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Familial mastocytosis: a clinical, immunophenotypic, light and electron microscopic study
Author(s) -
ANSTEY A.,
LOWE D.G.,
KIRBY J.D.,
HORTON M.A.
Publication year - 1991
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.1365-2133.1991.tb14800.x
Subject(s) - pathology , mast cell , ultrastructure , flow cytometry , hla dr , immunophenotyping , cutaneous mastocytosis , monocyte , immunology , biology , medicine , antigen , systemic mastocytosis
Summary A women and her daugter both developed diffuse cutaneous mastocytosis in early infancy. The immunophenotypes of the cutaneous mast cells in the two cases were shown to be identical: leucocyte common antigen (CD45) and macrophage/monocyte marker EMB11 were both strongly positive, HLA‐DR was positive and CD25 was weakly positive. The peripheral lymphocytes of the patients were analysed by flow cytometry and this revealed that there was no increase in the activation markers HLA‐DR and CD25, contrary to recent claims that lymphocytes have a contributory role in driving mast cell proliferation. Mast cell granules in both cases were found on electron microscopy to have an abnormal ultrastructure. The close similarity between the two cases at clinical, immunophenotypic and ultrastructural levels strongly suggests that genetic factors are important in the development of this type of mastocytosis.