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Multiple cutaneous reticulohistiocytomas in a patient with rheumatoid arthritis

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Multiple cutaneous reticulohistiocytomas in a patient with rheumatoid arthritis
Ricardo Vieira, Margarida Robalo Cordeiro, Angelina Mariano, Jose Pedro Reis, Oscar Tellechea, and Americo Figueiredo
Dermatology Online Journal 10 (2): 11

Department of Dermatology, University Hospital, Coimbra, Portugal.


A 64-year-old woman with a long-standing peripheral symmetric polyarthritis with positive rheumatoid serology was evaluated for multiple asymptotic papulonodules of fingers, mentum, lower lip, ears, and eyelids. Histopathologic examination showed a dermal infiltrate composed of histiocytes, multinucleate giant cells with ground-glass cytoplasm, and lymphocytes, suggestive of reticulohistiocytoma. The possibilities of multicentric reticulohistiocytosis with positive rheumatoid serology or coexistence of multiple cutaneous reticulohistiocytomas and rheumatoid arthritis are discussed.


Multicentric reticulohistiocytosis (MR) and multiple cutaneous reticulohistiocytomas (MCR) are rare granulomatous idiopathic disorders included in the reticulohistiocytosis spectrum [1, 2]. The reticulohistiocytoma is the primary skin lesion for both these diseases, and it usually presents as a firm skin-colored, yellow, or red papule or nodule [1]. The onset of multiple lesions without underlying systemic illness is the main feature of MCR, a very uncommon condition with few previous reports in the medical literature [2, 3]. MR is a systemic disorder typically associated with a severely erosive seronegative polyarthritis [4, 5, 6]. Involvement of other organs can occur and may include arterial hypertension, polyadenomegaly, myopathy, pulmonary infiltrates, myocardial infiltration with heart failure, bilateral carpal-tunnel syndrome, ocular manifestations, and neurologic manifestations [6, 7]. The coexistence of MR and autoimmune diseases such as systemic lupus erythematosus and Sjögren syndrome was previously reported [8, 9]. MR can also occur as a paraneoplastic condition [10, 11].

Clinical synopsis

A 64-year-old woman was sent to our department in February 2001 for evaluation of multiple asymptomatic skin-colored and reddish, firm, 2-6-mm papules or nodules localized on the fingers (mainly around the proximal nail folds), mentum, lower lip, ears, and eyelids; these progressively developed for 4 months.

Figure 1 Figure 2
Reddish firm papule in the ear (Fig. 1).
Reddish papules in lower lip (Fig. 2).

Figure 3 Figure 4
Characteristic papules and nodules along nail folds (Fig 3).
Reddish nodules with central erosion (Fig. 4).

The patient suffered since age 56 from an erosive symmetric polyarthritis with a high titer of rheumatoid factor. She was under treatment with methotrexate, sulfasalazine, and oral corticosteroids. Her past medical history included essential arterial hypertension, and in 1995 she had a bilateral carpal tunnel syndrome that was treated by surgical release of the transversal carpal ligament.

An extensive laboratory evaluation, including CBC, serum chemistry profile, serum protein electrophoresis, lipid profile, erythrocyte sedimentation rate, antinuclear antibody test, thyroid function study, and urinalysis, showed no abnormalities except an elevated level of triglycerides (235 mg/dL, normal range: 30-135 mg/dL). An X-ray of both hands showed slight erosions in some proximal interphalangeal joints. An interstitial infiltrate was suspected in a chest X-ray but was not confirmed by the pulmonary high-resolution computed tomography (CT) scan. Skin histopathology showed a dermal infiltrate comprising histiocytes, multinucleate giant cells with ground-glass cytoplasm, and lymphocytes, highly suggestive of reticulohistiocytoma.

Figure 5
Dermal infiltrate comprising histiocytes, multinucleate giant cells with ground-glass cytoplasm, and lymphocytes (H&E, x 400).

No treatment was proposed for the skin condition and the lesions remained stable with no significant disability. On a regular survey every 6 months there is no evidence of underlying malignancy after 12 months of followup.


Multiple acral reticulohistiocytoma nodules and peripheral polyarthritis were present in our patient and are typical findings in MR. However, rheumatoid factor is consistently negative in MR, and arthritis usually occurs with severe erosions and is associated with significant deformity [4, 5]. The absence of involvement of the distal interphalangeal joints and an 8-year period from the onset of the polyarthritis to the onset of skin lesions are not consistent with the diagnosis of MR. Carpal-tunnel syndrome, described in the literature as an extracutaneous manifestation of MR, appears to be related in this case to periarticular changes of the rheumatoid disease.

As a conclusion, the type and evolution of polyarthritis suggest the coexistence of rheumatoid arthritis with multiple cutaneous reticulohistiocytomas. MCR are very rare, and we found no previous references to their association with rheumatoid arthritis. This case seems unique and may enlarge the group of systemic diseases associated with reticulohistiocytocytic disorders.


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