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Striking cervical lymphadenopathy may occur in sinus histiocytosis with massive lymphadenopathy, also known as Rosai Dorfman Disease. There is a slight male and black predominance.


A small portion of patients may have cutaneous papulonodules and some patients have only cutaneous involvement. The skin lesions may appear as large red/purple non-scaly plaques or as annular lesions resembling GA [JAAD 1997;37;643]. Soft tissue lesions may occur.


Any cutaneous lesion may be excised. Spontaneous resolution may occur. Prednisone and/or cyclophosphamide have been used. Methotrexate worked well in one case with complete resolution in 11 months when accompanied by IL kenalog [JAMA Derm 2014;150;787]. One patient responded to a combination of prednisone 20/d, MTX, 6-thioguanine and leukovorin for 5 months.

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Dermatology Online Journal 18(11)

Anais Brasileiros de Dermatologia

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