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Skin-nontumor / Clinical Dermatology

Blistering disorders

Pemphigus


Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC (see Reviewers page)
Revised: 26 August 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Rare, nonhereditary, chronic, autoimmune disease wtih flaccid blisters and denuded skin
● Associated with internal malignancies, including thymoma

Epidemiology
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● Ages 30-59; no gender preference

Variants
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● Types: Erythematosus, Foliaceus, Paraneoplastic, Vegetans and Vulgris

Erythematosus:
● Also called Senear–Usher syndrome
● Involves a localized area, like a lupus malar rash on face
● Otherwise identical to pemphigus foliaceus combined with features of lupus erythematosus
● Usually middle aged adults (Clin Exp Dermatol 2009;34:708)

Pemphigus foliaceus:
● Endemic in Brazil; called fogo selvagem or “wild fire” in Portugese (Br J Dermatol 2006;155:446, Int J Dermatol 2005;44:293)
● Crusts and shallow erosions on healthy appearing skin (often trunk) with blisters that form after rubbing (Nikolsky’s sign)
● Typically spares mucous membranes
● Lesions may not appear bullous because crusts and erosions replace the bullae
● Clinically mild

Paraneoplastic pemphigus:
● Associated with lymphoma, thymoma, squamous cell carcinoma, carcinoma of bronchus, pancreas and breast
● Oral and cutaneous erosions and bullae
● Stomatitis may be the only symptom
● Treat by treating the tumor
Due to autoantibodies targeting proteins of the plakin and cadherin families involved in maintenance of cell architecture and tissue cohesion; recently identified as alpha-2-macroglobuline-like-1 (A2ML1) (PLoS One 2010;5:e12250)

Pemphigus vegetans:
● Rare (1-2% of pemphigus cases) variant of pemphigus vulgaris characterized by verrucous plaques in the flexural regions
● Oral cavity is commonly affected; cerebriform or scrotal tongue is a diagnostic clue of early involvement
● Reported in long standing nasal heroin abuse and with HIV+ patients

Pemphigus vulgaris:
● 80% of all pemphigus
● Oral mucosa; also scalp, face, eye, pharynx, larynx, axilla, groin, trunk; nail involvement
● Direct pressure to the center of the lesion is followed by lateral extension, the Asboe–Hansen sign
● Healing is often accompanied by post–inflammatory hyperpigmentation but no scarring
● Fatal if untreated because oral erosions impair swallowing
● Deaths are due to staphylococcal infection or pulmonary embolism

Treatment and prognosis
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● Corticosteroids or immunosuppressive agents

Case reports
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● 83 year old woman with pemphigus vegetans and colon cancer (Indian J Dermatol Venereol Leprol 2009;75:603)
● 85 year old Mexican woman with plaques on trunk and pemphigus foliaceus (Case of the Week #56)

Clinical images
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Pemphigus erythematosus


Pemphigus foliaceus

   
Paraneoplastic pemphigus

   
Paraneoplastic pemphigus associated with non-Hodgkin lymphoma


Pemphigus vegetans

   
Pemphigus vulgaris

Micro description
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● Intraepidermal blister is just above basal layer and due to acantholysis from IgG against desmosomes and production of plasminogen activator
● Epithelial cells are rounded due to loss of desmosomes
● Often eosinophilic spongiosis

Pemphigus foliaceus:
● Only the granular cell layer is affected with a discrete acantholytic bullae containing rounded, acantholytic keratinocytes and few inflammatory cells
● Stratum corneum may be denuded
● Often eosinophilic spongiosis
● Blister may contain many neutrophils, which makes distinction from subcorneal pustular disorders especially difficult

Paraneoplastic pemphigus
● Suprabasal acantholysis with cleft and vesicle formation
● Vacuolar interface with spongiosis, dyskeratotic keratinocytes and lymphocyte excocytosis
● Perivascular lichenoid chronic inflammatory cell infiltrate
● Frequent pigment incontinence

Pemphigus vegetans:
● Supra basal acantholysis is subtle, masked by exuberant proliferation of squamous epithelium which may show pseudo epitheliomatous hyperplasia
● Large intraepidermal microabscesses filled with eosinophils and occasional acantholytic keratinocytes

Pemphigus vulgaris:
● Numerous small, flaccid, suprabasilar bullae with single row of keratinocytes attached to basement membrane
● Prominent extension of acantholysis into follicular infundibula
● Ruptured bullae cause skin erosions
● Minimal dermal infiltrate

Micro images
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Pemphigus foliaceus: various images


Pemphigus foliaceus: C3 (left) and IgG (right)


Paraneoplastic pemphigus: Tzanck test revealing the presence of acantholytic cells


Paraneoplastic pemphigus: acantholytic blister in epidermis

   
Pemphigus vegetans


Pemphigus vulgaris

Positive stains
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● Necessary to confirm diagnosis - IgG against desmoglein 1 (usually) or desmoglein 3 (desmosome components) or C3 are present in a fishnet-like pattern in intercellular squamous region
● IgA is positive in IgA pemphigus
● In paraneoplastic pemphigus, granular deposits of C3 are also present at basement membrane

Videos
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Pemphigus foliaceus and Pemphigus erythematosus
Paraneoplastic pemphigus

Differential diagnosis
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● Differential diagnosis of acantholysis includes Darier's disease, familial benign pemphigus, Hailey-Hailey disease, viral vesicles, D-penicillamine related pemphigus like lesions
● Also actinic keratosis and associated squamous cell carcinoma, warty dyskeratoma and transient acantholytic dermatosis; focal changes also associated with other tumors

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Pemphigus


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