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Congenital self‐healing histiocytosis (Hashimoto‐Pritzker): An ultrastructural and immunohistochemical study
Author(s) -
Kanitakis Jean,
Zambruno Giovanna,
Schmitt Daniel,
Cambazard Frederic,
Jacquemier Daniel,
Thivolet Jean
Publication year - 1988
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19880201)61:3<508::aid-cncr2820610316>3.0.co;2-2
Subject(s) - birbeck granules , pathology , histiocyte , immunohistochemistry , medicine , histiocytosis , ultrastructure , langerhans cell , antigen , immunology , disease
Congenital self‐healing histiocytosis (CSHH) is a rare primary histiocytic skin disorder. Only a few cases have been studied by ultrastructure and immunohistochemistry. Here we report a new case that was investigated using an electron microscope and a panel of monoclonal (MCA) and polyclonal (PCA) antibodies. CSHH cells were found to bear the immunohistochemical phenotype of normal epidermal Langerhans cells (LC) and histiocytosis X (HX) cells (CD1a/c+, CD1b‐, CD4+/−, human leukocyte antigen [HLA]‐DR/DQ+, S‐100+). However, an electron microscope showed a paucity of Birbeck granule (BG)‐containing cells. This contrasted with their immunophenotype. This finding, along with other ultrastructural characteristics of CSHH cells, suggests that histologic differences exist between CSHH and HX. However, because no absolute histologic criterion is known that allows unequivocally the differential diagnosis between the two diseases, this distinction currently has to rely on clinical criteria, mainly the regressive course observed within a few months in CSHH. The precise nosologic position of CSHH among other histiocytic syndromes remains unsettled.