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Lymphomatoid Papulosis with Antigen Deletion and Clonal Rearrangement in a 4‐Year‐Old Boy
Author(s) -
Hellman Judith,
Phelps Robert G.,
Baral Jim,
Fasy Thomas M.,
Ahern Caitlin M.,
Strauchen James A.
Publication year - 1990
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.1525-1470.1990.tb01072.x
Subject(s) - lymphomatoid papulosis , medicine , southern blot , gene rearrangement , antigen , lymphoproliferative disorders , lymphoma , phenotype , pathology , immunology , gene , biology , genetics , mycosis fungoides
Lymphomatoid papulosis (LyP) is rarely seen in children. We report a case of LyP in a 4‐year‐old boy in whom immunopathologic studies demonstrated T cell antigen deletions. In contrast to all but two previous reports, a T suppressor (CD‐8) phenotype was predominant. Southern blot analysis of DNA isolated from a typical skin lesion indicated a clonal rearrangement of the T cell receptor β gene. Because of a 10% frequency of malignant lymphomas in patients with LyP, long‐term observation is cruciai, especially in chiidren. We recommend routine clonal rearrangement studies for aid in diagnosis and follow‐up, and as possible prognostic indicators in children with this condition.