Samantha S., a medical student at UC Berkeley, shares how VisualDx image collection helped her narrow the differential and make a diagnosis.
“I was seeing a patient with tense bullae in a very interesting clustered configuration. It made me think of bullous pemphigoid, but I couldn’t place what it really was. Using VisualDx, I was able to narrow it down to linear IgA bullous dermatosis! It was the ‘crown of jewels!’”
What is linear IGA bullous dermatosis?
Linear IgA bullous dermatosis (LABD) is a rare immune-mediated disorder that occurs in both children and adults. The disease is defined by the presence of a homogenous deposition of IgA in a linear band at the epidermal-dermal junction. The 3 main types of LABD include the drug-induced, pediatric, and adult forms of the disease.
In the adult form, LABD most commonly presents in the fifth decade of life. Clinical presentation is highly variable, ranging from urticarial plaques, papules, and papulovesicles to large erosions, and the rash can be focal or generalized.
What should we be aware of when making a diagnosis?
Classically, LABD in adults presents with widespread tense vesicles in an arciform or annular pattern (so-called string of pearls, crowns of jewels, or rosette appearance). However, clinical presentation can range from urticarial plaques, papules, and papulovesicles to large erosions. Involvement can be localized or generalized. The trunk, extensor surfaces, buttocks, and face (perioral area) are commonly involved.
How can we treat this?
Drug-induced and pediatric LABD can be self-resolving and require only temporary treatment. The adult form tends to have a more chronic course and treatment may include dapsone, sulfapyridine, or colchicine. Mild cases can be treated with a class 1 or 2 topical corticosteroid, although caution must be taken with long-term use of high-potency topical corticosteroids.