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Diffuse cutaneous mastocytosis treated with psoralen photochemotherapy: case report and review of the literature

SIR, A male infant aged 7 months was referred to Great Ormond Street Hospital for Children with a history of blistering and pruritus. His skin was normal until the age of 10 weeks, when he developed an erythematous rash over the back of his head, neck and shoulders. The primary symptom at this stage was pruritus, unalleviated by mild topical steroids, emollients and antihistamines from his general practitioner. He then developed blisters on his neck and groin, some of which became secondarily infected, and he began to have episodes of intense flushing, which would last for up to 2 h. His sleep was disturbed by pruritus, but he was otherwise well, gaining weight and developing normally. Examination revealed slightly thickened, granular-looking skin with a yellow tinge, marked dermographism, and thinwalled blisters. A clinical diagnosis of diffuse cutaneous mastocytosis was made, and was confirmed on punch biopsy. Initial therapy was with high-dose nonsedative and sedative antihistamines during the day and night, respectively, and the application of beclometasone dipropionate ointment diluted 1 : 10 in white soft paraffin under wet wraps twice daily. Relief of symptoms was, however, minimal, and by 14 months of age he was unable to sleep for more than a few hours at a time, and the episodes of flushing were occurring every other day. There were no other identifiable systemic symptoms. As a result, he was referred at the age of 16 months to St John’s Institute of Dermatology for psoralen photochemotherapy. The decision was made to apply psoralen topically for 15 min by careful immersion in a bath containing 15 mL of 1Æ2% 8-methoxypsoralen (MOP) in 50 L of water (0Æ00036%). His mother then held him on her knee inside the cubicle. The starting ultraviolet (UV) A dose was 0Æ1 J cm, treatments being administered twice weekly with very small increments of 0Æ1 J cm initially. Two problems were experienced during the treatment course: one mild phototherapy burn at 0Æ5 J cm, leading to a reduction in treatment dose to 0Æ1 J cm after it settled, and one outbreak of symptoms which was probably due to premature discontinuation of the antihistamines. Otherwise, the treatment proceeded smoothly and his symptoms and signs improved over the first 10 treatments. By the completion of 14 treatments (cumulative dose 3Æ76 J cm) his condition had almost completely resolved, requiring only occasional use of oral antihistamines. He had no pruritus, and very little flushing. His skin still showed dermographism but much less dramatically, and the leathery thickening had disappeared. This remission continued until the age of 3 years when his symptoms began to be resistant to conventional therapies once again. At age 3 years 10 months he started another course of psoralen photochemotherapy, this time with oral psoralen, as he was old enough at this stage to take the necessary protective measures before and after UVA exposure. He was given 8-MOP 10 mg and a starting and maximum UVA dose of 0Æ3 J cm. Twelve treatments at this dose gave little benefit, and he was started on a further course of oral psoralen photochemotherapy at the age of 4 years 2 months. This consisted of 19 treatments with a maximum UVA dose of 2Æ0 J cm and a cumulative UVA dose over the three courses of treatment of 24Æ2 J cm. During this last treatment course his psoralen was changed to 5-MOP 20 mg due to nausea and vomiting. He did not otherwise experience any side-effects. The third treatment course was very beneficial and his skin has been relatively well controlled on oral antihistamines only since then. His current skin symptoms at the age of 6 years are flushing around once a week (and of less severity than as a young child), and small patches of urticaria once a day. Psoralen photochemotherapy has been used occasionally in the treatment of childhood mastocytoses for the last 20 years, although the exact mechanism of action has not Table 1 Criteria for the initiation of biweekly a-galactosidase A infusions for Fabry disease in the Netherlands. Patients must have Fabry disease and at least one major or two minor objective criteria