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A CASE OF WEBER‐CHRISTIAN DISEASE SUGGESTING CELLULAR IMMUNO‐DEFICIENCY
Author(s) -
Kaneko Fumio,
Ito Shunsuke,
Nagai Morihito
Publication year - 1975
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1975.tb00952.x
Subject(s) - lymphocytopenia , immunology , histiocyte , disease , pathogenesis , etiology , pathology , infiltration (hvac) , medicine , lymphocyte , physics , thermodynamics
Weber‐Christian disease in a 25‐year‐old female was reported. She had recurrent erythematous nodules on the trunk and extremities, with fever, and microscopic examination of the nodules showed panniculitis due to lymphocytic and histiocytic infiltration and necrotizing angiitis. Studies of the peripheral blood showed marked anemia, leucopenia, and especially lymphocytopenia. There was a marked susceptibility to bacterial and viral infections. An increase of serum immunoglobulin level, a marked lymphocytopenia, a rather low percentage of lymphocytes transformed by PHA, and a reversion of the tuberculin reaction from positive to negative were found. She could not be sensitized by DNCB. These findings suggest a deficiency of cellular immunity and it is believed that these immunological abnormalities might be related to the etiology and pathogenesis of Weber‐Christian disease.

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