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.

Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature

“…PNH most commonly affects young to middle-aged adults who present with nonpainful, nonpruritic, widely disseminated, randomly distributed red-brown cutaneous papules and nodules [6]. A gender predilection has not been reported, and there is no syndromic association.…”

“…Case Reports in Pathology and antineoplastic agents like imatinib [6]. Unfortunately for afflicted patients, recurrence may occur after treatment [9].…”

Disfiguring periocular yellow nodules in a normolipemic patient

“…[ 2 ] Ocular involvement in the form of epibulbar infiltrates[ 2 ] and association with Eale's disease has been reported. [ 3 ] Chemotherapy with corticosteroids, methotrexate, imatinib, and vinblastin has been tried with variable success and the disease is often treatment resistant. Disfiguring lesions can be surgically excised but recurrences are not uncommon.…”

Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report

“…recently described the use of Imatinib and Pazopanib (in a patient having PNH with Eale's disease), which had to be discontinued due to lack of response. [15]…”