Diltiazem-associated, photo-distributed hyperpigmentation in a patient with Sjögren’s syndrome

Abstract

Sir, Herein, we report a case of hyperpigmented macules on ultraviolet exposed areas during the intake of diltiazem hydrochloride (DTH) in a patient with Sjögren’s syndrome. A 63-year-old Japanese female with a history of Sjögren’s syndrome and vasospastic angina presented to our hospital with brown, irregular macules on the neck, face, and both hands, which she first noticed one year earlier. The patient had a history of taking DTH for vasospastic angina for the previous fifteen years. A physical examination revealed pale brown macules and plaques irregularly scattered around the corners of the mouth, lower lip, and neck (Figs. 1a and 1b). In addition, there were numerous erythematous papules on the dorsal side of both hands. The oral mucosal membrane, scalp, and nails were not involved. A biopsy specimen taken from the dorsal side of a hand revealed individual cell keratinization in the epidermis, intercellular edema, liquefaction degeneration of the basal layer, and band-like lymphocyte infiltrate with melanophages in the superficial dermis (Fig. 2a). Immunohistochemistry revealed the infiltration of CD3+, CD4+, CD8+, and HLA-DR+ lymphocytes in the superficial dermis (Figs. 2b and 2c). Routine laboratory examinations of the blood cell count, serum, and urine showed no abnormalities. Other tests showed positive antinuclear antibody (1:80, nucleolar) and anti-SS-A antibody (240 U/mL; normal: < 9.9), whereas hepatitis C virus antibody, rheumatoid factor, anti-CCP antibody, and anti-SS-B antibody were negative. DTH was switched to another medicine, and ascorbic acid, calcium pantothenate, and topical steroids were initiated. In addition, we advised the patient to avoid sun exposure. Although no improvement was observed in the rash in the first four months, it disappeared at follow-up several years later (Figs. 3a and 3b).