Fibroblastic Rheumatism

Background

This is a rare syndrome that is characterized by multiple cutaneous nodules with symmetric polyarthritis. The cause is unknown.

OUTLINE

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

GENERAL

Fibroblastic rheumatism: case report and review of the literature.

Lee JM, Sundel RP, Liang MG.
Pediatr Dermatol 2002 Nov-Dec;19(6):532-5 Abstract quote
A previously healthy 7-year-old boy presented with polyarthritis and skin lesions. Multiple, skin- to pink-colored, firm papules were noted on the periungual areas, hands, feet, and nose. There was swelling of the proximal interphalangeal joints, wrists, elbows, ankles, and knees.

A skin biopsy specimen revealed dermal fibrosis and interspersed histiocytes and lymphocytes. These findings were consistent with fibroblastic rheumatism, a condition characterized by cutaneous nodules and a symmetric polyarthritis.

He was treated with methotrexate and corticosteroids with improvement in the symptoms of his arthritis and skin lesions. This early treatment was beneficial in our patient.

VARIANTS

ARTHRITIS

The arthropathy of fibroblastic rheumatism.

Romas E, Finlay M, Woodruff T.

University of Melbourne Department of Medicine, St. Vincent's Hospital, Fitzroy, Melbourne, Australia.

Arthritis Rheum 1997 Jan;40(1):183-7 Abstract quote
Fibroblastic rheumatism (FR) is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis.

The unique histologic finding in the skin and synovium of patients with FR is a proliferation of myofibroblast-like cells within a background matrix of collagen. The occurrence of erosive arthritis has not been emphasized in previous descriptions of FR.

We describe a patient with FR who presented with symmetric polyarthritis, skin thickening, and dermal nodules. Despite treatment with prednisone and D-penicillamine, he developed a progressive, destructive polyarthropathy that mimicked multicentric reticulohistiocytosis.

WITHOUT RHEUMATOLOGIC SYMPTOMS

Fibroblastic rheumatism: a case without rheumatological symptoms.

Colonna L, Barbieri C, Di Lella G, Zambruno G, Annessi G, Puddu P.

Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy.
Acta Derm Venereol 2002;82(3):200-3 Abstract quote
Fibroblastic rheumatism is a rare syndrome characterized by the association of multiple cutaneous nodules with symmetric polyarthritis.

We report on a patient who presented a 4-year history of pink to skin-coloured nodular lesions symmetrically localized at para-articular sites without evident rheumatological symptoms. Histopathology of a skin nodule led to the diagnosis of fibroblastic rheumatism showing a poorly circumscribed dermal proliferation of spindle and stellate fibroblast-like cells embedded in thickened collagen bundles with a marked reduction of elastic fibres. X-rays of both hands and feet showed metacarpophalangeal, metatarsalphalangeal and interphalangeal erosions, unexpected by patient history.

This case of fibroblastic rheumatism appears unique in view of the absence of any clinical manifestation of polyarthritis at 7 years from appearance of skin lesions.

HISTOLOGICAL TYPES CHARACTERIZATION

GENERAL

SKIN

Fibroblastic rheumatism: clinical and histologic evolution of cutaneous manifestations.

Fam AG, Hanna W, Mak V, Assaad D.

Division of Rheumatology, Sunnybrook Health Science Centre, Toronto, Ontario, Canada.
J Rheumatol 1998 Nov;25(11):2261-6 Abstract quote
Correlation of the clinical manifestations of a patient with fibroblastic rheumatism with sequential skin biopsy findings revealed an early inflammatory stage with cutaneous nodules, patchy erythematous skin lesions, polyarthritis, dermal lymphomonocytic infiltrates, and fibroblastic and myofibroblastic proliferation, followed by a chronic stage with sclerodactyly, joint ankylosis, deformities and dense dermal fibrosis.

Treatment of this rare disorder in its early active stages may prevent the development of incapacitating joint sequelae.

Fibroblastic rheumatism.

Kanzler MH, Dhillon I, Headington JT.

Division of Dermatology, Santa Clara Valley Medical Center, San Jose, Calif, USA.
Arch Dermatol 1995 Jun;131(6):710-2 Abstract quote
BACKGROUND: Fibroblastic rheumatism was first described in the French literature in 1980. Since that time, 11 other patients with this disorder have been identified in the literature, mostly from France. This is a unique syndrome characterized by the sudden onset of symmetric polyarthritis and cutaneous nodules ranging from 5 to 20 mm in diameter, with predilection for the upper and lower extremities. While the cutaneous findings resolve spontaneously after several months, permanent joint sequelae are common.

OBSERVATIONS: We present the first two patients with fibroblastic rheumatism reported in the United States. The clinical features and histologic findings were identical to those of patients described in the European literature.

CONCLUSIONS: Fibroblastic rheumatism is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis. The clustering of cases in France may, in part, be attributable to increased recognition by clinicians owing to prevalence of reports in the European literature. Perhaps, as clinicians in the United States become familiar with this disorder, further cases will be diagnosed, and the pathogenesis of the disorder will be elucidated.

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES

DERMAL DISORDERS

PROGNOSIS CHARACTERIZATION

GENERAL Variable

TREATMENT CHARACTERIZATION

GENERAL

METHOTREXATE

Preliminary experience with low-dose methotrexate in fibroblastic rheumatism.

Vittecoq O, Mejjad O, da Silva F, Joly P, Thomine E, Lauret P, Thomine JM, le Loet X.

