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Congenital self‐healing Langerhans cell histiocytosis with persistent cellular immunological abnormalities
Author(s) -
WHITEHEAD B.,
MICHAELS M.,
SAHNI R.,
STROBEL S.,
HARPER J.I.
Publication year - 1990
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.1365-2133.1990.tb14735.x
Subject(s) - langerhans cell histiocytosis , pathology , medicine , histiocytosis , immunohistochemistry , langerhans cell , immunology , antigen , disease
SUMMARY We describe an infant who presented at birth with numerous haemorrhagic and encrusted skin lesions, hepatomegaly, lymphadenopathy, raised hepatic transaminases, leucopenia and thrombocytopenia. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemistry, which demonstrated the presence of CD 1, S‐100 and DR positive cells in the skin infiltrate. The skin lesions resolved spontaneously after 6 weeks but recurred at 3 months and again were self involuting with resolution by 9 months. Persistent circulating T‐cell abnormalities, including T‐cell lymphopenia and the presence and persistence of peripheral blood CD 1 + cells were noted throughout the first year of life.