necrobiotic xanthogranuloma ( nxg ) was first described as a separate entity by kossard and winkelmann in 1980 .
the disease is characterized by multiple , plaque- like , yellow - brown lesions , with a predilection for the periorbital area ( spectacle - like distribution ) .
about 100 cases have been reported in world literature . to our knowledge , this is the first case of isolated cutaneous nxg being reported from india .
a 65-year - old female presented with a 3-year history of gradually enlarging , periorbital , asymptomatic lesions .
however , it recurred in a few months and was accompanied by new larger nodules .
the patient 's medical history was not pertinent.on examination , there were multiple , yellowish brown , firm nodules distributed symmetrically in the periorbital area .
the size of the nodules varied from 3 to 7 cm in their widest dimensions .
the surface showed a few telangiectasias and mild atrophy [ figures 1 and 2 ] .
yellow brown firm periorbital nodules superficial telangiectasias and mild atrophy with this history and clinical findings , a provisional diagnosis of a xanthomatous disorder was made .
histopathological examination of a surgical wedge biopsy showed ill - defined large foci of dense granulomatous infiltrate of large histiocytes and histiocytic foamy cells with lymphocytes and few plasma cells .
several histiocytic giant cells resembling touton giant cells were present [ figures 3 and 4 ] . however , typical findings of necrobiosis were not seen .
dense granulomatous infiltrate of histiocytes laboratory investigations were normal for blood counts , electrolytes , glucose levels , lfts , except for s.triglycerides and s.cholesterol levels which were mildly elevated .
immunohistochemistry was positive for cd 68 [ figure 5 ] and negative for cd 1a and s100 .
thus , a final diagnosis of isolated cutaneous nxg without paraproteinemia or associated disorders was made .
the patient was maintained on a regular follow - up of every 15 days for the first 2 months and then once a month for 9 months .
nxg is a rare , progressive , histiocytic disease that features destructive cutaneous lesions , a close association with paraproteinemia , and multisystem extracutaneous manifestations .
nxg is a disease of adults with an average age of onset in the sixth decade .
nxg begins as papules and nodules that slowly enlarge into indurated plaques ranging from 0.5 to 25 cm .
characteristic lesions have a red - orange or yellow color as well as telangiectasias , atrophy , scarsand ulcers .
the most common site of involvement is the face , particularly the periorbital region ( 85% of cases ) ; the trunk and proximal extremities are other affected sites .
ophthalmological complications affect approximately 50% of cases and include orbital masses , conjunctival involvement , keratitis , scleritis , ectropion , uveitis and blindness .
nxg may involve other extracutaneous sites , including the heart , lungs , kidneys , liver , spleen , intestines , skeletal muscle and central nervous system .
paraproteinemia is closely associated with nxg , with approximately 80% of patients demonstrating a monoclonal gammopathy of igg- on serum protein electrophoresis ; furthermore , 10% of these patients develop multiple myeloma .
hepatosplenomegaly , lymphadenopathy , an increased erythrocyte sedimentation rate , leukopenia , hypocomplementemia , thrombocytopenia and cryoglobulinemia are other common findings .
theories regarding pathogenesis include deposition of immunoglobulins and lipid complexes with a foreign body giant - cell reaction and monocyte activation with intracellular lipid accumulation .
histopathological examination shows a palisading xanthogranuloma with areas of necrobiotic , degenerated collagen that infiltrates the mid - dermis with extension into the subcutaneous fat .
multiple xanthomatized histiocytes , touton giant cells , other bizarre giant cells , cholesterol clefts and lymphoid follicles are often present within granulomas .
improvement in skin lesions has been seen with low - dose chlorambucil , melphalan with or without prednisolone , methotrexate , localized radiotherapy , systemic glucocorticoids , interferon -2a , and plasmapheresis with hydroxychloroquine . topically nitrogen mustard , bncu , intralesional corticosteroids and intravenous immunoglobulin have also been tried .
surgical excision has also been advocated . in our patient , we decided to surgically excise the lesions in view of absence of systemic involvement .
 necrobiotic xanthogranuloma ( nxg ) is a very rare , progressive variant of non - langerhans cell histiocytosis . 
 it is known to be associated with multisystem involvement and paraproteinemias . a 65-year - old female presented with chronic , slowly growing , asymptomatic periorbital nodules . 
 the lesions had recurred after local excision elsewhere . 
 no systemic involvement or paraproteinemias were detected . 
 a provisional diagnosis of isolated cutaneous nxg was made which was confirmed by histopathology and immunohistochemistry staining . 
 the lesions were surgically excised with excellent cosmetic and functional results . 
 there was no recurrence over a period of 9 months . 
 to our knowledge , this is the second case of nxg reported from india and the first without any systemic manifestations . 
Introduction
Case Report
Discussion