Clinical correlation of inflammatory skin lesions

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Clinical correlation of inflammatory skin lesions

• Mary Jo Robinson, D.O.
• UMDNJ-SOM
• Oct.3, 2007

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5 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.

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8 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

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gt2000 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

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100 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

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So how does the pathologist correlate 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

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Pustular

• Macroscopic
• Pustular dermatosis - impetigo -
  folliculitis - acne
• - Rosacea
• - candidiasis
• - Pustular psoriasis
• - Sweets

• Microscopic
• Collections of neutrophils, eosinophils or
  lymphocytes in epidermis, follicle, sebaceous
  glands, etc

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Generalized and intense erythematous rash with
pustules
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Palmoplantar pustules
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Macropustule
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Pustular psoriasis

• Biopsy to exclude fungus, pustular drug,
  impetigo, superficial pemphigus, impetigo
  herpetiformis
• Should culture

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Histology not specific, CC necessary

• Pustular psoriasis cannot be distinguished on
  histology from - acrodermatitis
  continua(pustular eruption on one of more
  fingers) - Reiters disease(arthritis,
  conjunctivitis, balanitis, pustular
  dermatosis) - impetigo herpetiformis(
  pustular dermatosis of pregnancy assoc w/
  hypocalcemia)

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Papular minivesicular

• Macroscopic
• Papular minivesicular dermatitis - contact
  dermatitis - atopic dermatitis - scabies -
  dermatophytosis
• - stasis dermatitis
• - Grovers
• - Hailey Hailey
• - Mucha-Habermann - Dermatitis herpetiformis

• Microscopic
• Epidermal spongiosis w/ scale crust

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Intensely pruritic rash of elbows, knees, back
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Early
Late
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Floor of blister
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DIF granular IgA in dermal papillae
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D/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

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Dermatitis herpetiformis

• clinically Grovers, 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

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• 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

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Vesiculobullous

• Macroscopic
• Burn
• Erythema multiforme
• Pemphigus vulgaris
• Dermatitis herpetiformis
• Herpes simplex
• Bullous pemphigoid
• Contact dermatitis
• Fixed drug eruptions

• Microscopic
• Epidermal or subepidermal vesicle(lt10mm)
• Bullae(gt10 mm)

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Firm bullae w/ erosions, crusts, papules and
wheals

• groin, axillae, forearms, oral
• Intertriginous to generalized
• Pink to red

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Histopathology
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DIF Salt split skin IgG
DIF linear IgG
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Type IV collagen present along base of blister
EBA - collagen along roof of blister
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Bullous 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

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Histologic 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

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Pruritic vesiculopustular disease of trunk and
proximal extremities
Note the vesicles start clear and then fill with
white creamy pus, then erosions form
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Histology

• Subcorneal pustules to bullae with neutrophilic
  infiltrate w/ sparse to moderate numbers of
  eosinophils

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IgA 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

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IEN type
Intraepidermal pustules of neutrophils and some
eosinophils
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IgA 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

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Papulosquamous

• Macroscopic
• Lichen planus
• Psoriasis
• Lupus erythematosus
• Pityriasis rosea
• Seborrheic dermatitis
• Solar keratosis
• Scaly dermatophytosis
• Ichthyosis
• Mycosis fungoides
• Pityriasis rubra pilaris

• Microscopic
• Confluent orthokeratosis, parakeratosis or
  alternating OK/PK sometimes with minimal serum

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Clinical

• Scaly
• Large scale(flakes) gt 1mm size large scale
  dz (psoriasiform)

• Small scales lt 1 mm size small scale
  dz (pityriasis)
• Shiny compact scales compact scale dz (lichenoid)

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Histopathology

• 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

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Plaques with overlying scale and erythematous
borders
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IgG, complement band at base of epidermis on IF
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Lupus 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

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Follicular plugging
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Polymorphous autoimmune disease

• 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

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Stage of disease affects histology

• 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

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Histologic D/DX

• Seborrheic dermatitis- early forms w/ pyknotic
  neutrophils _at_ 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

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Flat topped violaceous papules w/ shiny scale
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Histology
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Lichen 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

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Dermis in LP

• lichenoid lymphohistiocytic infiltrate fills
  papillary dermis, is dense and close to base of
  epidermis
• Coarse collagen bundles
• No mucin, no edema

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D/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

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Papulonodular

• Macroscopic
• Prurigo nodularis
• Granuloma annulare
• Amyloidosis
• Sarcoid
• Acne
• Follicular cysts
• Arthropod assaults
• Lymphocytoma cutis
• Polyarteritis nodosa

• Microscopic
• Scale crust and spongiosis are ABSENT
• Acanthosis, dermal deposits or inflammation are
  PRESENT

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Grouped 1-2 mm flesh colored to pink papules in
arcuate distribution on extremities
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Clinical d/dx

• Sarcoid
• Lichen planus
• Urticaria pigmentosa
• Papular mucinosis
• Tinea corporis
• Necrobiosis lipoidica
• Rheumatoid nodule
• Foreign body
• Granulomatous rosacea

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Granuloma annulare Histology
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D/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

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Vascular 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
• Schambergs
• telangiectasia Hemangiomas
• Kaposis

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Purpura as a clinical clue

• Purpuric residual erythema persists with
  pressure ecchymosis and petechiae
• Leukocytoclastic vasculitis, septic vasculitis,
  pigmented purpura, dysproteinemic purpura,
  thrombocytopenic purpura

• Non-purpuric complete blanching with
  application of pressure, no extravasated rbcs in
  dermis
• Sunburn, urticaria, macular papular erythema,
  erythema nodosum, fixed drug, gyrate erythemas

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Bright red to brown red purpuric papules lower
extremities
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Histology
Endothelial cell swelling, angiocentric
neutrophilic inflammation with nuclear dust,
fibrin in vessel walls, extravasated erythrocytes
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Henoch-Schoenlein purpura-IgA mediated in
kids(beta strept)
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Pigmentary

• 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

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White to yellow linear plaques with violaceus to
erythematous halo
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Histology linear morphea
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Tumor

• Macroscopic
• Mycosis fungoides
• Kaposi sarcoma
• Melanoma
• Basal cell carcinoma
• Seborrheic keratosis
• Sebaceous hyperplasia
• Etc.

• Microscopic
• neoplastic proliferation of cells
• epidermal
• Dermal
• Melanocytic
• other

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15 top inflammatory skin lesions submitted to
pathology

• Arthropod assault
• Erythema multiforme
• Fixed drug
• Granuloma annulare
• Jessners/ lymphocytoma cutis
• Lesion
• Lichen planus
• Leukocytoclasitc vasculitis

• Mycosis fungoides/ parapsoriasis
• Polymorphous light eruption
• Psoriasis
• Scleroderma/morphea
• Urticaria
• vasculitis

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References

• Bolognia, Jorizzo Rapini, Dermatology,
  2003Elsevier, www.dermtext.com
• McKee, et.al. ,Pathology of the Skin with
  Clinical Correlations, 3rd ed. 2005Elsevier.
• Bozzo P Miller RC Clinical Dermatology and
  Dermatopathology A Dynamic Interface series of
  ASCP lectures.