Multicentric reticulohistiocytosis in the course of undifferentiated connective tissue disease

Multicentric reticulohistiocytosis (MRH) is a rare disease with unknown aetiology, characterized by the reactive proliferation of histiocytes in the skin, mucous membrane, subcutaneous tissue, synovium, and the bones. Its clinical symptoms are skin changes with red, brown, or pink papules and nodules. They can be located on the hands, face, trunk, or mucous membranes. The skin changes are typically accompanied with symmetrical arthritis, primarily of minor hand joints, which may lead to joint destruction and in extreme cases, to arthritis mutilans changes. MRH is typically related to rheumatoid diseases or considered to be a paraneoplastic syndrome [1, 2].
Here, we present the case of a woman, aged 39 years, with skin changes observed for the previous 5 months in the form of reddish brown painless nodules located near the minor hand joints, typically at the metacarpophalangeal and proximal interphalangeal joints and on the auricles. These skin changes were accompanied by pain and swelling in the metacarpophalangeal and proximal interphalangeal joints. To determine the diagnosis, a specimen was obtained from the skin changes for a histopathological examination; the obtained image corresponded to MRH. The following treatment was started: methylprednisolone 4 mg every other day and sulfasalazine 3 g/day in divided doses. Further, the patient was referred to our department. On admission to the Rheumatology Department, the described skin changes were still present (Figure 1), while the pain and swelling of the joints had decreased slightly after the applied treatment. The laboratory examination revealed normal erythrocyte sedimentation rate (ESR), normal C-reactive protein (CRP) concentration, benign anaemia (Hb 10.7 g/dl), presence of rheumatoid factor (RF), antibodies against cyclic citrullinated peptide (aCCP), and antinuclear antibodies of titre 1 : 1280 with the presence of the SSA and SSB antibodies. The levels of tumour markers (CEA, AFP, Ca125, and CA19.9) did not exceed the reference values. The chest X-ray did not exhibit changes in the organs. An ultrasonography of the abdominal organs did not reveal any changes. Hand X-ray did not reveal any changes in the osseous structure (Figure 2). Because of the serological profile, despite the absence of symptoms both in the eyes and the oral cavity, Schirmer’s test was performed; a positive result was obtained (right eye 10 mm, left eye 10 mm). Undifferentiated connective tissue disease (UCTD) was...


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