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Pseudoainhum and autoamputation associated with lamellar ichthyosis

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728 A 6‐month‐old female child born out of first‐degree consanguineous marriage was brought by the parents with complaints of scaling all over the body since birth. The parents gave a… Click to show full abstract

728 A 6‐month‐old female child born out of first‐degree consanguineous marriage was brought by the parents with complaints of scaling all over the body since birth. The parents gave a positive history of collodion membrane at birth which gradually peeled off after 1 month of life. Cutaneous examination revealed large thick brown‐colored adherent plate‐like scales all over the body, ectropion, contracture of fingers, and palmoplantar keratoderma without eclabium. Bilateral fingers and toes showed multiple partial constriction bands around them; a feature suggestive of pseudoainhum [Figures 1 and 2]. The tip of the right middle finger was autoamputated [Figure 1]. The nail plates of few nails were completely replaced by hyperkeratotic scales. Skin biopsy showed marked compact hyperkeratosis with a normal granular layer, mild‐to‐moderate acanthosis, and mild perivascular lymphocytic infiltration. A diagnosis of lamellar ichthyosis was made and the child was started on capsule acitretin 1 mg/kg/day orally.

Keywords: autoamputation associated; lamellar ichthyosis; pseudoainhum; pseudoainhum autoamputation; associated lamellar

Journal Title: Indian Journal of Dermatology, Venereology and Leprology
Year Published: 2017

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