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ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyAssociated MedicationsReferencesView all Images (20)
Pemphigus vegetans - Skin
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Pemphigus vegetans - Skin

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Contributors: Natalia Khalaf MD, Sylvia Hsu MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Pemphigus vegetans, a rare variant of pemphigus vulgaris, is an autoimmune bullous disease of the skin and mucous membranes. The exact cause is unknown, but the mechanism underlying the pathology is well established to be IgG autoantibodies against desmoglein 3, a keratinocyte adhesion protein. This disruption in epidermal adhesion results in acantholysis (separation or dissolution of the intercellular bridges in the squamous layer of the epidermis) histologically and bulla formation clinically.

The essentials of diagnosing pemphigus disorders include the following:
  • Groups of bullae that remit and relapse, often preceded by erosion, ulceration, and blistering of mucous membranes
  • Positive Nikolsky's sign
  • Biopsy of lesions: histopathology shows acantholysis
  • Diagnostic confirmation of autoantibodies via direct immunofluorescence
Blisters and erosions develop in intertriginous areas and on mucous membranes, with the vast majority of cases presenting initially with erosions in the oral cavity. It can take weeks to months after the presentation of mucosal lesions for blisters and erosions to form on the skin. These lesions are typically filled with clear fluid and are easily ruptured, leaving only erosions (and a more challenging diagnostic picture) in their place. Pemphigus vegetans is a type of pemphigus vulgaris in which vegetative plaques form over erosions. A positive Nikolsky's sign (positive when gentle shearing pressure to pink or normal looking skin results in the formation of an erosion or extension of a bulla) is a common finding.

The incidence of pemphigus vulgaris is approximately one per million people, with the disease being more common among people of Ashkenazi Jewish and Mediterranean descent. Pemphigus vegetans makes up an estimated 2% to 5% of the total cases of pemphigus. Although this disease can affect people of all age groups, it occurs primarily during middle age with a median onset of 40-60 years. It affects males and females equally.

Pemphigus vegetans has been divided into two subtypes relating to clinical presentation and disease course: the Neumann type and the Hallopeau type. In the Neumann type, lesions are classically flaccid bullae and erosions in intertriginous areas that progress into vegetations. The Neumann type is more common, with a course very similar to that of pemphigus vulgaris. It usually requires high-dose systemic corticosteroids to treat; relapses are frequent. The Hallopeau type has a more benign course and is characterized by crops of pustules erupting and eventually progressing to dark plaques. Lower doses of systemic corticosteroids can be used, and some cases may even remit spontaneously. Oral mucosal involvement is nearly always present in both subtypes, as is the development of hyperpigmented vegetative plaques with thickened granulation tissue at the lesion's rim.

Codes

ICD10CM:
L10.1 – Pemphigus vegetans

SNOMEDCT:
81285006 – Pemphigus vegetans

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Differential Diagnosis & Pitfalls

Generalized pemphigus vulgaris – similar in presentation to Neumann type but not limited to intertriginous areas and without vegetating plaques.
 
Paraneoplastic pemphigus – associated with malignancy, most commonly non-Hodgkin's lymphoma; mucous membranes are the site of most severe involvement.
 
IgA pemphigus – mucous membranes are spared; IgA autoantibodies versus IgG in pemphigus vegetans.

Drug-induced pemphigus – most commonly captopril and D-penicillamine implicated; a precise drug history is critical, as these can be difficult to distinguish, especially if only oral lesions are present. Pemphigus foliaceus is more common than pemphigus vulgaris in cases of drug-induced pemphigus.

Pemphigus foliaceus (PF) – mucous membranes are spared; scales and crusts over erythematous skin in seborrheic areas.
  • Pemphigus erythematosus (also known as Senear-Usher syndrome) is a localized variety of PF largely confined to the malar region and seborrheic sites.
  • Fogo selvagem is an endemic form of PF found in rural Brazil.
Pemphigoid – IgG that targets hemidesmosomes; results in subepidermal blisters. In pemphigus vegetans the blisters are intraepidermal. Oral involvement occurs in only 10-35% of cases of pemphigoid. Nikolsky's sign is negative in pemphigoid.

Bullous impetigo – flaccid bullae; acantholysis on histology, but direct immunofluorescence (DIF) is negative. Blisters often become pustular.

Pemphigoid vegetans – a rare variant of pemphigoid with vegetative plaques also found in intertriginous areas; has the immunopathological features of pemphigoid.

Pyostomatitis vegetans – intraoral blistering most often associated with ulcerative colitis, but may also be associated with other inflammatory bowel diseases; clinically similar to pemphigus vegetans and only distinguishable by characteristically negative IF findings for autoantibodies.

Linear IgA dermatosis – annular configuration of bullae; subepidermal blisters with DIF showing IgA at dermal-epidermal junction.

Dermatitis herpetiformis – positive for antiendomysial and anti-transglutaminase antibodies; classically, crops of vesicles and urticarial plaques develop on extensor surfaces, gluteal-sacral, and scapular regions; DIF shows IgA in the dermal papillae. Extremely itchy.

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Therapy

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References

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Last Updated: 12/07/2010
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Pemphigus vegetans - Skin
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Pemphigus vegetans : Axilla, Bullae, Chin, Crust, Erythema, Face, Flexural, Oral erosions, Scalp, Vegetative plaque, Inguinal region
Clinical image of Pemphigus vegetans
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