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Case Reports
. 2023 Jan 16;11(2):456-463.
doi: 10.12998/wjcc.v11.i2.456.

Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

Ping-Ping Liu  1 , Zong-Wen Shuai  1 , Li Lian  1 , Kang Wang  2
Affiliations
Case Reports

Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

Ping-Ping Liu et al. World J Clin Cases. .

Abstract

Background: Multicentric reticulohistiocytosis (MRH)/systemic lupus erythematosus (SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.

Case summary: A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.

Conclusion: MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.

Keywords: Case report; Cyclophosphamide; Multicentric reticulohistiocytosis; Systemic disorder; Systemic lupus erythematosus.

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Conflict of interest statement

Conflict-of-interest statement: All authors declare that they have no conflict of interest.

Figures

Figure 1
Figure 1
The patient was admitted to the hospital with new skin nodules on the distal interphalangeal of the hands and feet and a mass over the right sternoclavicular joint. A and B: Papulonodular lesions over the interphalangeal joint of the both hands; C: Feet; D: Right sternoclavicular joint. Scale bar = 50 μm.
Figure 2
Figure 2
X-ray of both hands showed bone erosion at the distal interphalangeal joints with appearance of a pencil-in-cup deformity and articular soft tissue swelling.
Figure 3
Figure 3
Immunohistochemical staining (with 40 × magnification). A: Hematoxylin-eosin stain; B: Acid-fact stain; C: Positive for CD68; D: Negative for S100). Scale bar = 50 μm.
See this image and copyright information in PMC

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