|IMAGES IN RHEUMATOLOGY
|Year : 2017 | Volume
| Issue : 4 | Page : 223-225
Multicentric reticulohistiocytosis: Unusual presentation of an uncommon disease
Jeet Hemant Kumar Patel1, Neeraj Jain1, Lalit Duggal1, Shilpa Garg2, Bhandari Gurbir1, Seema Rao3, Amal Basnet1
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 Publication||16-Nov-2017|
Jeet Hemant Kumar Patel
2208 A, SSRB, Sir Ganga Ram Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
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 2019 Oct 22];12:223-5. Available from: http://www.indianjrheumatol.com/text.asp?2017/12/4/223/211697
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 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].
| Discussion|| |
Multicentric reticulohistiocytosis is a rare disease with approximately 300 reported cases., 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. 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. Multicentric reticulohistiocytosis can involve any joint but has predilection for distal and proximal interphalangeal joints of fingers as in our patient. It is associated with internal malignancies (mostly solid tumors of breast, bronchial, gastric, and cervix) in 15%–27% of cases. However, cancer screening in our patient was negative. As in our case, fibrotic-to-nodular histiocytic infiltration of lung, hepatomegaly with diffuse steatosis, and pericardial effusion  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.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]