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Year : 2020  |  Volume : 4  |  Issue : 2  |  Page : 176-178

Multicentric reticulohistiocytosis with pure cutaneous involvement

1 Department of Dermatology, Sparsha Skin Care Clinic, Mandya, Karnataka, India
2 Department of Dermatology, Sapthagiri Institute of Medical Sciences and Research Center, Bengaluru, Karnataka, India
3 Department of Dermatology, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India

Date of Submission17-Jun-2019
Date of Acceptance01-Dec-2019
Date of Web Publication18-Aug-2020

Correspondence Address:
Basavapura Madegowda Shashi Kumar
Shashi Kumar, Sparsha Skin Care Clinic, #1301, 2nd Cross, Nehru Nagar, Mandya - 571 401, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/CDR.CDR_23_19

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Multicentric reticulohistiocytosis is a rare type of non-Langerhans cell histiocytosis. The typical lesions include skin involvement with erosive arthritis. Diagnosis is confirmed by the presence of histiocytes and multinucleated giant cells. About 15%–30% of cases are associated with malignancy. Here, we report a case of an 18-year-old boy who presented with sudden onset of brown asymptomatic lesions on the face and back of 2 months' duration, which on biopsy showed dense infiltrate of histiocytes and giant cells. However, he did not have any symptoms of systemic involvement and all routine investigations were normal. The lesions have not progressed on 1 year of follow-up.

Keywords: Multicentric reticulohistiocytosis, non-Langerhans cell histiocytosis, pure cutaneous

How to cite this article:
Shashi Kumar BM, Savitha SA, Kirti K. Multicentric reticulohistiocytosis with pure cutaneous involvement. Clin Dermatol Rev 2020;4:176-8

How to cite this URL:
Shashi Kumar BM, Savitha SA, Kirti K. Multicentric reticulohistiocytosis with pure cutaneous involvement. Clin Dermatol Rev [serial online] 2020 [cited 2023 Feb 5];4:176-8. Available from: https://www.cdriadvlkn.org/text.asp?2020/4/2/176/292467

  Introduction Top

Multicentric reticulohistiocytosis (MRH) is a rare multisystem disorder, the commonly described clinical features being papulonodular lesions on the extremities along with erosive arthritis.[1]

Goltz and Laymon proposed the name in 1954 because of the multifocal systemic nature of the disease.[2] Histopathology is characterized by infiltration with histiocytes and multinucleate giant cells. About 300 cases have been reported in medical literature, and among them, about 15%–20% have been associated with malignancy.[3]

  Case Report Top

We present a case of a healthy 18-year-old boy who developed small brown asymptomatic lesions over the face, arms, and back over 2 months. The lesions were of sudden onset and he had not noticed much of progression. The patient did not have any other systemic complaints. On physical examination, multiple grouped brown-to-skin-colored, nonscaly, firm, and nontender papules about 2–4 mm each were observed over the face, back, and arms [Figure 1]a and [Figure 1]b. Mucosa, periungual area, and joint examination were normal. A 4-mm skin biopsy was performed with the differential diagnosis of verruca plana, epidermodysplasia verruciformis, and verruca plana-like seborrheic keratosis. Dermoscopy showed homogenous pattern with shades of yellow and brown [Figure 2]. Routine hematological laboratory examinations such as complete hemogram, liver function test, thyroid tests, C-reactive protein, and rheumatoid arthritis (RA) factor were within normal limits. Chest X-ray and ultrasound abdomen were unremarkable. On histopathology, circumscribed focus of dense infiltrate of histiocytes and histiocytic giant cells in the papillary dermis with scattering of lymphocytes was noted. The histiocytes showed rounded vesicular nuclei and pale cytoplasm; some had foamy cytoplasm. Occasional histiocytic giant cells with abundant foamy cytoplasm resembling Touton giant cells were seen [Figure 3]a and [Figure 3]b. Immunostains were positive for CD68 and negative for S100 and CD1a. Based on the histopathology report, a diagnosis of reticulohistiocytosis was made. He was prescribed methotrexate at a dose of 15 mg/week. The patient has not noticed any progress of the lesions over the past 1 year.
Figure 1: (a) Multiple grouped brown-to-skin-colored, nonscaly, firm, and nontender papules over forehead. (b) Multiple brownish papules over back

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Figure 2: Dermoscopy of papular lesions showing homogenous pattern with shades of yellow and brown

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Figure 3: (a) Dense dermal infiltrate of histiocytes (H and E, ×10). (b) Histiocytes with occasional histiocytic giant cells (H and E, ×40)

