Darier's disease with esophageal involvement.

Dear Editor, We report the case of a Japanese man with Dari-er's disease (DD) that affected the esophagus as well as the skin. A 49-year-old man, who was diagnosed with DD 19 years earlier, visited us again in October 2008 because his skin lesions had exacerbated. Physical examination revealed reddish-brown crusted fol-licular papules mostly coalesced to produce irregularly shaped warty plaques on his trunk, hip, upper and lower limbs, and scalp (Figure 1, a, b). Skin biopsy taken from the hip showed hyperkeratosis, papillo-matosis, and suprabasal acantholysis with lacunae formation (Figure 1, c). The diagnosis of DD was confirmed and treatment with etretinate at 20 mg daily was started. The dose was increased to 50 mg 22 days later because his skin lesions failed to respond to the initial dose. When the dose was tapered to 20 mg after 2 months, painful erosions appeared on the hip. Tzanck smear testing showed balloon cells, and the serum level of Immunoglobulin M (IgM) antibody against herpes simplex virus (HSV) was elevated. The erosion was successfully treated with intravenous acy-clovir (750 mg/day for 5 consecutive days). The oral administration of valaciclovir (500 mg/day) was continued as prophylaxis against the recurrence of HSV infection. While the disease was well managed with 20 mg etretinate/day, the patient experienced aggravation in April 2010 and painful swallowing in Sep-tember 2010. Gastrointestinal endoscopy revealed multiple hyperkeratotic lesions in the middle of the esophagus (Figure 2, a). A biopsy showed histology similar to lesions on the skin including acantholysis and lacunae formation (Figure 2, b). Immunostaining did not detect either HSV-1, HSV-2, or human papillo-ma virus (HPV) in the esophageal mucosa. The skin le-sions improved but the esophageal lesions persisted unchanged 8 months after increasing the daily dose of etretinate to 40 mg. DD is a rare autosomal dominant genodermatosis characterized by abnormal keratinization that primarily affects the skin. A total of 8 cases of DD affecting the esophagus have been reported previously (1-6). Several important issues emerged from our experience and literature review. Firstly, DD predisposes to infections with HSV, vari-cella-zoster virus (VZV), HPV, and pox virus (7,8). HSV infection was diagnosed in 1 of 8 previously-reported cases of esophageal DD. In one case, a 20-year-old man had severe thoracic pain; his esophageal lesion was im-munohistochemically positive for HSV type I, and acy-clovir treatment produced an early clinical response (6). In our case, HSV was detected in the …