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Hydroxychloroquine Quiets Follicular Mucinosis

Summary and Comment |
September 3, 2010

Hydroxychloroquine Quiets Follicular Mucinosis

  1. Mark V. Dahl, MD

A study in a small group of patients points to a definitive cure.

  1. Mark V. Dahl, MD

Until now, no agent or approach has been identified as the treatment of choice for follicular mucinosis. The condition is characterized by localized pink skin and scaling, with alopecia in hair-bearing areas. In about 50% of these patients, skin biopsy specimens show evidence of a clonal T-cell–receptor gene rearrangement. Such a finding can suggest an evolving T-cell lymphoma, especially in patients with lesions in areas beyond the head and neck. Happily, progression to clinical T-cell lymphoma and death is uncommon. However, the plaques tend to persist and do not respond well to treatment with topical steroids.

These investigators report the effects of hydroxychloroquine therapy in six patients with biopsy-proven follicular mucinosis. The patients received 200 mg three times daily for 10 days, followed by a lower dose based on lean body weight (usually, 200 mg twice daily). All six patients responded within 6 weeks. Hair began to grow again in bald areas, and complete remission of lesions occurred in all patients after 2 to 5 months. The dosage was tapered, and treatment was eventually discontinued altogether (except in one patient who was free of lesions but feared relapse). No patients had a recurrence after 3 to 14 years follow-up, and none developed clinical lymphoma.

Comment

Response is one thing; cure is another. These six patients were cured. They had no residual skin lesions and no systemic disease. Only two patients were assessed for a T-cell gene rearrangement, which was identified in one. Hydroxychloroquine will likely be my first choice for treating patients with this curious and ominous disorder.

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