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Early‐onset sarcoidosis mimicking refractory cutaneous histiocytosis
Author(s) -
Ohga Shouichi,
Ichino Kiyomi,
Urabe Kazunori,
Ishimura Masataka,
Takada Hidetoshi,
Nishikomori Ryuta,
Furue Masutaka,
Hara Toshiro
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21173
Subject(s) - medicine , sarcoidosis , histiocytosis , histiocyte , prednisolone , pathology , langerhans cell histiocytosis , giant cell , bone marrow , vinblastine , dermatology , biopsy , disease , chemotherapy
Abstract A 10‐year‐old female was diagnosed as having early‐onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon‐γ‐nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis. Pediatr Blood Cancer 2008;50:723–726. © 2007 Wiley‐Liss, Inc.