ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (2)
Pemphigus herpetiformis
Print
Other Resources UpToDate PubMed

Pemphigus herpetiformis

Print Images (2)
Contributors: Chase W. Kwon BA, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Pemphigus herpetiformis (PH) is a rare, acquired autoimmune disorder that is characterized by a clinical appearance resembling dermatitis herpetiformis, along with histological and immunopathological features of pemphigus. While some have argued that PH represents an atypical variant of pemphigus foliaceus (PF) or pemphigus vulgaris (PV), PH is generally considered a distinct clinical entity.

PH is a rare condition, with approximately 100 reported cases, typically manifesting in middle-aged adults as an intensely pruritic cutaneous eruption with herpetiform clusters of vesicles. It is even rarer in children, with fewer than 10 reported pediatric cases. No ethnic or gender predilections have been observed.

The inciting factors of the disease have not been identified. IgG autoantibodies bind to desmoglein 1 (less commonly, desmoglein 3 or desmocollin 1 or 3) on keratinocytes, stimulating upregulation of interleukin-8, which serves as a chemoattractant for eosinophils and neutrophils. In turn, these inflammatory cells secrete proteases that mediate eosinophilic or neutrophilic spongiosis and blister formation.

Unlike the classical pemphigus disorders, PH usually exhibits a benign clinical course. However, cases of PH progressing to PF or PV have been reported.

Codes

ICD10CM:
L10.89 – Other pemphigus

SNOMEDCT:
84855007 – Acantholytic vesicular dermatitis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Dermatitis herpetiformis – History of gluten-insensitive enteropathy. Can differentiate with histopathological survey and immunofluorescence studies, as well as serologic testing.
  • Pemphigus foliaceus (PF) – Clinical appearance of PH resembles dermatitis herpetiformis more so than PF.
  • Pemphigus vulgaris (PV) – Clinical appearance of PH resembles dermatitis herpetiformis more so than PV. Lesions of PV tend to be painful, in contrast to the nontender, pruritic lesions of PH. PV commonly involves the mucous membranes, which are often spared in PH.
  • Bullous pemphigoid – Can differentiate with histopathological survey and immunofluorescence studies.
  • Linear IgA bullous dermatosis – Can differentiate with histopathological survey and immunofluorescence studies.
  • IgA pemphigus – Can differentiate with histopathological survey and immunofluorescence studies.
  • Bullous drug eruption
  • Allergic contact dermatitis

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 05/04/2017
Last Updated: 06/13/2017
Copyright © 2019 VisualDx®. All rights reserved.
Pemphigus herpetiformis
Print 2 Images
View all Images (2)
(with subscription)
Pemphigus herpetiformis : Pruritus, Smooth papules, Vesicles, Skin erosions
Copyright © 2019 VisualDx®. All rights reserved.