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Progressive nodular histiocytosis: a case report and literature review
Author(s) -
Nofal Ahmad,
Assaf Magda,
Tawfik Ahmad,
Elsayed Noha,
Nofal Eman,
Elnakib Nasser,
Elmosalamy Khaled
Publication year - 2011
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4632.2011.04904.x
Subject(s) - medicine , university hospital , family medicine , library science , computer science
Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder first described by Taunton et al. in 1978. Since then, 14 cases, including that described here, have been reported. Progressive nodular histiocytosis belongs to a subgroup of nonLangerhans cell histiocytoses (LCHs), the juvenile xanthogranuloma (JXG) family, which includes xanthoma disseminatum (XD), benign cephalic histiocytosis (BCH), adult xanthogranuloma, and generalized eruptive histiocytosis (GEH). These disorders seem to overlap and probably represent a spectrum of manifestations deriving from a single underlying process that may take one or more of several clinical forms depending on the patient’s immune status and the triggers of macrophage proliferation. Herein, we present a new case of PNH associated with retinitis pigmentosa and optic atrophy, review the literature, and discuss the diagnostic criteria, the differential diagnosis, and the classification of this rare entity.