-clinical correlation of
TRANSCRIPT
Clinical correlation of Clinical correlation of inflammatory skin inflammatory skin
lesionslesionsMary Jo Robinson, D.O.Mary Jo Robinson, D.O.
UMDNJ-SOMUMDNJ-SOMOct.3, 2007Oct.3, 2007
5 clinical keys to diagnosis5 clinical keys to diagnosis
• Type of lesions-very very important
• Region of body affected- also important
• Distribution of lesions-not that important
• Color-somewhat important
• Configuration/shape-less important
• Summary- pertinent history and complete skin examination is best key.
8 clinical diagnostic groups8 clinical diagnostic groups• Pustular-pustule• Vesicular bullous-vesicles or bullae• Papular mini-vesicular- vesicles less than
2 mm• Papulosquamous-scales• Papulonodular-non red nodule, no scale
or crust• Vascular dominant-red macule, papule or
nodule w/o epidermal changes• Pigmentary-brown, black, white or yellow
lesions• Tumor –large papules or nodules
>2000 clinical dermatologic diseases>2000 clinical dermatologic diseases
• Many with variety of presentations and tendency to change during the chronology of disorder
• Thus one disease can have a myriad of radically different presentations, ie. Lupus
100 most common dermatologic dzs100 most common dermatologic dzs
• Represent 85% of problems seen by practicing dermatologist
• But most do not need bx-acne, rosacea, seborrheic dermatitis , psoriasis, tinea corporis….diagnosed clinically w/o bx
• But atypical presentations of above and a subgroup of patients called “GOK” tend to get biopsies
So how does the pathologist So how does the pathologist correlate these clinical impressionscorrelate these clinical impressions??• Given the clinical impression
(macroscopic)try to subclassify into microscopic appearance
• Such as clinician describes scales, slide shows alternating parakeratosis and orthokeratosis= PRP
• Clinician describes pustules, slide shows collections of neutrophils in stratum corneum= pustular psoriasis
PustularPustular
• Microscopic• Collections of
neutrophils, eosinophils or lymphocytes in epidermis, follicle, sebaceous glands, etc
• Macroscopic• Pustular dermatosis
- impetigo-
folliculitis - acne
- Rosacea
- candidiasis
- Pustular
psoriasis
- Sweet’s
Generalized and intense Generalized and intense erythematous rash with pustuleserythematous rash with pustules
Palmoplantar pustulesPalmoplantar pustules
Macropustule Macropustule
Pustular Pustular psoriasis psoriasis
• Biopsy to exclude fungus, pustular drug, impetigo, superficial pemphigus, impetigo herpetiformis
• Should culture
Histology not specific, CC Histology not specific, CC necessary necessary
• Pustular psoriasis cannot be distinguished on histology from - acrodermatitis continua(pustular eruption on one of more fingers) - Reiter’s disease(arthritis, conjunctivitis, balanitis, pustular dermatosis)
- impetigo herpetiformis( pustular dermatosis of pregnancy assoc w/ hypocalcemia)
Papular minivesicularPapular minivesicular
• Microscopic• Epidermal
spongiosis w/ scale crust
• Macroscopic• Papular minivesicular
dermatitis- contact dermatitis- atopic dermatitis- scabies- dermatophytosis
- stasis dermatitis
- Grover’s
- Hailey Hailey
- Mucha-Habermann- Dermatitis
herpetiformis
Intensely pruritic rash of Intensely pruritic rash of elbows, knees, backelbows, knees, back
Early
Late
Floor of Floor of blisterblister
DIF granular IgA in dermal DIF granular IgA in dermal papillaepapillae
D/Dx DH via DIFD/Dx DH via DIF
• DIF DH- granular IgA
• Linear IgA dermatosis-linear IgAalso lack of gluten sensitive
enteropathy, no association w/HLA-B8 &DR-3 antigens, less response to dapsone tx
• Bullous pemphigoid-linear IgG
Dermatitis herpetiformisDermatitis herpetiformis
• clinically Grover’s, atopic dermatitis, scabies & Pityriasis lichenoides are always part of differential.
• Commonly due to intense pruritus of DH, a bx will come in as r/o scabies or atopic dermatitis
• Biopsy may often show only erosions or scale crusts
• Clinician should biopsy nonexcoriated, non-vesicular erythematous plaque or papule for best diagnosis
• d/dx of neutrophils in papillary dermis includes Bullous eruption of LE, mucous membrane pemphigoid, flea bites, leukocytoclastic vasculitis, linear IgA dermatosis.
