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Progressive Cardiomyopathic Lentiginosis: Report of Six Cases and One Autopsy
Author(s) -
RuizMaldonado Ramon,
Trevizo Lourdes,
Tamayo Lourdes,
Rios Miguel F.,
Skurovich Mario,
Carrillo Joaquin,
Dominguez Dagoberto,
Castillo Victoria
Publication year - 1983
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1983.tb01106.x
Subject(s) - medicine , short stature , genitourinary system , autopsy , dermatology , pathology
Progressive Cardiomyopathic lentiginosis (PCL) is a syndrome with progressive multisystem abnormalities. Lentigines, the cutaneous hallmark, are usually present from early age and are of utmost diagnostic value. Six children with PCL arc reported. The most constant diagnostic features in this age group were multiple cutaneous lentigines, structural cardiac anomalies, and electrocardiographic alterations. Less constant findings were neurosensory deafness, short stature, and osseous, ocular, and genitourinary abnormalities.