Clouston Syndrome Associated With Eccrine Syringofibroadenoma

    Ana Carolina Dias Viana de Andrade, Denise Cardoso Vieira, Olga Maria Oiticica Harris, Matheus Melo Pithon
    TLDR Clouston Syndrome can be linked to rare sweat gland tumors.
    Eccrine syringofibroadenoma is a rare benign tumor originating from eccrine sweat gland cells, typically affecting the extremities of elderly individuals. It can appear as solitary or multiple lesions, with its pathogenesis and differentiation remaining controversial. This condition has been linked to underlying disorders like hypohidrotic ectodermic dysplasias. The document detailed a case report of a patient with Clouston Syndrome who exhibited papules and nodules on the extremities, which were clinically and histologically consistent with eccrine syringofibroadenoma. This case was notable as it was only the fourth instance reported in the literature of such an association with Clouston Syndrome.
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      Clouston’s Syndrome: A Rare Case Report

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      August 2020 in “International Journal of Research in Dermatology”
      Clouston's syndrome is a rare disorder affecting nails, hair, teeth, and skin, caused by a gene mutation, and currently has no treatment, only supportive care.

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      November 2025 in “Indian Dermatology Online Journal”
      A 20-year-old male with Clouston syndrome, characterized by alopecia, nail dystrophy, and palmoplantar keratoderma, developed cutaneous squamous cell carcinoma (SCC) on his right great toe. Despite treatment for chronic paronychia, the condition persisted, leading to the discovery of SCC, which was surgically treated with toe amputation. This case is notable as the first reported instance of Clouston syndrome presenting with cutaneous SCC, emphasizing the importance of monitoring for SCC in patients with chronic paronychia and Clouston syndrome. The patient's family history revealed similar symptoms in multiple relatives, indicating a hereditary pattern.

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      February 2009 in “Journal of the American Academy of Dermatology”
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      November 2019 in “Harper's Textbook of Pediatric Dermatology”
      The document is a detailed medical reference on skin and genetic disorders.