• Clinical response to dapsone can be used as confirmatory test
• 2/3 pts have asymptomatic celiac –like disease on jejunal bx & endomysial antibodies
VesiculobullousVesiculobullous
• Microscopic• Epidermal or
subepidermal vesicle(<10mm)
• Bullae(>10 mm)
• Macroscopic• Burn• Erythema
multiforme• Pemphigus vulgaris• Dermatitis
herpetiformis• Herpes simplex• Bullous pemphigoid• Contact dermatitis• Fixed drug
eruptions
Firm bullae w/ erosions, Firm bullae w/ erosions, crusts, papules and whealscrusts, papules and wheals
• groin, axillae, forearms, oral
• Intertriginous to generalized
• Pink to red
Histopathology Histopathology
DIF linear IgGDIF Salt split skin IgG
Type IV collagen present along Type IV collagen present along base of blisterbase of blister
EBA - collagen along roof of blister
Bullous pemphigoidBullous pemphigoid
• Histology- epidermal spongiosis
• Rete ridge pattern preserved
• Subepidermal blister
• Early bullae will have many eosinophils
• Clinician should biopsy erythematous skin with early bullae
• Perilesional skin should be biopsied for DIF
Histologic d/dxHistologic d/dx
• Spongiotic arthropod assault
• Herpes gestationis• Porphyria cutanea
tarda• Erythema
multiforme• Dermatitis
herpetiformis
• Linear IgA bullous dermatosis
• Epidermolysis bullosa
• Bullous lichen planus
• Bullous drug eruption
• Bullous LE
Pruritic vesiculopustular disease Pruritic vesiculopustular disease of trunk and proximal extremitiesof trunk and proximal extremities
Note the vesicles start clear and then fill with white creamy pus, then erosions form
HistologyHistology
• Subcorneal pustules to bullae with neutrophilic infiltrate w/ sparse to moderate numbers of eosinophils
IgA pemphigusIgA pemphigus
• Presented case is SPD type (subcorneal pustular dermatosis)
• Resemble SPD/Sneddon- Wilkinson, pemphigus foliaceous
• Second clinical type is IEN type (interepidermal neutrophilic bullae)
• Annular erythema with peripheral vesicular eruption
IEN typeIEN type
Intraepidermal pustules of neutrophils and some eosinophils
IgA DIFIgA DIF• Intercellular IgA deposits• SPD form shows antibodies to
desmocollin-1• Some cases of IEN form antibodies to
desmoglein1• Both have serum antibodies to IgA
epithelial cell surfaces by IFA• Differentiates from Pemphigus foliaceus
which has IgG epithelial cell surface
Papulosquamous Papulosquamous
• Microscopic• Confluent
orthokeratosis, parakeratosis or alternating OK/PK sometimes with minimal serum
• Macroscopic• Lichen planus• Psoriasis • Lupus erythematosus• Pityriasis rosea• Seborrheic dermatitis• Solar keratosis• Scaly dermatophytosis• Ichthyosis• Mycosis fungoides• Pityriasis rubra pilaris
ClinicalClinical
• Scaly • Large scale(flakes)
> 1mm size= large scale dz(psoriasiform)
• Small scales< 1 mm size=small scale dz(pityriasis)
• Shiny compact scalescompact scale
dz (lichenoid)
HistopathologyHistopathology
• Large scale usually psoriasiform
may be spongiotic, interface vacuolar or interface
• Small scale usually spongiotic, but may be interface vacuolar or interface
• Compact scale usually interface but spongiotic or interface vacuolar may be seen
Plaques with overlying scale Plaques with overlying scale and erythematous borders and erythematous borders
IgG, complement band at base of epidermis on IF
Lupus erythematosusLupus erythematosus
• Annular to plaques• Photosensitive distribution• Scales• Atrophy/scarring(late)• Follicular plugging(late)• Dermal edema &/or mucin deposits• Telangiectases• Lichenoid to sup & deep pv lymph
infiltrate
Follicular pluggingFollicular plugging
Polymorphous autoimmune Polymorphous autoimmune diseasedisease
• primary changes at epidermal dermal interface including hair follicle
• Vacuolar change
• BM thickening –chronic cases PAS
• Compact Orthokeratosis
• Loss of rete ridges late
• Necrotic keratinocytes occasionally
Stage of disease affects Stage of disease affects histologyhistology
• Early – maculopapular more superficial sparse inflammation, lichenoid and may be neutrophilic
• Later smudging subtle to progress to more obvious vacuolar
• Then plaque stage shows dermal mucin and adnexal inflammation
• Late- scarring, atrophy, melanophages
Histologic D/DXHistologic D/DX• Seborrheic dermatitis- early forms w/
pyknotic neutrophils @ follicular ostia, later chronic forms more spongiosis
• Actinic keratosis- interface changes due to solar damage, check the follicular ostia, no interface change there? It is not DLE
• Lichen planus• PMLE- no atrophy, no foll. plugging, no
fibrosis• Rosacea –central face especially, but more
vascular than LE & assoc clinically w/ flushing, perifollicular infl, no mucin
