Skin inflammatory (nontumor)
Vesiculobullous and acantholytic reaction patterns
Pemphigus


Topic Completed: 1 July 2011

Revised: 28 March 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Pemphigus [title] skin

Mowafak Hamodat, M.B.Ch.B., M.Sc.
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Cite this page: Hamodat M Pemphigus. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skinnontumorpemphigus.html. Accessed November 13th, 2019.
Definition / general
  • Rare, nonhereditary, chronic, autoimmune disease wtih flaccid blisters and denuded skin
  • Associated with internal malignancies, including thymoma

Variants:
  • 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 (Nikolskyís 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 AsboeñHansen sign
    • Healing is often accompanied by postñinflammatory hyperpigmentation but no scarring
    • Fatal if untreated because oral erosions impair swallowing
    • Deaths are due to staphylococcal infection or pulmonary embolism
Epidemiology
  • Ages 30 - 59; no gender preference
Case reports
Treatment
  • Corticosteroids or immunosuppressive agents
Clinical images

Contributed by Mark R. Wick, M.D.

Pemphigus erythematosus (breast skin)

Pemphigus vulgaris (breast skin)



Images hosted on other servers:

Paraneoplastic pemphigus

Paraneoplastic pemphigus associated with non-Hodgkin lymphoma

Pemphigus vegetans

Pemphigus vulgaris

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

Case of the Week #56

Pemphigus foliaceus

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



Contributed by Mark R. Wick, M.D.

Pemphigus vulgaris (breast skin)

Pemphigus vulgaris (breast skin), IgG stain



Contributed by Asmaa Gaber Abdou, M.D.

Pemphigus foliaceus



Images hosted on other servers:

Paraneoplastic pemphigus, Tzanck test

Paraneoplastic pemphigus, acantholytic blister

Pemphigus vegetans

Pemphigus vulgaris, various images

Positive stains
  • 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
Differential diagnosis
  • Differential diagnosis of acantholysis includes:
  • Also:
    • Actinic keratosis
    • Focal changes associated with other tumors
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