Metastatic melanoma in a patient with Clouston syndrome successfully treated with isolated hyperthermic limb perfusion

Background: Clouston syndrome is an autosomal dominant disorder characterized by nail dystrophy, partial or total alopecia, and hyperkeratosis of the palms and soles. Objective: Although a variety of unusual cutaneous manifestations have been described, the incidence of melanoma in this population is unknown. Methods: This article reports a case of in-transit metastatic subungual melanoma in a patient with Clouston syndrome successfully treated with hyperthermic limb perfusion with melphalan. Results: Six months postperfusion, the patient is doing well, with resolution of the extremity erythema and edema. Followup ultrasonography revealed reduction in size of the eight subcutaneous nodules, with the largest measuring 3.3 cm in maximum diameter, representing nearly a 50% reduction in tumor volume postperfusion. Conclusion: Although melanoma has also been associated with some forms of ectodermal dysplasia, such as ectrodactyly - ectodermal dysplasia - clefting (EEC) syndrome, the incidence of melanoma in patients with Clouston syndrome is unknown. Thus far, to our knowledge, this is the first case report of melanoma arising in a patient with Clouston syndrome.