TY - JOUR
T1 - Steatocystoma multiplex and leuconychia in a child with Alagille syndrome
AU - Cambiaghi, S.
AU - Riva, S.
AU - Ramaccioni, V.
AU - Gridelli, B.
AU - Gelmetti, C.
PY - 1998
Y1 - 1998
N2 - Alagille syndrome is a rare autosomal dominant developmental disorder, characterized by congenital paucity of interlobular bile ducts, peculiar facies, posterior embryotoxon, bone abnormalities, and peripheral pulmonary artery stenosis. Cutaneous involvement in this disorder is quite rare and the association of Alagille syndrome with multiple comeclones and cysts has been described only once. Here, we report the clinical and histological findings of a 7-year-old patient affected by Alagille syndrome who presented multiple cystic lesions and comedones reminiscent of steatocystoma multiplex and a congenital total true leuconychia. Although unexplained, the association of this syndrome with a developmental disorder of the pilosebaceous unit may not be fortuitous.
AB - Alagille syndrome is a rare autosomal dominant developmental disorder, characterized by congenital paucity of interlobular bile ducts, peculiar facies, posterior embryotoxon, bone abnormalities, and peripheral pulmonary artery stenosis. Cutaneous involvement in this disorder is quite rare and the association of Alagille syndrome with multiple comeclones and cysts has been described only once. Here, we report the clinical and histological findings of a 7-year-old patient affected by Alagille syndrome who presented multiple cystic lesions and comedones reminiscent of steatocystoma multiplex and a congenital total true leuconychia. Although unexplained, the association of this syndrome with a developmental disorder of the pilosebaceous unit may not be fortuitous.
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U2 - 10.1046/j.1365-2133.1998.02043.x
DO - 10.1046/j.1365-2133.1998.02043.x
M3 - Article
C2 - 9536240
AN - SCOPUS:0031887592
VL - 138
SP - 150
EP - 154
JO - British Journal of Dermatology
JF - British Journal of Dermatology
SN - 0007-0963
IS - 1
ER -