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StatPearls, LLC

  • Autoantibodies
  • Azathioprine
  • Dermatitis Herpetiformis
  • Fluorescent Antibody Technique, Direct
  • Pemphigus

Pemphigus Herpetiformis

Pemphigus herpetiformis is a rare pemphigus variant that clinically mimics dermatitis herpetiformis while exhibiting immunopathological features of pemphigus. Intensely pruritic, grouped vesicles or papulovesicles, urticarial plaques, and erythematous patches commonly appear in herpetiform or annular patterns. Lesions favor the trunk, proximal extremities, and flexural regions. Mucosal involvement occurs infrequently. Histopathology often reveals eosinophilic or neutrophilic spongiosis, occasionally with subcorneal pustules and minimal or absent acantholysis, which complicates diagnosis. Direct immunofluorescence demonstrates intercellular IgG, sometimes C3, within the epidermis. Most patients demonstrate circulating autoantibodies targeting desmoglein 1. Others exhibit reactivity to desmoglein 3 or desmocollins. Autoantibody-mediated disruption of desmosomal proteins underlies disease pathogenesis, consistent with other pemphigus variants. Systemic corticosteroids are commonly used, although dapsone often achieves rapid clinical improvement due to its neutrophil-modulating effects. Immunosuppressive agents, including azathioprine and mycophenolate mofetil, are employed in resistant cases. Compared to pemphigus vulgaris and pemphigus foliaceus, pemphigus herpetiformis typically follows a more indolent course with fewer complications and reduced pharmacologic requirements. Accurate diagnosis requires clinicopathologic correlation to distinguish this entity from dermatitis herpetiformis and other bullous dermatoses. Early recognition permits effective therapeutic intervention and minimizes unnecessary immunosuppression. This activity for healthcare professionals is designed to enhance learners’ proficiency in evaluating and managing pemphigus herpetiformis. Participants will broaden their grasp of the condition’s etiology, epidemiology, genetics, and clinical presentation. Emphasis will be given to the best diagnostic and therapeutic practices. Improved skills will…

Credit & MOC Details

Credit information is provided by the course provider. We recommend confirming credit eligibility with the provider or your licensing board before starting the activity.

Credit types

AAPA Category 1 Credit™Up to 1 creditAMA PRA Category 1 Credit™Up to 1 creditNursingUp to 1 creditPharmacyUp to 1 credit

MOC types

ABS — Accredited CMEUp to 1 pointABPATH — Lifelong LearningUp to 1 pointABTS — Accredited CMEUp to 1 pointABIM — Medical KnowledgeUp to 1 pointABS — Self-AssessmentUp to 1 pointABP — Lifelong Learning and Self-AssessmentUp to 1 pointABTS — Self-AssessmentUp to 1 point

FDA REMS

No

Qualifies for MIPS

No

Target Specialties

Pricing

Fee varies

This provider typically $49–98

See fees on statpearls.com

Listed by the provider

  • Article / monograph / reference
  • Credit · Up to 1 credit
  • MOC · Up to 1 point
  • Fee varies · this provider typically $49–98
  • Released
    Sep 1, 2026
  • Last day to claim credit
    Sep 1, 2029 3 years left

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