Progressive Nodular Histiocytosis Associated with Eale's Disease

Abstract

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.

Keywords: Eale's disease; non-Langerhans cell histiocytosis; progressive nodular histiocytosis.

Figure 1

Papular and nodular lesions on the face and eyelid

Figure 2

Large nodules seen over the elbows, hand and sole

Figure 3

(a) Dermal tumor with a nodular and diffuse growth pattern. HandE ×20. (b) Spindle-shaped tumor cells arranged in short fascicles. HandE ×40. (c) Storiform pattern. HandE ×100. (d) Histiocytes admixed with lymphocytes. HandE ×400

Figure 4

Histiocytes positive for CD68 immunostain. DAB chromogen ×100