Woolly hair-palmoplantar keratoderma syndrome
Synonyms: KWWH type IV | Keratoderma with woolly hair type IV | Woolly hair-palmoplantar hyperkeratosis syndrome
Woolly hair-palmoplantar keratoderma syndrome is a very rare hereditary epidermal disorder characterized by hypotrichosis/woolly scalp hair sparse body hair eyelashes and eyebrows leukonychia and striate palmoplantar keratoderma (more severe on the soles than the palms) which progressively worsens with age. Pseudo ainhum of the fifth toes was also reported. Although woolly hair-palmoplantar keratoderma syndrome shares clinical similarities with both Naxos disease and Carvajal syndrome cardiomyopathy is notably absent.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.
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