fata si o biopsie este efectuata.Care este pasul urmator in tratament? A. Informeaza pacientul si parintii despre natura benigna a

acestei leziuni si programeaza pacientul la controala regulate.

B Programeaza pentru excizie cu margini largi de siguranta



B. Programeaza pentru excizie cu margini largi de siguranta.

C. Trimite specimenul pentru teste de "fluorescence in situ


tumorii.

D. Trimite specimenul pentru teste de "comparative genomic


tumorii.

Spitz¶s NevusSpitz¶s Nevus

�� Red colored papule, usually < 1 cm inRed colored papule, usually < 1 cm in

diameter diameter �� Children or young adultsChildren or young adults

�� Common on lower extremities, but canCommon on lower extremities, but can

occur at almost any body siteoccur at almost any body site



occur at almost any body siteoccur at almost any body site



S pitz¶s nevus

















Spitz¶s Nevus (micro)

� Symmetry

� Spindled or epithelioid cells� Maturation with increasing depth of dermal

component

� May have some pagetoid spread of



May have some pagetoid spread of solitary melanocytes (extensive in

pagetoid Spitz)

Spitz¶s Nevus (micro 2)

� Circumscribed nests which separate from

adjacent keratinocytes (clefting)� Multinucleate cells

� Little pleomorphism

� No deep atypical mitoses



No deep atypical mitoses� Dermal edema/telangiectasia

Cazul 30


este: A. Angioleiomiom

B. Leiomiosarcom

C

.C

arcinom squamocelularD D t fib



. arcinom squamocelular D. Dermatofibrom

E. Leiomiom pilar

Cazul 30


este: A. Angioleiomiom

B. Leiomiosarcom

C. Carcinom squamocelular

D D t fib



D. Dermatofibrom

E. Leiomiom pilar

PILAR LEIOMYOMAPILAR LEIOMYOMA

�� Erector Erector pilipili musclemuscle

�� Multiple lesionsMultiple lesions�� Familial syndrome associated with renalFamilial syndrome associated with renal

carcinoma and uterinecarcinoma and uterine leiomyomasleiomyomas











Alte optiuni

Angioleiomiom

LeiomiosarcomC arcinom squamocelular

Dermatofibrom



ANGIOLEIOMYOMAS ANGIOLEIOMYOMAS


± ± painfulpainful subQsubQ tumorstumors ± ± WomenWomen

± ± 4040 ± ± 60 yrs60 yrs

± ± Lower legLower leg



gg







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