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reticulohistiocytosis is a spectrum of disorders ranging from solitary and diffuse cutaneous forms without systemic involvement to multicentric reticulohistiocytosis ( mr ) .
the primary skin lesion , reticulohistiocytoma , usually presents as a firm , skin colored , yellowish or reddish papule or nodule . in multiple cutaneous reticulohistiocytoma ( mcr )
, there is onset of multiple lesions without underlying systemic illness , whereas in mr there are extensive skin lesions in association with a severe , often destructive arthropathy , and systemic features .
mcr is more common in young adult males and mr in middle aged women associated with systemic symptoms .
both the variants are characterized in histo - pathology by the presence of large mononucleated or multinucleated histiocytes with an abundance of eosinophilic , homogenous to finely granular cytoplasm having a ground glass appearance .
we report here a case of mcr in a middle aged female for its rarity and the importance of ruling out mr in such a patient .
a 35-year - old female presented with gradual onset of multiple skin colored , asymptomatic pea sized swellings , which started with face and gradually involved back , arms , and legs .
there was no history of preceding local trauma , discharge , pain in these lesions .
there were no constitutional symptoms like fever , joint pain , and complaints referable to other systems .
the patient 's main concern was regarding the cosmetic appearance as lesions were present over the face .
her family history was negative and she had no history of any other chronic illness .
mucocutaneous examination revealed multiple ( 20 in number ) , discrete , skin colored nodules , 12 cm in size scattered over her face , back , abdomen , thighs , and lower legs [ figure 1 ] .
her complete blood count along with general blood picture , liver , and renal function tests , fasting lipid profile and urinalysis were normal .
rheumatoid factor and antinuclear antibodies were found to be within normal limits . on the basis of history and clinical examination , differential diagnosis of xanthoma , reticulohistiocytoma , sarcoidosis , and histoid leprosy
( a ) multiple discrete skin colored nodular lesions over back ( arrow ) ( b ) discrete skin colored nodular lesions over face , ( c ) discrete skin colored nodular lesions over back , ( d ) discrete skin colored nodular lesions over posterior aspect of left thigh and leg excisional biopsy of a nodule over the trunk was done and histopathological examination revealed circumscribed large focus of diffuse dense infiltrate of large histiocytes and histiocytic giant cells with scattering of lymphocytes and a few neutrophils [ figure 2 ] .
several histiocytic giant cells resembling the touton giant cells were also seen [ figure 3 ] .
overlying epidermis showed flattening and thinning and was covered by parakeratosis containing collection of neutrophils . based on clinical and histopathological findings a diagnosis of multiple cutaneous reticulohistocytoma
the nature of the disease was explained to the patient and was referred to the plastic surgeon for the cosmetic correction of the facial lesion .
skin biopsy showing lymphocytic infiltrate with histiocytes and giant cells [ h and e , 100 ] skin biopsy showing multinucleated giant cells ( arrow ) with an amorphous eosinophilic cytoplasm [ h and e , 400 ]
non - langerhans cells histiocytosis represents a broad group of different disorders characterized commonly by proliferation of histiocytes other than langerhans cells .
mcr represents a unique pattern in the spectrum of the reticulohistiocytoses , characterized by histiocytic proliferations of the skin and soft tissues .
it may represent an abnormality histiocytic reaction to different stimuli . local trauma may play a role in the pathogenesis of solitary cutaneous histiocytoma , whereas in diffuse forms , the association with internal malignancies and autoimmune diseases suggest an immunologic basis .
reticulohistiocytomas are usually solitary lesions of less than 1 cm in diameter . however , multiple lesions and large reticulocytomas have been rarely reported .
histopathology of reticulohistiocytoma often shows mid - dermal infiltration of mononuclear histiocytes and multinucleated histiocytes with a ground - glass appearance and a variable number of vacuolated , spindle shaped , and xanthomatized mononuclear histiocytes .
vimentin is universally positive but all other markers like s-100 , desmin , and smooth muscle - specific actin remain negative .
pulsed dye laser , oral corticosteroids , and methotrexate has been tried in the treatment of extensive lesions .
the only concern in our patient was regarding the physical appearance of the lesions as they were also present over the face .
however , patient of mcr needs to be followed up regularly for long time to look for the development of mr which has various life threatening systemic complications .
this case thus lays emphasis on considering mcr in the differential diagnosis of asymptomatic skin colored papulo - nodular lesions and adequate systemic evaluation and follow up of such patients .
multiple cutaneous reticulohistiocytoma ( mcr ) and multicentric reticulohistiocytosis ( mr ) are rare , idiopathic histiocytic granulomatous disorders presenting in a spectrum . a 35-year - old female presented with multiple , firm , 
 discrete , asymptomatic nodules , 1 - 2 cm in size over face , back , abdomen , thighs , and legs . 
 there were no systemic symptoms . 
 histopathology of a nodule over trunk showed diffuse , dense infiltrate of large histiocytes , and histiocytic giant cells . 
 the histiocytes had rounded vesicular nuclei and abundant pink homogenously stained ground glass cytoplasm . 
 the diagnosis of mcr was made . 
 systemic evaluation did not reveal any abnormality . 
 considering the cosmetic appearance of facial lesions , patient was referred to the plastic surgeon . 
 no treatment was advised for rest of lesions and patient was asked to review every 6 months or if any new complaints develop .