[Gottron's erythrokeratodermia congenitalis progressiva symmetrica with atypical involvement of nails in the sense of pachyonychia].
Erythrokeratodermia congenitalis progressiva symmetrica Gottron (ECPSG) is a rare hereditary disorder characterized by plaques of hyperkeratosis on an erythematous basis. The onset of the disease occurs predominantly in early childhood. Morphological and histological findings give hint for the diagnosis. Ultrastructural findings as well as HLA type (A2, A9, B18) are reported. ECPSG connected with pachyonychia has been observed for the first time.
D000293:Adolescent; D007644:Darier Disease / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000235:genetics* / Q000473:pathology; D008297:Male; D009264:Nails, Malformed / Q000235:genetics* / Q000473:pathology; D012867:Skin / Q000473:pathology
Erythrokeratodermia congenitalis progressiva symmetrica Gottron mit atypischem Befall der Nägel im Sinne einer Pachyonychie.