Linear IgA bullous dermatosis

Antoine Costenoble, Dominique Tennstedt (1) Published in the journal : April 2018 Category : Dermatology

Summary :

Linear IgA bullous dermatosis is a rare autoimmune bullous disease. It proves to be the most common pediatric bullous dermatosis, affecting mainly children older than 4 years. The histopathological aspect of the lesion is highly suggestive, with direct immunofluorescence (DIF) of the perilesional skin showing intense and homogeneous linear IgA deposits on the basement membrane zone (BMZ). These deposits prove pathognomonic for the condition.

Two presentations have been classically reported : a childhood-onset and an adult-onset form, the latter being often drug-related from an etiological point of view.

Clinical lesions characterized by a vesicular or bullous eruption with a typical annular rosette-like aspect are mainly located on the lower abdomen, perineum, perioral area, and buttocks.

Dapsone® (diaminodiphenylsulfone) is the first-choice treatment for this affection, although pre-therapeutic precautions must be considered.

Nevertheless, a spontaneous remission may occur in many cases within 3 to 6 years, whereas prolonged clinical courses prove to be very rare.

What is already known about the topic?

- Linear IgA bullous dermatosis is the most common pediatric autoimmune bullous disease with standard diagnostic errors.

- It exhibits a specific pathognomonic immunohistochemical pattern.

- Specific treatment with Dapsone® is indicated.

What does this article bring up for us?

- This article emphasizes the need of performing a biopsy for immunohistochemical analysis prior to initiating specific and long-term treatment.

- It also underlines the necessity of a pre-therapy investigation when using Dapsone®.

- A close dermatopediatric collaboration proves necessary for managing pediatric bullous diseases.

Key Words Linear

IgA dermatosis, autoimmune bullous disease, Dapsone®