title: Metastatic melanoma in a patient with Clouston syndrome successfully treated with isolated hyperthermic limb perfusion.
abstract: 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.
  
  
  CONCLUSIONS
  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.
mesh_terms: D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D010478:Chemotherapy, Cancer, Regional Perfusion / Q000379:methods*; D004476:Ectodermal Dysplasia / Q000150:complications / Q000235:genetics*; D005260:Female; D006801:Humans; D006979:Hyperthermia, Induced; D035002:Lower Extremity; D008545:Melanoma / Q000150:complications / Q000235:genetics / Q000556:secondary / Q000628:therapy*; D008558:Melphalan / Q000627:therapeutic use; D009364:Neoplasm Recurrence, Local / Q000628:therapy*; D012878:Skin Neoplasms / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000150:complications / Q000235:genetics / Q000556:secondary / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D018906:Antineoplastic Agents, Alkylating; D008558:Melphalan