A Chronically Ill Teenager with Blisters and Scars

Figure 19.1

Blisters of varying sizes on the limb

Figure 19.2 demonstrating an active blister with the background of an ulcer and secondary leucoderma

Figure 19.2

An active blister with the background of an ulcer and secondary leucoderma

What is your diagnosis?

EBA
Bullous Pemphigoid
Cicatricial Pemphigoid
Bullous SLE

Biopsy showed a subepidermal blister with cell poor infiltrate and IFM was positive for IgG, C3 and IgM at the DEJ. She was found to have low complement C3 and ANF was positive in a titre of 1: 1250.

This patient was diagnosed with Bullous SLE and therapy with prednisone started at 0.5 mg/kg and Dapsone.

Bullous SLE

Bullous LE is an autoimmune blistering condition, often transient that occurs in the setting of systemic lupus erythematosus. It is commonly seen in young female patients of African descent [1–2].

Bullous lesions of Lupus Erythematosus can be single or widespread. They are commonly but not limited to sun exposed areas [1–2]. These lesions are painful but not pruritic. Patients with bullous lupus erythematosus meet the criteria for systemic lupus erythematosus hence (bullous SLE), but patient do exist with similar lesions but have fewer symptoms to meet the criteria (disease in evolution [1]). The occurrence of blisters is not related to flares of systemic disease [1]. Histology shows a sub-epidermal blister rich in neutrophils. Direct Immunofluorescence shows IgG, IgA, IgM and C3 in a granular or linear pattern at the basement membrane zone [1]. These antibodies target type VII collagen. Dapsone is first line therapy and is most effective [1–2].

Only gold members can continue reading. Log In or Register to continue