Endolymphatic non-langerhans cell histiocytosis of the larynx: report of an uncommon disease manifestation.
We report on an uncommon laryngeal non-Langerhans cell histiocytosis. An 11-year-old boy presented with a 6 months history of progressive breath inhibition. Magnetic resonance imaging showed diffuse laryngeal and local lymph node swelling. Histology first resembled sarcoidosis, however, corticosteroids were ineffective. Lymphoma, infection, immunodeficiency, and autoimmune disease were excluded. Six months later, biopsies were repeated, now showing numerous ectatic lymph vessels with clusters of histiocytes bearing stellate extensions and emperipolesis. S100 protein and CD1a were negative. Indomethacin treatment led to a gradual improvement. In conclusion, we observed a nonmalignant non-Langerhans cell endolymphatic reticulohistiocytosis, not fitting into any of the described categories.
Classen
Carl Friedrich
CF
Department of General Pediatrics, University Children's Hospital Rostock, Germany. carl-friedrich.classen@med.uni-rostock.de
Haffner
Dieter
D
Nizze
Horst
H
Möller
Peter
P
Prall
Friedrich
F
D018949:Antigens, CD1 / Q000378:metabolism; D002648:Child; D004710:Endolymph; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D006801:Humans; D007818:Laryngeal Diseases / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D009418:S100 Proteins / Q000378:metabolism
D018949:Antigens, CD1; D009418:S100 Proteins