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  • Gok=god only knows
  • DIF DH- granular IGA; Linear IgA dermatosis-linear IgA, lack of gluten sensitive enteropathy, Bullous pemphigoid-linear IgG
  • Linear IgG which on salt split skin binds to roof of blister
  • Bx usually done for medico/legal reasons prior to tx w/ steroids, to exclude LE and lichenoid drug
  • Transcript

    • 1. Clinical correlation ofClinical correlation of inflammatory skininflammatory skin lesionslesions Mary Jo Robinson, D.O.Mary Jo Robinson, D.O. UMDNJ-SOMUMDNJ-SOM Oct.3, 2007Oct.3, 2007
    • 2. 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.
    • 3. 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
    • 4. >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
    • 5. 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
    • 6. So how does the pathologistSo 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
    • 7. 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
    • 8. Generalized and intenseGeneralized and intense erythematous rash with pustuleserythematous rash with pustules
    • 9. Palmoplantar pustulesPalmoplantar pustules
    • 10. MacropustuleMacropustule
    • 11. PustularPustular psoriasispsoriasis • Biopsy to exclude fungus, pustular drug, impetigo, superficial pemphigus, impetigo herpetiformis • Should culture
    • 12. Histology not specific, CCHistology not specific, CC necessarynecessary • 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)
    • 13. 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
    • 14. Intensely pruritic rash ofIntensely pruritic rash of elbows, knees, backelbows, knees, back
    • 15. Early Late
    • 16. Floor ofFloor of blisterblister
    • 17. DIF granular IgA in dermalDIF granular IgA in dermal papillaepapillae
    • 18. D/Dx DH via DIFD/Dx DH via DIF • DIF DH- granular IgA • Linear IgA dermatosis-linear IgA also lack of gluten sensitive enteropathy, no association w/HLA-B8 &DR-3 antigens, less response to dapsone tx • Bullous pemphigoid-linear IgG
    • 19. 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
    • 20. • 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
    • 21. 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
    • 22. 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
    • 23. HistopathologyHistopathology
    • 24. DIF linear IgG DIF Salt split skin IgG
    • 25. Type IV collagen present alongType IV collagen present along base of blisterbase of blister EBA - collagen along roof of blister
    • 26. 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
    • 27. 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
    • 28. Pruritic vesiculopustular diseasePruritic 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
    • 29. HistologyHistology • Subcorneal pustules to bullae with neutrophilic infiltrate w/ sparse to moderate numbers of eosinophils
    • 30. 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
    • 31. IEN typeIEN type Intraepidermal pustules of neutrophils and some eosinophils
    • 32. 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
    • 33. PapulosquamousPapulosquamous • 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
    • 34. ClinicalClinical • Scaly • Large scale(flakes) > 1mm size= large scale dz (psoriasiform) • Small scales < 1 mm size= small scale dz (pityriasis) • Shiny compact scales compact scale dz (lichenoid)
    • 35. 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
    • 36. Plaques with overlying scalePlaques with overlying scale and erythematous bordersand erythematous borders
    • 37. IgG, complement band at base of epidermis on IF
    • 38. 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
    • 39. Follicular pluggingFollicular plugging
    • 40. Polymorphous autoimmunePolymorphous 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
    • 41. Stage of disease affectsStage 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
    • 42. 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
    • 43. Flat topped violaceousFlat topped violaceous papules w/ shiny scalepapules w/ shiny scale
    • 44. HistologyHistology
    • 45. 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
    • 46. 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
    • 47. 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
    • 48. PapulonodularPapulonodular • 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
    • 49. Grouped 1-2 mm flesh colored toGrouped 1-2 mm flesh colored to pink papules in arcuatepink papules in arcuate distribution on extremitiesdistribution on extremities
    • 50. Clinical d/dxClinical d/dx • Sarcoid • Lichen planus • Urticaria pigmentosa • Papular mucinosis • Tinea corporis • Necrobiosis lipoidica • Rheumatoid nodule • Foreign body • Granulomatous rosacea
    • 51. Granuloma annulare HistologyGranuloma annulare Histology
    • 52. 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
    • 53. 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
    • 54. 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 • Purpuric residual erythema persists with pressure ecchymosis and petechiae • Leukocytoclastic vasculitis, septic vasculitis, pigmented purpura, dysproteinemic purpura, thrombocytopenic purpura
    • 55. Bright red to brown red purpuricBright red to brown red purpuric papules lower extremitiespapules lower extremities
    • 56. HistologyHistology Endothelial cell swelling, angiocentric neutrophilic inflammation with nuclear dust, fibrin in vessel walls, extravasated erythrocytes
    • 57. Henoch-Schoenlein purpura-IgAHenoch-Schoenlein purpura-IgA mediated in kids(beta strept)mediated in kids(beta strept)
    • 58. 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
    • 59. White to yellow linear plaquesWhite to yellow linear plaques with violaceus to erythematouswith violaceus to erythematous halohalo
    • 60. Histology linear morpheaHistology linear morphea
    • 61. TumorTumor • Microscopic • neoplastic proliferation of cells • epidermal • Dermal • Melanocytic • other • Macroscopic • Mycosis fungoides • Kaposi sarcoma • Melanoma • Basal cell carcinoma • Seborrheic keratosis • Sebaceous hyperplasia • Etc.
    • 62. 15 top inflammatory skin lesions15 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
    • 63. 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.

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