Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare autosomal dominant
palmoplantar keratosis, which manifests in infants and becomes more evident in
adulthood. Its mode of inheritance is autosomal dominant with mutation in loricrin
and Connexin 26 genes. Patients with this mutation present hyperkeratosis of the
palms and soles, constricting bands of the digits, usually on the fifth, and
starfish-shaped hyperkeratosis on the dorsal aspects of the hands and feet. The
disease mostly occurs in white women, where constricting fibrous bands appear on the
digits and can lead to progressive strangulation and auto-amputation
(pseudo-ainhum).The authors report a rare case of a patient with a clinical
ichthyosiform variant of Vohwinkel syndrome.
Psoriasis is a chronic inflammatory disease that directly affects the quality of life. Biologics are prescribed for patients unresponsive to conventional treatments and with severe forms of the disease. Ustekinumab is a fully human monoclonal antibody against the p40 subunit of interleukins 12/23 that is being used with satisfactory responses, achieving an improvement in the baseline Psoriasis Area and Severity Index of approximately 75% after 12 weeks of treatment. It has few side effects, including grater susceptibility to infections and development of reactions to the drug. Our report discusses a case of a cutaneous reaction to the use of ustekinumab in a 27 year-old male patient after the third dose of the medication. No similar case has been reported in the literature.
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