Congenital self-healing non-Langerhans cell histiocytosis.
Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). The relationship of the vesicles and pneumonia to the histiocytic disorder is not clear. Ultrastructurally, worm-like (comma-shaped) particles, dense bodies and Birbeck granules were not found. Histiocytes were Leu-6 negative, and S100 (partly), Leu M3 and HLA-DR positive. Positive reactions were also obtained with anti-lysozyme and non-specific esterase. Several aspects of this case and of others described previously are discussed.
Oranje
A P
AP
Department of Dermatology and Venereology (Subdivision of Paediatric Dermatology), Sophia Children's Hospital, Rotterdam, The Netherlands.
Vuzevski
V D
VD
de Groot
R
R
Prins
M E
ME
D005260:Female; D006644:Histiocytes / Q000648:ultrastructure; D006801:Humans; D007231:Infant, Newborn; D008206:Lymphatic Diseases / Q000151:congenital* / Q000473:pathology; D011014:Pneumonia / Q000151:congenital; D012075:Remission, Spontaneous; D012872:Skin Diseases, Vesiculobullous / Q000151:congenital; D014844:Vulva / Q000473:pathology