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Pachyonychia congenita Jackson‐Lawler type: a distinct malformation syndrome
Author(s) -
CLEMENTI M.,
STEFANI E.CARDIN,
ROSSI C.DEI,
AVVENTI V.,
TENCONI R.
Publication year - 1986
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1986.tb02829.x
Subject(s) - hyperhidrosis , dermatology , hyperkeratosis , medicine , keratosis , dyskeratosis congenita , dyskeratosis , pathology , biology , telomere , genetics , dna
SUMMARY A family with three members in two generations affected by pachyonychia congenita, hyperkeratosis and hyperhidrosis of the palms and soles, follicular keratosis, neonatal teeth and epidermoid cysts (Jackson‐Lawler syndrome) is described. The nosological autonomy of this condition is proposed and a further heterogeneity is suggested on the basis of histopathological changes in the subcutaneous cysts.

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