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Multicentric reticulohistiocytosisA cause of dysphagia with response to corticosteroids.

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Multicentric reticulohistiocytosis (MR) is a rare
disorder characterized by the widespread infiltration
of tissue with lipid-laden histiocytes ( 1 ) . The most
disabling feature is polyarthritis, which ultimately may
result in joint destruction. Patient response to corticosteroids has been inconsistent and difficult to distinguish from naturally occurring spontaneous remission.
We describe a patient with MR whose articular and
extraarticular symptoms responded dramatically to
corticosteroids on 2 separate occasions.
Case report. A 38-year-old black woman was
first admitted to St. Vincent’s Hospital and Medical
Center in January 1983, because of progressive weakness and pain in the right hand associated with decreased function over several months. Several weeks
prior to admission, she noted the onset of pain and
stiffness in her knees, shoulders, and proximal interphalangeal (PIP) joints of both hands. Several days
before admission, she began to experience dysphagia.
There was no history of fever, skin rash, Raynaud’s
phenomenon, alopecia, visual disturbances, or subcutaneous nodules. She had no history of arthritis, and
the family history was noncontributory.
Physical examination revealed a thin, 38-yearold black woman in no acute distress. Vital signs were
normal except for a rectal temperature of 100°F.
From the Departments of Medicine and Pathology, St.
Vincent’s Hospital and Medical Center, New York, New York.
Peter J. Zeale, MD; David Miner, MD; Stephen Honig,
MD. FACP; Marian Waxman, MD; Harry Bartfield, MD.
Address reprint requests to Stephen Honig, MD, FACP, St.
Vincent’s Hospital and Medical Center, 153 West 11th Street, New
York, N Y 10011.
Submitted for publication January 17, 1984; accepted in
revised form August 21, 1984.
Arthritis and Rheumatism, Vol. 28, No. 2 (February 1985)
Abnormal findings were limited to the joints. She had
diffuse swelling, warmth, and tenderness of the metacarpophalangeal (MCP) joints and the PIP joints of
both hands (the right greater than the left). There was
markedly diminished range of motion of the PIPjoints
bilaterally, and the extension of the elbows was also
limited. No skin nodules were present.
Her hemoglobin value was 11.3 gm, hematocrit
35.6%, and the white blood cell count was 7,300
cells/mm3. The erythrocyte sedimentation rate ranged
from 25-43 mm/hour during her hospitalization.
Roentgenographic studies of her chest and hands
showed normal results, as did the barium esophagram.
The bone scan revealed increased uptake of the radioisotope in the hands, wrists, elbows, sternoclavicular
joints, ankles, and knees, bilaterally. Tests for rheumatoid factor and antinuclear antibodies gave negative
results. An electromyogram of the muscles of both
upper extremities was interpreted as being consistent
with bilateral carpal tunnel syndrome.
The patient was discharged with a presumptive
diagnosis of reflex sympathetic dystrophy of the hands
in association with an undifferentiated connective tissue disease. She was treated with prednisone, 30 mg
twice a day, with prompt and dramatic relief of all
symptoms. Hand strength and range of motion returned to normal. Her dysphagia, the etiology of which
remained uncertain, fully resolved.
She continued to improve over the ensuing 3
months, while the dosage of prednisone was gradually
tapered. However, upon discontinuation of her corticosteroid therapy, the dysphagia and arthritis recurred. Aspirin was prescribed but did not alleviate
her symptoms. She noted an outbreak of painless
nodules over her face and the dorsum of her hands,
and she was readmitted to the hospital, approximately
4 months after her first admission, for further evaluation.
Examination at the time of readmission revealed numerous firm, yellow-brown nodules, ranging
in size from 1.5 cm to about 5 cm, involving the skin of
the nose, nasolabial folds, hands, and elbow areas,
bilaterally. There was swelling, warmth, and tenderness of the MCP, PIP, and distal interphalangeal
joints, bilaterally. There was extensive synovial thickening and limited ability to extend her fingers. Hand
grip strength was markedly diminished.
Barium esophagram again showed normal results as did a manometric study of the esophagus.
However, upper endoscopy revealed a 2-cm nodule on
the posterior wall of the hypopharynx, and below this
area, several 2-3-mm nodules were seen. Biopsies of
the skin nodules from the right elbow area, including
the fascia, were performed, followed by a biopsy of
the pharyngeal nodule.
The biopsy samples revealed nodular aggregates and diffuse collections of large histiocytic cells,
situated in the dermis and subcutaneous fat (Figure 1).
were characterized by abundant cytoplasm’
which was either finely granular and pinkish-glassy or
Figure 2. High-power view of the multinucleated reticulohistiocytes with abundant pink-glassy cytoplasm (hematoxylin and eosin
stained, magnification 40).
Figure 1. Dermal infiltrate of multinucleated reticulohistiocytes
(hematoxylin and eosin stained, magnification x 100).
pale and foamy (Figure 2). The nuclei were round and
Although the infiltrate was principally mononuclear, rare multinucleate histiocytic giant cells were
also readily identified (Figure 3). The cytoplasm of the
histiocytes stained strongly with periodic acid-Schiff
reagent stain, which remained resistant to prior digestion with diastase. Identical histiocytic infiltrates
were found in the biopsy sample of the right elbow
fascia and in the specimen of the nodule from the
posterior pharyngeal wall (Figure 4).
