[Progressive nodular histiocytosis--rare variant of cutaneous non-Langerhans cell histiocytosis].
Progressive nodular histiocytosis is an extremely rare skin disease is clinically characterized by the coincidence of two distinct lesions, namely, superficial xanthomatous papules up to 5 mm and deep nodules and tumors 1-3 cm. Histologically the nodules represent spindle cell xanthogranulomas. We report a 24-year-old women with these findings. The distinction from other non-Langerhans cell histiocytoses, in particular multiple juvenile xanthogranulomas, which may be more likely to show spontaneous remission, is somewhat unclear; patients with progressive nodular histiocytosis usually follow a serious and disfiguring clinical course.
Lüftl
Matthias
M
Hautklinik, Universitätsklinikum Erlangen, Germany.
Seybold
Heidi
H
Simon
Miklós
M
Burgdorf
Walter
W
D000328:Adult; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000145:classification / Q000000981:diagnostic imaging* / Q000473:pathology*; D006801:Humans; D011877:Radionuclide Imaging; D035583:Rare Diseases / Q000000981:diagnostic imaging / Q000473:pathology; D012871:Skin Diseases / Q000145:classification / Q000000981:diagnostic imaging* / Q000473:pathology*