Premium
Congenital self‐healing reticulohistiocytosis – A benign Langerhans cell disease
Author(s) -
SchaumburgLever G.,
Rechowicz E.,
Fehrenbacher B.,
Möller H.,
Nau P.
Publication year - 1994
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.1994.tb00692.x
Subject(s) - birbeck granules , pathology , langerhans cell , electron microscope , histopathology , histiocyte , medicine , epidermis (zoology) , biology , anatomy , immune system , immunology , physics , optics
An‐11‐day old girl was seen with brownish nodular lesions scattered over (he body with emphasis on the face and seal). Several lesions had started lo involute. Tissue was studied by histopathology, immunohistopathology, routine electron microscopy, and immuno‐electron microscopy using cryosubstitution and embedding in K4M. Immunohistopathology revealed that the cells of the dermal infiltrate were Langerhans cells. They expressed Leu 6 and HLA‐DR. On routine electron microscopy no Birbeck granules were (bund in the dermal cells. Birbeck granules in epidermal Langerhans cells were deformed and often situated next to laminated dense bodies. The latter expressed Leu 6 and HLA‐DR. on immuno‐electron microscopy. It was concluded that congenital self‐healing reticulohistiocytosis is a benign Langerhans cell disease in which Birbeck granules are transformed to laminated dense bodies and possibly degraded by lysosomal enzymes.