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. 

In this learning module, you will find a key point review on DH and LABD focused on epidemiology, pathogenesis, clinical features, differential diagnosis and management. To test your knowledge, we refer you to the associated quiz (short answer format questions) and clinical vignettes simulating OSCE examinations. We hope you find this module useful and if you choose to submit new questions or vignettes to increase the content of this module or post comments on how we can improve this module for your learning, we look forward to them.

Select how you would like to proceed below:

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