Hopital de Boisguillaume, CHU de Rouen, France.
Arthritis Rheum 1996 Dec;39(12):2070-3 Abstract quote
Fibroblastic rheumatism (FR) is a rare disease characterized by joint manifestations without joint destruction, and associated with cutaneous nodules and sclerodactyly.

The diagnosis is usually based on histologic examination of a skin nodule or synovium specimen. In the 11 previously reported cases, corticosteroid treatment, which has been widely used, was shown to have an unpredictable effect on the evolution of FR.

We report a case of FR that was treated with methotrexate for 5 years, which led to complete recovery. This drug may be considered an effective treatment for FR. Further cases are needed to confirm this preliminary experience.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

Commonly Used Terms

Basic Principles of Disease
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Commonly Used Terms
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Diagnostic Process
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Surgical Pathology Report
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Special Stains
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Internet Links

Last Updated 1/23/2003

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Fibroblastic rheumatism: case report and review of the literature. Lee JM, Sundel RP, Liang MG.	Pediatr Dermatol 2002 Nov-Dec;19(6):532-5 Abstract quote A previously healthy 7-year-old boy presented with polyarthritis and skin lesions. Multiple, skin- to pink-colored, firm papules were noted on the periungual areas, hands, feet, and nose. There was swelling of the proximal interphalangeal joints, wrists, elbows, ankles, and knees. A skin biopsy specimen revealed dermal fibrosis and interspersed histiocytes and lymphocytes. These findings were consistent with fibroblastic rheumatism, a condition characterized by cutaneous nodules and a symmetric polyarthritis. He was treated with methotrexate and corticosteroids with improvement in the symptoms of his arthritis and skin lesions. This early treatment was beneficial in our patient.
VARIANTS
ARTHRITIS
The arthropathy of fibroblastic rheumatism. Romas E, Finlay M, Woodruff T. University of Melbourne Department of Medicine, St. Vincent's Hospital, Fitzroy, Melbourne, Australia.	Arthritis Rheum 1997 Jan;40(1):183-7 Abstract quote Fibroblastic rheumatism (FR) is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis. The unique histologic finding in the skin and synovium of patients with FR is a proliferation of myofibroblast-like cells within a background matrix of collagen. The occurrence of erosive arthritis has not been emphasized in previous descriptions of FR. We describe a patient with FR who presented with symmetric polyarthritis, skin thickening, and dermal nodules. Despite treatment with prednisone and D-penicillamine, he developed a progressive, destructive polyarthropathy that mimicked multicentric reticulohistiocytosis.
WITHOUT RHEUMATOLOGIC SYMPTOMS
Fibroblastic rheumatism: a case without rheumatological symptoms. Colonna L, Barbieri C, Di Lella G, Zambruno G, Annessi G, Puddu P. Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy.	Acta Derm Venereol 2002;82(3):200-3 Abstract quote Fibroblastic rheumatism is a rare syndrome characterized by the association of multiple cutaneous nodules with symmetric polyarthritis. We report on a patient who presented a 4-year history of pink to skin-coloured nodular lesions symmetrically localized at para-articular sites without evident rheumatological symptoms. Histopathology of a skin nodule led to the diagnosis of fibroblastic rheumatism showing a poorly circumscribed dermal proliferation of spindle and stellate fibroblast-like cells embedded in thickened collagen bundles with a marked reduction of elastic fibres. X-rays of both hands and feet showed metacarpophalangeal, metatarsalphalangeal and interphalangeal erosions, unexpected by patient history. This case of fibroblastic rheumatism appears unique in view of the absence of any clinical manifestation of polyarthritis at 7 years from appearance of skin lesions.

Gross Appearance and Clinical Variants
Histopathological Features and Variants
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
SKIN
Fibroblastic rheumatism: clinical and histologic evolution of cutaneous manifestations. Fam AG, Hanna W, Mak V, Assaad D. Division of Rheumatology, Sunnybrook Health Science Centre, Toronto, Ontario, Canada.	J Rheumatol 1998 Nov;25(11):2261-6 Abstract quote Correlation of the clinical manifestations of a patient with fibroblastic rheumatism with sequential skin biopsy findings revealed an early inflammatory stage with cutaneous nodules, patchy erythematous skin lesions, polyarthritis, dermal lymphomonocytic infiltrates, and fibroblastic and myofibroblastic proliferation, followed by a chronic stage with sclerodactyly, joint ankylosis, deformities and dense dermal fibrosis. Treatment of this rare disorder in its early active stages may prevent the development of incapacitating joint sequelae.
Fibroblastic rheumatism. Kanzler MH, Dhillon I, Headington JT. Division of Dermatology, Santa Clara Valley Medical Center, San Jose, Calif, USA.	Arch Dermatol 1995 Jun;131(6):710-2 Abstract quote BACKGROUND: Fibroblastic rheumatism was first described in the French literature in 1980. Since that time, 11 other patients with this disorder have been identified in the literature, mostly from France. This is a unique syndrome characterized by the sudden onset of symmetric polyarthritis and cutaneous nodules ranging from 5 to 20 mm in diameter, with predilection for the upper and lower extremities. While the cutaneous findings resolve spontaneously after several months, permanent joint sequelae are common. OBSERVATIONS: We present the first two patients with fibroblastic rheumatism reported in the United States. The clinical features and histologic findings were identical to those of patients described in the European literature. CONCLUSIONS: Fibroblastic rheumatism is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis. The clustering of cases in France may, in part, be attributable to increased recognition by clinicians owing to prevalence of reports in the European literature. Perhaps, as clinicians in the United States become familiar with this disorder, further cases will be diagnosed, and the pathogenesis of the disorder will be elucidated.

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
DERMAL DISORDERS