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 Table of Contents  
Year : 2017  |  Volume : 12  |  Issue : 4  |  Page : 223-225

Multicentric reticulohistiocytosis: Unusual presentation of an uncommon disease

1 Department of Rheumatology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Dermatology, Sir Ganga Ram Hospital, New Delhi, India
3 Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication16-Nov-2017

Correspondence Address:
Jeet Hemant Kumar Patel
2208 A, SSRB, Sir Ganga Ram Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_66_17

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Keywords: Erosive arthritis, histiocytosis, nodular skin lesion

How to cite this article:
Patel JH, Jain N, Duggal L, Garg S, Gurbir B, Rao S, Basnet A. Multicentric reticulohistiocytosis: Unusual presentation of an uncommon disease. Indian J Rheumatol 2017;12:223-5

How to cite this URL:
Patel JH, Jain N, Duggal L, Garg S, Gurbir B, Rao S, Basnet A. Multicentric reticulohistiocytosis: Unusual presentation of an uncommon disease. Indian J Rheumatol [serial online] 2017 [cited 2022 Jun 27];12:223-5. Available from:

A 45-year-old woman developed tingling and numbness of hands and difficulty in raising arms above the shoulder for 1 year. She was diagnosed as a case of compressive cervical neuropathy for which she was operated. Six months back, she developed pain and swelling of small joints of hands, followed by elbow and knee joints. She was diagnosed as rheumatoid arthritis and treated for the same without much relief. Along with the joint involvement, she also developed asymptomatic skin-colored lesions over the nose which spread to involve the entire face, hands, forearms, arms, and upper chest and were waxing and waning in nature. She also noticed photosensitivity and had a history of significant weight loss in 5 months.

General and systemic examination was unremarkable except for the musculoskeletal system. There was restriction in lateral rotation and lateral flexion at cervical spine with tenderness in shoulder joints with restriction in all movements. Synovitis at elbows, wrist, metacarpophalangeal joints, all proximal and distal interphalangeal joints of fingers, knees, and ankle joints was present [Figure 1]a,[Figure 1]b,[Figure 1]c. Dorsiflexion and plantar flexion at ankle joints were restricted. Distal phalanx of all fingers was swollen with rounded erythematous nodular swelling [Figure 1]d. Dermatological examination revealed leonine facies with multiple, discrete, skin-colored to erythematous, firm, nontender, shiny, papules and nodules present all over the face with sparing of periorbital and upper lip. Similar lesions were present on the ala of the nose, nasal mucosa, lower lip, external ears including external auditory meatus, dorsum of tongue, periungual area of fingernails, photoexposed areas of the upper chest, back, dorsum of fingers and hands, and extensor surface of forearms and arms [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d,[Figure 2]e,[Figure 2]f,[Figure 2]g. Possibility of sarcoidosis, lepromatous leprosy, post Kala-azar dermal leishmaniasis, generalized granuloma annulare, mucinosis, mycosis fungoides, and rheumatoid nodules was entertained. Slit-skin smear showed no acid-fast bacilli or Leishman-Donovan bodies. Skin biopsy from chin showed sheets of closely packed histiocytes and numerous multinucleate giant cells involving the entire dermis. The histiocytic cells were large and showed abundant pale eosinophilic “glassy” cytoplasm with few showing cytoplasmic vacuolation [Figure 3]a and [Figure 3]b. The lesion abutted the epidermis forming a nodular bulge and extended into the subcutaneous tissue. These features were in keeping with multicentric reticulohistiocytosis. Being a multisystem disorder, the patient was further investigated. X-ray chest showed pericardial effusion. X-ray of hands showed multiple erosions of carpometacarpal bones with periarticular osteopenia with proximal interphalangeal joint arthropathy and subluxation of the left little finger [Figure 3]c.
Figure 1: (a) DIP joint synovitis, (b) Knee synovitis and (c) Ankle synovitis, (d) Nodular skin lesions over finger pulp

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Figure 2: (a-g) Papulonodular skin lesions

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Figure 3: (a-b) Histopathology of lesions showing reticulohistiocytosis, (c) X-ray hands showing erosive arthritic changes, (d) X-ray chest showing cardiomegaly (pericardial effusion) before and after treatment

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The patient was started on oral prednisolone 40 mg daily, methotrexate 20 mg/week, hydroxychloroquine 200 mg twice a day, and supportive treatment. She experienced improvement in her joint swelling and pain with decrease in the pericardial effusion within 2 months of treatment [Figure 3]d after which steroids were tapered to 10 mg with continuation of rest of the treatment. Skin lesions also improved [Figure 4].
Figure 4: Improved skin lesions after treatment

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  Discussion Top

Multicentric reticulohistiocytosis is a rare disease with approximately 300 reported cases.[1],[2] It is more common in females (2–3:1) between 40 and 50 years of age. The disease usually remits after 7–8 years. Papulonodular skin lesions have predilection for face and hands but can involve any part of the body. Photodistributed lesions are seen in around 15% of cases.[3] In our patient, skin involvement was florid leading to leonine facies along with photosensitivity and photodistributed lesions. Periungual involvement (coral bead) reported in 40% of cases was seen in our patient.[3] Multicentric reticulohistiocytosis can involve any joint but has predilection for distal and proximal interphalangeal joints of fingers as in our patient.[4] It is associated with internal malignancies (mostly solid tumors of breast, bronchial, gastric, and cervix) in 15%–27% of cases.[3] However, cancer screening in our patient was negative. As in our case, fibrotic-to-nodular histiocytic infiltration of lung,[5] hepatomegaly with diffuse steatosis,[6] and pericardial effusion [3] have been described.

Being a rare disease, it lacks clear guidelines for the treatment. Various drugs used include steroids, immunosuppressive, biologics, nonsteroidal anti-inflammatory drugs, bisphosphonates, hydroxychloroquine, leflunomide, sulfasalazine, thalidomide, and minocycline. Our patient responded to oral steroids, methotrexate, and hydroxychloroquine.[2]

We report this case because of its rarity, its atypical presentation with leonine facies and photosensitivity, features mimicking rheumatoid arthritis, and to remind the readers to keep this as a possibility in patients presenting with skin and joint involvement.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sroa N, Zirwas MJ, Bechtel M. Multicentric reticulohistiocytosis: A case report and review of the literature. Cutis 2010;85:153-5.  Back to cited text no. 1
Tariq S, Hugenberg ST, Hirano-Ali SA, Tariq H. Multicentric reticulohistiocytosis (MRH): Case report with review of literature between 1991 and 2014 with in depth analysis of various treatment regimens and outcomes. Springerplus 2016;5:180.  Back to cited text no. 2
Gelmetti C. Non-langerhans cell histiocytosis. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. United States of America: McGraw-Hill; 2012. p. 1795-808.  Back to cited text no. 3
Kaul A, Tolat SN, Belgaumkar V, Mhaske CB. Multicentric reticulohistiocytosis. Indian J Dermatol Venereol Leprol 2010;76:404-7.  Back to cited text no. 4
[PUBMED]  [Full text]  
West KL, Sporn T, Puri PK. Multicentric reticulohistiocytosis: A unique case with pulmonary fibrosis. Arch Dermatol 2012;148:228-32.  Back to cited text no. 5
Yang HJ, Ding YQ, Deng YJ. Multicentric reticulohistiocytosis with lungs and liver involved. Clin Exp Dermatol 2009;34:183-5.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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