Successful treatment of epidermolysis bullosa pruriginosa with topical tacrolimus.

A 53-year-old white woman with epidermolysis bullosa (EB) pruriginosa presented to the dermatology clinic seeking therapy for localized severe and intractable pruritus. At the age of 4 years the patient developed an extremely pruritic bullous eruption characterized by fragile blisters, erosions, and excoriations affecting sites subject to friction and pressure, particularly the ankles and knees. By the age of 21 years, the patient had large lichenified plaques, areas of linear violaceous scarring, multiple milia, and erosions over much of the extensor surfaces of the legs, forearms, buttocks, and dorsum of the feet (Figure 1A and B). All of her 20 nails were grossly dystrophic (Figure 1C). Mucous membranes were normal. There was a family history of EB pruriginosa, as the patient’s younger brother and daughter were similarly affected. Electron microscopical features supported the diagnosis of EB pruriginosa: separation at the sublamina densa level of the dermoepidermal junction and abnormal anchoring fibrils that were scanty, disorganized, and not fully developed (Figure 2). Blood cell count and serum biochemistry findings were normal. Serum levels of thyroid hormones, ferritin, and IgE were all within normal ranges. Throughout the course of the disease, intractable pruritus was the most prominent and severe feature, far outweighing the significant cosmetic disability. Continuous rubbing and scratching generated large areas of disease over the shins and forearms. The patient often presented with areas of frank bleeding resulting from deep excoriations. Treatments included inpatient and outpatient care with a combination of intensive emolliation, potent topical corticosteroids, sedating antihistamines, and behavioral therapy. These resulted in short-term control of the cutaneous manifestations, but not sustained improvement or control of the pruritus. Thinning of the skin secondary to the ongoing use of potent topical steroids was also evident.