progressive nodular histiocytosis ( pnh ) is a rare normolipemic macrophage disorder first described by taunton et al . , in 1978 . it belongs to a subgroup of non - langerhans cell histiocytosis ( lchs ) , the juvenile xanthogranuloma ( jxg ) family , which includes xanthoma disseminatum ( xd ) , benign cephalic histiocytosis ( bch ) , multiple adult xanthogranuloma ( maxg ) and generalized eruptive histiocytosis ( geh ) . according to weitzman and jaffee , they present positive reaction to factor xiiia , cd68 , cd14 and negative reactions to cd1a and s-100 proteins . these disorders are difficult to categorize , primarily because of the diverse pathological findings and due to overlapping clinical and microscopic features . a 30-year - old gentleman presented to us with complaints of asymptomatic nodular skin lesions all over the body of 10 years duration . at the age of 20 years , he started developing few small skin - colored lesions on the bilateral peri - orbital areas . these lesions continued to increase in size over time and few lesions became pedunculated especially over the calves . along with the appearance of the cutaneous lesions , he also noticed a gradually progressive , bilateral painless loss of vision . he did not complain of itching , watering , photophobia or discharge from the eyes . he was diagnosed to have eale 's disease and underwent laser photocoagulation for the same . he developed premature cataracts of the eyes and was treated with phacoemulsification and posterior capsular intraocular lens implantation in both eyes . prior to presentation to us , he underwent intra - lesional steroid therapy using triamcinolone acetonide in the peri - orbital lesions with good response seen in smaller lesions but no effect on the larger lesions . the lesions at other sites were removed using various modalities like cryotherapy , radiosurgery and surgical excision of the larger , troublesome lesions . cutaneous examination revealed multiple , approximately 30 to 40 , yellowish to skin - colored , firm , non - tender papules and nodules ranging in diameter from 5 to 40 mm , over the face [ figure 1 ] , trunk , extremities , elbows , hands , palms and soles [ figure 2 ] in decreasing order of frequency . these lesions were located almost symmetrically over the peri - orbital area , mostly over the outer canthi , but also over the lower eyelids , leading to ectropion . the smaller lesions on the face were mounted on the skin , while the larger lesions on the calf area were pedunculated . papular and nodular lesions on the face and eyelid large nodules seen over the elbows , hand and sole visual acuity at the time of presentation was 6/18(r ) and 6/60(l ) . the clinical differential diagnoses considered at the time of presentation to us were multiple eruptive dermatofibromas , xanthoma disseminatum and pnh . blood examination revealed hemoglobin of 10.5 gm / dl with a microcytic hypochromic blood picture , total leukocyte count of 7200/mm and a normal differential leukocyte count . fasting lipid profile , renal , total serum proteins and albumin were normal with no reversal of the a : g ratio . a cellular tumor was seen centered in the dermis , composed of sheets of oval to spindle - shaped cells arranged haphazardly , in short fascicles and in a storiform pattern at places [ figures 3a - c ] . the spindle cells had oval to elongated benign looking nuclei with vesicular chromatin and small nucleoli . on immunohistochemistry the cells were positive for cd68 [ figure 4 ] but negative for s-100 , cd34 , cd21 , cd35 and hmb45 supporting a diagnosis of spindle cell histiocytic tumor . dab chromogen 100 based on the history , clinical examination and histopathological correlation , his dermatological disorder was diagnosed as pnh . he was started on tab imatinib 400 mg od and some of the lesions developed encrustation but it was stopped after 5 months as he continued to develop new lesions while on this drug . second - line treatment with pazopanib ( 800 mg od ) was started after stopping tab imatinib , in lines of soft tissue sarcoma . based at the age of the onset of the lesions , areas of the body involved , progression of disease , lab abnormality ( serum lipids ) , it is possible to differentiate pnh from other forms in the jxg family . benign cephalic histiocytosis and jxg usually occur in early childhood , but jxg has also been reported in adults and the lesions have a tendency to resolve spontaneously over a period of time . multiple adult xanthogranuloma is another important differential diagnosis of pnh which has similar features to jxg but occurs in adults . xanthoma disseminatum was ruled out based on the absence of mucosal and systemic involvement and with normal lipid profile as it usually presents with hyperchylomicronemia and hypertriglyceridemia and diabetes insipidus . since the accurate diagnosis of a specific disorder within the jxg subgroup is quite difficult , we made the diagnosis of pnh based on the history , presence of the typical skin lesions , progressive course and histopathological demonstration of spindle cells that were positive on immunohistochemistry for histiocyte marker , cd68 , but negative for s-100 . non - langerhan 's cell histiocytosis disorders are difficult to diagnose and our case presents another clinical expression of this variable and confusing disorder . to our knowledge this is the first case report of progressive nodular histiocytosis associated with eale 's disease .