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April 2016

Well-Differentiated Syringofibrocarcinoma in a Patient With Clouston Syndrome

Author Affiliations
  • 1Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut
  • 2Department of Podiatric Surgery, Yale University School of Medicine, New Haven, Connecticut
  • 3Department of Pathology, Yale University School of Medicine, New Haven, Connecticut
JAMA Dermatol. 2016;152(4):484-486. doi:10.1001/jamadermatol.2015.4496

An elderly man presented with syringofibrocarcinoma of the foot, which prompted examination and workup that confirmed a diagnosis of Clouston syndrome (hidrotic ectodermal dysplasia).

A man in his early 80s presented after surgical excision of a large tumor on the right dorsal foot. He had a lifelong history of wispy hair on the lateral scalp, short thick fingernails with distal separation, and dystrophic toenails with distal wedge-shaped subungual hyperkeratosis (Figure 1A). His palms and soles had generalized mild hyperkeratosis and discrete large areas of pink papillomatous plaques. His shins had large scales and erythema. He had no eyelid cysts, and the few teeth he retained appeared normal; he had no history of delayed loss of deciduous teeth. His mother, maternal grandmother, sister, and brother reportedly had similar skin, hair, and nail findings. Both the patient and his brother developed tumors on a foot around age 80 years. The present patient’s tumor is shown in Figure 1B. It was treated with surgical excision and fifth-ray resection followed by placement of a split-thickness skin graft and healed well.

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