The patient was again started on a regimen of
prednisone, 30 mg twice daily. Within days, her hand
grip strength returned to normal, and her dysphagia
resolved. Over the ensuing weeks, the nodules on her
hands and face began to regress, and many of them
have subsequently disappeared. As of July 1983, the
patient exhibited no evidence of arthritis or skin
nodules, while being maintained with prednisone treatment of 10 mg/day.
Discussion. Multicentric reticulohistiocytosis is
a rare disorder of unknown cause, manifested clinicall Y by PolYarthritis, mucOcutaneOuS nodules, and a
variety of systemic symptoms (1). Because of the
Figure 3. Multinucleate histiocytes with foamy cytoplasm (center)
(hematoxylin and eosin stained, magnification x 400).
rarity of the disease, it remains a difficult diagnostic
and therapeutic problem for the rheumatologist. Early
in the disease course it is frequently misdiagnosed,
since the examination and radiographs may suggest
such entities as gout, rheumatoid arthritis, or psoriatic
arthritis (2). The nodules typically appear months to
years after the onset of polyarthritis, frequently raising
the diagnostic possibility of sarcoidosis or even leprosy (3). The definite diagnosis of MR requires a biopsy
of skin nodules or synovium, which reveals the typical
nodular aggregates of histiocytes with multinucleated
cells showing a ground-glass or foamy cytoplasm (4).
The clinical presentation and histologic findings
in our patient are classic for a diagnosis of MR. The
unusual and interesting aspects of this case are twofold: 1) the patient experienced dysphagia that was
caused by a pharyngeal nodule; and 2) the patient
responded dramatically to corticosteroid therapy on 2
separate occasions.
Mucosal nodular infiltrates are commonly seen
in MR. Barrow and Holubar (1) found them in 52% of
their 33 patients, mostly involving the lips, buccal
mucosa, nasal septum, tongue, and gingiva. Warin et
al(5) reported the finding of typical MR nodules in the
larynx of 1 of their patients. We do not know of any
reports citing the presence of a dysphagia-causing
hypopharyngeal nodule as one of the presenting symptoms in MR. We are confident in attributing our
patient’s dysphagia to the presence of the nodule, in
light of the negative results of the barium swallow and
upper endoscopy and motility studies.
Of additional interest is this patient’s dramatic
response to corticosteroid therapy on 2 separate occasions. Because of the rarity of the disease and its
obscure etiology, there has been no consistent approach to therapy. Response to therapy has been
difficult to assess because of the highly variable nature
of the disease. Various modalities have been used in
treating this disease, including salicylates, corticosteroids, adrenocorticotropic hormone, hydroxychloroquine, nitrogen mustard, cyclophosphamide, azathioprine, chlorambucil, as well as low-fat diets, all of
which have shown variable response (1,2,4,6-8).
Our patient demonstrated a marked response,
in that all symptoms-arthritis,
dysphagia, and the
presence of skin nodules-resolved with steroid therapy. We believe that her response was not part of the
natural remission one may see in this disorder, because her second disease flare and remission subse-
Figure 4. Diffuse mucosal infiltrate of multinucleated reticulohistiocytes in the pharyngeal biopsy specimen (hematoxylin and eosin
stained, magnification X 400).
quent to a second course of therapy were so temporalI p related to the use of steroids.
We have presented a case of MR with distinctive articular and extraarticular symptoms that responded to corticosteroid therapy on 2 separate occasions. The patient presently remains asymptomatic on
tapering doses of steroids.
1. Barrow MV, Holubar K: Muticentric reticulohistiocytosis: a review of 33 patients. Medicine (Baltimore) 48:287305, 1969
2. Gold RH, Metzger AL, Mirra JM, Weinberger HJ, Killibrew K: Multicentric reticulohistiocytosis (lipoid dermato-arthritis): an erosive polyarthritis with distinctive
clinical, roentgenographic and pathologic features. AJR
124:6 10-624, I975
3. Flam M, Ryan S, Mak-Poy GL, Jacobs KF, Nelder KH:
Multicentric reticulohistiocytosis: report of a case with
atypical features and electron microscopic study of skin
lesions. Am J Med 52:841-848, 1972
4. Davies NEJ, Roenigk HH, Hawk WA, O’Duffy JD:
Multicentric reticulohistiocytosis: a report of a case with
histochemical studies. Arch Dermatol 97543-547, 1968
5. Warin RP, Evans CD, Hewitt M, Taylor AL, Price CHG,
Middlemiss JH: Reticulohistiocytosis (lipoid dermatoarthritis). Br Med J 1:1387-1391, 1957
6. Krey PR, Comerford FR, Cohen AS: Multicentric reticulohistiocytosis: fine structural analysis of the synovium
and synovial fluid cells. Arthritis Rheum 17:615-633,
7. Hanauer LB: Reticulohistiocytosis: remission after cyclophosphamide therapy. Arthritis Rheum 15:636-640,
8. Taylor DR: Multicentric reticulohistiocytosis. Arch Dermatol 113:330-332, 1977
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reticulohistiocytosisa, response, multicentric, causes, dysphagia, corticosterone
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