Dermatitis herpetiformis (DH)  is characterized by grouped papulovesicles (“herpetiform”) on an erythematous base in a symmetrical distribution, occasionally accompanied by urticarial plaques, most commonly on the elbows, knees, extensor forearms, back, and buttocks.  While 90% of DH patients have gluten-sensitive enteropathy, only 20% have symptomatic malabsorption. The course of the disease is lifelong, with periods of worsening and improvement, but 10% of patients experience spontaneous resolution. It is rare in children and adolescents and uncommon in African-Americans and Asians.

Linear IgA bullous disease is a rare autoimmune blistering disease characterized by linear deposition of immunoglobulin A at the dermo-epidermal junction, as demonstrated by immunofluorescence. In adults, this pathology presents with polymorphic clinical features that can resemble those of DH or bullous pemphigoid, including vesiculobullae in a herpetiform arrangement on normal or erythematous skin, tense bullae, expanding annular plaques, “crown of jewels,” and scattered asymmetric vesiculobullous lesions. Drug-induced LABD can have a TEN-like or morbilliform appearance. In children, “chronic bullous disease of childhood” (CBDC) has a unique clinical appearance, with annular erythematous bullae (crown of jewels) with central crusting in flexural areas such as the lower trunk, thigh, and groin.

This quiz is designed to test your knowledge on the epidemiology, pathogenesis, clinical features, differential diagnosis and management of DH and LABD. We encourage you to review the associated learning module before taking the quiz.

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Sources

Bolognia, J., Cerroni, L., & Schaffer, J. V. (2018). Dermatology.

  • Release Date: March 6, 2023

  • Last Updated: March 6, 2023

  • Time to complete: 30 minutes

  • Author:

    • Dr. Justina Guirguis, MD
    • Dr. Kayadri Ratnarajah, MD, McGill University
    • Dr. Elena Netchiporouk, MD, McGill University