Progressive nodular histiocytoma

Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. We report a 30-year-old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision. On examination, approximately 30 to 40, skin-colored, firm, non-tender papules and nodules were noted over the body especially on the face and trunk. A skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle-shaped cells, positive for CD68 but negative for S-100, CD34, CD21, CD35 and HMB45, supporting a diagnosis of spindle cell histiocytic tumor. Ophthalmic examination revealed a generalized arteriolar attenuation in both eyes. He received Tab Imatinib 400 mg OD for 5 months followed by Tab Pazopanib 800 mg OD for 4 months and both the drugs were stopped due to lack of any response in the skin lesions. We report this case due to its rarity, characteristic clinical presentation, and its association with Eale's disease. Primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown.

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“…[ 3 ] Very few published reports of PNH exist. [ 4 5 6 7 ] We present a new case of PNH associated with Eale's disease.…”

Section: Introductionmentioning

confidence: 99%

Progressive nodular histiocytosis associated with eale′s disease

et al. 2015

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Unusual variants of non-Langerhans cell histiocytoses

Caputo¹,

Marzano²,

Passoni³

et al. 2007

Journal of the American Academy of Dermatology

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Histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of histiocytes. In addition to the classic variants, the subset of non-Langerhans cell histiocytoses comprises rare entities that have more recently been described. These last include both forms that affect only the skin or the skin and mucous membranes, and usually show a benign clinical behavior, and forms involving also internal organs, which may follow an aggressive course. The goal of this review is to outline the clinical, histologic, and ultrastructural features and the course, prognosis, and management of these unusual histiocytic syndromes.

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Solitary spindle-cell xanthogranulomaof the eyelid

DeStafeno

Meyer

2002

Ophthalmology

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Progressive nodular histiocytoma

Cited by 11 publications

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Progressive nodular histiocytosis associated with eale′s disease

Progressive nodular histiocytosis associated with eale′s disease

Unusual variants of non-Langerhans cell histiocytoses

Solitary spindle-cell xanthogranulomaof the eyelid

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