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  Discussion Top

Reticulohistiocytosis belongs to non-Langerhans cell histiocytic disorders characterized by dermal histiocytic infiltration of large eosinophilic histiocytes with a glassy cytoplasm. These classes of disorders are characterized by accumulation of histiocytes that do not represent the phenotype of Langerhans cells.[4] MRH, also known as lipoid dermato-arthritis, has a worldwide distribution, with a female preponderance (60%–75%). It usually begins during the fourth decade of life with isolated polyarthritis (50%), cutaneous lesions (25%), or both concurrently (25%).[5] Cutaneous nodules and distinctive arthritis are the most prominent clinical features. In the early stages, only skin changes may be evident. The exact etiology for this has not yet been elucidated and no consistent effective treatment has emerged to date. The onset of cutaneous lesions is much earlier in our patient who is still in his second decade. Patients with pure cutaneous involvement has been reported earlier by Balachandran et al.[6] The cutaneous lesions described are heterogeneous ranging from papulonodular, plaque-like macular erythematous rash, reddish-brown nodules, photosensitive rash, mucosal lesions, xanthelasma, to periungual telangiectasia. Lesions can be isolated or scattered and can be accompanied by pruritus. Most commonly affected areas are the face, ears, hands, elbows, shoulders, trunk, and lower extremities.[7] About one-third of the patients have mucosal lesions, which was absent in our patient.[5] If there is no skin involvement, then differential diagnosis of MRH is difficult. Serology will be helpful to distinguish it from connective tissue diseases and RA. The skin is commonly considered the primary site for histology evaluation. Early lesions manifest a lymphohistiocytic infiltrate of the dermis with small, sometimes multinucleated, histiocytes. Advanced lesions show the pathognomonic multinucleated giant cells with a ground-glass eosinophilic cytoplasm.[8]

There is a strong positivity of histiocytes for periodic acid–Schiff and the expression of acid phosphatase, nonspecific esterase, lysozyme, vimentin, CD68 and CD45, MAC387, and human alveolar macrophage-56. There is conspicuous negativity for S100 protein, CD1a, and factor XIIIa.[9]

The course and outcome of MRH are unpredictable, and although the disease is often self-limiting, joint deformities remain as nondenying remnants of the disease. We have reported this case for the rarity of pure cutaneous involvement in an earlier age of onset. Regular follow-up will be required to determine if the patient develops other features of MRH. According to a systemic review conducted by Tariq et al., the most reported effective initial disease-modifying anti-rheumatic drugs is methotrexate in combination with prednisone as the first-line therapy.[10] Several treatment regimens have been tried with variable success in MRH. Systemic steroids, cytotoxic drugs such as cyclophosphamide and chlorambucil, and biologics such as etanercept, rituximab, and infliximab have been reported to be effective.[7] Bisphosphonates such as alendronate and zoledronate have been reported to improve both arthritis and skin lesions.[5]

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

Farokhi A, van Vugt RM, Hoekzema R, Nurmohamed MT. Multicentric reticulohistiocytosis: A case report. BMC Res Notes 2018;11:647.  Back to cited text no. 1
Goltz RW, Laymon CW. Multicentric reticulohistiocytosis of the skin and synovia; reticulohistiocytoma or ganglioneuroma. AMA Arch Derm Syphilol 1954;69:717-31.  Back to cited text no. 2
Tirumalae R, Rout P, Jayaseelan E, Shet A, Devi S, Kumar KR. Paraneoplastic multicentric reticulohistiocytosis: A clinicopathologic challenge. Indian J Dermatol Venereol Leprol 2011;77:318-20.  Back to cited text no. 3
[PUBMED]  [Full text]  
Rao AG, Lakshmi TS, Vani V. Multicentric reticulohistiocytosis. Indian J Dermatol Venereol Leprol 2003;69:35-6.  Back to cited text no. 4
[PUBMED]  [Full text]  
Shah SP, Shah AM, Prajapati SM, Bilimoria FE. Multicentric reticulohistiocytosis. Indian Dermatol Online J 2011;2:85-7.  Back to cited text no. 5
[PUBMED]  [Full text]  
Balachandran C, Sabitha L, Acharya S, Ravikumar BC. Multicentric reticulohistiocytosis. Indian J Dermatol Venereol Leprol 1998;64:193-4.  Back to cited text no. 6
[PUBMED]  [Full text]  
Lim K, D'Souza J, Vasquez JB, Vuyyuru S. Looks can be deceiving: A case report on multicentric reticulohistiocytosis successfully treated with rituximab. Cureus 2017;9:e1220.  Back to cited text no. 7
Salisbury JR, Hall PA, Williams HC, Mangi MH, Mufti GJ. Multicentric reticulohistiocytosis. Detailed immunophenotyping confirms macrophage origin. Am J Surg Pathol 1990;14:687-93.  Back to cited text no. 8
Zelger B, Cerio R, Soyer HP, Misch K, Orchard G, Wilson-Jones E. Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. Am J Dermatopathol 1994;16:577-84.  Back to cited text no. 9
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. 10


  [Figure 1], [Figure 2], [Figure 3]


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