Flat topped violaceous Flat topped violaceous papules w/ shiny scalepapules w/ shiny scale
Histology Histology
Lichen planusLichen planus• Compact orthokeratosis
• If rubbed, parakeratosis &/or hypertrophic
• Acanthosis with jagged sawtoothed rete ridges
• Focal wedge-shaped hypergranulosis that is more prominent next to acrosyringium
• Colloid bodies- more prominent in lower epidermis
Dermis in LPDermis in LP
• lichenoid lymphohistiocytic infiltrate fills papillary dermis, is dense and close to base of epidermis
• Coarse collagen bundles
• No mucin, no edema
D/Dx of lichenoid lesionsD/Dx of lichenoid lesions• Lichenoid photodermatitis-sup & deep w/
spongiosis• Lichenoid solar keratosis-atypical budding
w/ alternating ok/pk• Lichenoid LE-vacuolar change prominent,
dermal mucin, may be tough call• Lichen aureus-pigmented purpuric
dermatosis, hemosiderin macrophages• MF- epidermotropism, lamellar fibrosis• LPLK- usually solitary, peripheral SK/SL
Papulonodular Papulonodular
• Microscopic• Scale crust and
spongiosis are ABSENT
• Acanthosis, dermal deposits or inflammation are PRESENT
• Macroscopic• Prurigo nodularis• Granuloma annulare• Amyloidosis• Sarcoid • Acne• Follicular cysts• Arthropod assaults• Lymphocytoma cutis• Polyarteritis nodosa
Grouped 1-2 mm flesh colored to Grouped 1-2 mm flesh colored to pink papules in arcuate pink papules in arcuate
distribution on extremitiesdistribution on extremities
Clinical d/dxClinical d/dx• Sarcoid
• Lichen planus
• Urticaria pigmentosa
• Papular mucinosis
• Tinea corporis
• Necrobiosis lipoidica
• Rheumatoid nodule
• Foreign body
• Granulomatous rosacea
Granuloma annulare Histology Granuloma annulare Histology
D/Dx palisading granulomaD/Dx palisading granuloma
• Granuloma annulare• Rheumatoid nodules• Necrobiosis
lipoidica• Churg-Strauss
granulomatosis• Lupus miliaris
disseminatus facei
• Bovine collagen injections
• Actinic granuloma• Foreign body
granuloma• Infectious
granuloma
Vascular dominantVascular dominant• Microscopic• Proliferations of
blood vessels • Or perivascular
inflammatory infiltrate w/ no epidermal changes
• Redness, macular or papular erythema
• Wheals • purpura
• Macroscopic• Urticarial vasculitis• Macular papular
erythema• Vasculitis• Gyrate erythema• Schamberg’s• telangiectasia
Hemangiomas• Kaposi’s
Purpura as a clinical cluePurpura as a clinical clue• Non-purpuric
complete blanching with application of pressure, no extravasated rbc’s in dermis
• Sunburn, urticaria, macular papular erythema, erythema nodosum, fixed drug, gyrate erythemas
• Purpuricresidual erythema
persists with pressureecchymosis and
petechiae • Leukocytoclastic
vasculitis, septic vasculitis, pigmented purpura, dysproteinemic purpura, thrombocytopenic purpura
Bright red to brown red purpuric Bright red to brown red purpuric papules lower extremitiespapules lower extremities
Histology Histology
Endothelial cell swelling, angiocentric neutrophilic inflammation with nuclear dust, fibrin in vessel walls, extravasated erythrocytes
Henoch-Schoenlein purpura-IgA Henoch-Schoenlein purpura-IgA mediated in kids(beta strept)mediated in kids(beta strept)
PigmentaryPigmentary• Microscopic• Pigment containing
macrophages in upper dermis
• decreased or increased number of melanocytes in epidermis
• Of dermis with collections of histiocytic foams cells in dermis fibrosis
• Macroscopic • Lichen sclerosus• Vitiligo• Lentigo• Xanthelasma• Lupus erythematosus• Morphea• Tinea versicolor• Melanocytic nevus• Basal cell carcinoma,
pigmented• Seborrheic keratosis• Dermatofibroma
White to yellow linear plaques White to yellow linear plaques with violaceus to erythematous with violaceus to erythematous
halohalo
Histology linear morpheaHistology linear morphea
TumorTumor
• Microscopic• neoplastic
proliferation of cells• epidermal• Dermal• Melanocytic• other
• Macroscopic • Mycosis fungoides• Kaposi sarcoma• Melanoma • Basal cell
carcinoma• Seborrheic
keratosis• Sebaceous
hyperplasia• Etc.
15 top inflammatory skin lesions 15 top inflammatory skin lesions submitted to pathologysubmitted to pathology
• Arthropod assault• Erythema multiforme• Fixed drug• Granuloma annulare• Jessner’s/
lymphocytoma cutis• Lesion• Lichen planus• Leukocytoclasitc
vasculitis
• Mycosis fungoides/ parapsoriasis
• Polymorphous light eruption
• Psoriasis• Scleroderma/morphea• Urticaria• vasculitis
ReferencesReferences • Bolognia, Jorizzo & Rapini,
Dermatology, 2003:Elsevier, www.dermtext.com
• McKee, et.al. ,Pathology of the Skin with Clinical Correlations, 3rd ed. 2005:Elsevier.
• Bozzo P & Miller RC Clinical Dermatology and Dermatopathology: A Dynamic Interface series of ASCP lectures.