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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.
[email protected]
Abstract
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.
Introduction
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.
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.
Figure 5
Discussion
Dermal infiltrate
comprising histiocytes,
multinucleate giant cells
with ground-glass
cytoplasm, and
lymphocytes (H&E, x 400).
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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