integumentary system disorder keratoderma hereditarium mutilans https://github.com/monarch-initiative/mondo/issues/10033 https://github.com/monarch-initiative/mondo/issues/6749 https://github.com/monarch-initiative/mondo/issues/6877 https://www.malacards.org/card/vohwinkel_syndrome UMLS:C0265964 deafness, congenital, with keratopachydermia and constrictions of fingers and toes GARD:0003092 Orphanet:494 Vohwinkel syndrome mutilating keratoderma of Vohwinkel PPK mutilans and deafness KHM A rare, diffuse, mutilating, hereditary palmoplantar keratoderma disorder characterized by severe, honeycomb-pattern palmoplantar keratosis and pseudoainhum of the digits leading to autoamputation, associated with mild to moderate congenital sensorineural hearing loss. Additional features include stellate keratosis on the extensor surfaces of the fingers, feet, elbows and knees. Alopecia, onychogryphosis, nail dystrophy or clubbing, spastic paraplegia and myopathy may also be associated. keratoderma hereditarium mutilans MESH:C536457 ICD9:757.39 MONDO:0007422 SCTID:24559001 MEDGEN:78579 OMIM:124500 mutilating keratoderma VOWNKL DOID:0111339 mutilating keratoderma plus deafness GJB2-related disorder