A Middle-Aged Female with Multiple Brownish Plaques and Nodules : A Case Report of Multiple Cutaneous

Non-Langerhans-Cell histiocytosis spectrum of disorders includes multicentric reticulohistiocytosis (MR) and multiple cutaneous reticulohistiocytomas (MCR), which are very uncommon granulomatous conditions. In reticulohistiocytoma, the most common sites of involvement are extensor surfaces, particularly the hands and forearms and the classical lesions are characterised by firm brown or yellow papules, nodules and plaques. As MCR is a very rare granulomatous condition, we report here a middle-aged female a case of MCR and we highlight the significance of considering reticulohistiocytoma in the differential diagnoses of chronic and persistent brownish plaques or nodules on skin.


Introduction
N on-Langerhans-Cell his ocytosis spectrum of disorders include mul centric re culohis ocytosis (MR) and mul ple cutaneous re culohis ocytomas (MCR), which are very uncommon granulomatous condi ons.In re culohis ocytoma, the most common site of involvement are extensor surfaces, par cularly the hands and forearms and the classical lesions are characterised by fi rm brown or yellow papules, nodules and plaques. 1 In MR, skin lesions occur in associa on with a severe, destruc ve, arthropathy, mucosal involvement and other systemic features.MCR cutaneous lesions are histologically iden cal to MR, developing in the absence of arthri s or other systemic lesions. 2 As MCR is a very rare granulomatous condi on, we report here a middle-aged female a case of MCR and we highlight the signifi cance of considering re culohis ocytoma in the diff eren al diagnoses of persistent and chronic brownish plaques or nodules on skin.

Case report
A 30-year-old female presented in Dermatology OPD with the appearance of mul ple brownish coloured pea sized raised skin lesions over extensor aspects of bilateral (b/l) upper and lower limbs associated with itching for 3 years.No history of (h/o) anorexia, weight loss, weakness, pallor, joint pain, ngling sensa on, yellowish discolora on of sclera, shortness of breath, chest pain, epigastric pain, fever, atopy, no personal and family history of tuberculosis.Cutaneous examina on showed the presence of mul ple brownish coloured papules, nodules and plaques over extensor aspects of bilateral upper and lower limb measuring around 0.3cm x 0.3cm, 0.8 cm x 0.8 cm and plaques measuring smallest of 1.5 cm x 0.8cm to largest of 3cm x 2cm respec vely (Figure 1).All mucosae, hair and nails were normal. http://dx.doi.org/10.3126/njdvl.v16i1.19417 On laboratory inves ga ons -Complete blood count, peripheral blood smear, absolute eosinophil count, liver func on test, renal func on test and chest x-ray were normal.While deranged parameters were ESR-34 mm/hour, Cholesterol-239 mg/dl and X-ray bilateral hand showed diff use osteopenia in the distal end of radius, ulna, carpal bones, and juxta-ar cular region of metacarpal and phalanges but no sclero c and ly c lesions were seen.Histopathological examina on showed vaguely nodular collec on of mononuclear his ocytes with plump nucleus and moderate amount of eosinophilic cytoplasm.Foci of foamy, macrophages, intermingled mul -nucleated giant cells some of touton type with prolifera on of fi broblasts and infl ammatory cells such as lymphocytes and occasional neutrophils.Lesional biopsy was histologically sugges ve of re culohis ocytoma (Figure 2).Therapy with oral hydroxychloroquine, tapering dose of oral prednisolone (over a period of 6weeks) and topical tacrolimus 0.1% resulted in complete clearance by 20 weeks leaving behind mild post-infl ammatory hyperpigmenta on (Figure 3a

Discussion
MCR is characterized by prolifera on and diff eren a on of an anomalous his ocy c clone in response to unknown s muli. 3Although the exact pathogenesis is not known but the disease has been regarded as a reac ve his ocytosis.In MCR infec ve causa ve agents have no role, however it is associated with exposure to tuberculosis.This can be evidenced by a study in which, of the total pa ents with MCR, 33% had exposure to tuberculosis, 5% had ac ve tuberculosis and 20% had malignancy. 4No any gene c associa ons has been found. 1R is rarely reported in the literature. 3,5,6istopathology of re culohis ocytoma shows numerous mul nucleated giant cells and oncocy c macrophages showing abundant eosinophilic, fi nely granular cytoplasm with ground glass appearance. 4istology with immunocytochemistry usually confi rms the diagnosis of MCR.Staining with Vimen n and CD45 are posi ve but FXIIIa is nega ve. 4 Other diseases that necessitate to be diff eren ated include sarcoidosis, xanthoma, mastocytosis, leprosy and lymphoma.
Systemic steroid with azathioprine or other immunosuppressive drug (cyclophosphamide, ciclosporin) usually improves the condi on. 5Pulsed dye laser, oral cor costeroids, and methotrexate has been tried in the treatment of extensive lesions. 6

Conclusion
Although re culohis ocytoma is an extremely rare granulomatous condi on.One should consider re culohis ocytoma in the diff eren al diagnoses of persistent brownish plaques or nodules on the skin in any middle-aged female.
Fi nancial disclosure: None.Confl icts of interest to disclosure: None declared.

Figure 1 :
Figure 1: Mul ple brownish coloured papules, nodules and plaques over dorsum of bilateral hands (a) and feet (b).