Diffuse fasciitis with eosinophilia: A clinicopathologic study

doi:10.1016/0002-9343(80)90257-0

The American Journal of Medicine

Volume 68, Issue 5, May 1980, Pages 701-709

https://doi.org/10.1016/0002-9343(80)90257-0 Get rights and content

Abstract

Three cases of diffuse fasciitis with eosinophilia are presented, and their clinical, laboratory and histologic follow-up are outlined. Furthermore histologic comparison with scleroderma is attempted and the literature is reviewed. From our findings, we conclude that diffuse fasciitis with eosinophilia is a distinct clinicopathologic entity.

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Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome? (abstract.)
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There are more references available in the full text version of this article.

Cited by (70)

Eosinophilic fasciitis (Shulman's disease): A case series of 11 patients
2010, Revue de Medecine Interne
La fasciite avec éosinophilie, ou syndrome de Shulman, est une maladie rare, de cause inconnue et dont le cadre nosologique n’est pas clairement défini.
Nous rapportons une série rétrospective multicentrique de 11 patients, répartis en sept hommes et quatre femmes, dont une observation pédiatrique, et rappelons les principales caractéristiques de cette maladie à travers une revue de la littérature.
L’âge moyen des patients était de 46 ans. L’induration sous-cutanée représentait le motif de consultation chez tous les malades. Elle était de siège atypique, thoracique, chez deux patients. Aucune atteinte viscérale ni complication hématologique n’a été observée. L’éosinophilie sanguine était absente chez cinq patients. Le diagnostic histologique de la fasciite était confirmé dans tous les cas. L’éosinophilie tissulaire était présente chez sept malades. Avec une corticothérapie, l’évolution était favorable dans huit cas. Une rechute a été enregistrée chez un seul patient chez qui l’évolution s’est faite vers une sclérodermie systémique.
La fasciite avec éosinophilie doit être évoquée devant des myalgies et une induration sous-cutanée touchant les membres, une éosinophilie sanguine, une vitesse de sédimentation élevée et une hypergammaglobulinémie. Le traitement repose essentiellement sur la corticothérapie générale.
Eosinophilic fasciitis or Shulman's disease is a rare condition of unknown etiology.
We report a retrospective case series of 11 patients with eosinophilic fasciitis (seven men and four women, including a single pediatric case) and perform a systematic literature review to determine the main features of this disease.
Mean age of the patients was 46 years. Subcutaneous induration of limbs observed in all the patients was the major presenting symptom. The induration was atypically located in the chest area in two patients. Blood eosinophilia was absent in five cases. Histological fasciitis was demonstrated in all patients and eosinophilic infiltration was present in seven patients. Relapse of subcutaneous induration was observed in only one patient who gradually developed systemic sclerosis.
Diagnosis of eosinophilic fasciitis should be considered in the presence myalgia and subcutaneous induration of limbs, blood eosinophilia and hypergammaglobulinemia. Treatment is based on systemic corticosteroids.
Hypereosinophilia with systemic thrombophlebitis
2005, Human Pathology
A 34-year-old Japanese woman developed subcutaneous induration in the left thigh, then showed extreme eosinophilia, and died of hemorrhagic infarction of the brain. Autopsy revealed endocarditis with eosinophil infiltration and systemic thrombophlebitis, including pulmonary veins and intrahepatic branches of the portal vein. Arterial structure was relatively preserved. She had no clinical history of asthma and had anti–ascarid IgE antibody at postmortem serological examination; thus, her disease does not fulfill the diagnostic criteria of Churg-Strauss syndrome and idiopathic hypereosinophilic syndrome (HES). Her organ involvement is, however, consistent with that of HES; thus, her pathophysiological conditions would resemble those of HES. Systemic thrombophlebitis without arterial lesion in patients with hypereosinophilia has never been reported, and this case would broaden the spectrum of vascular lesions in these patients.
Association of Shulman's syndrome and morphea: A case report [3]
2003, Joint Bone Spine
Association of Schulman's syndrome and Morphea: A case report [3]
2003, Revue du Rhumatisme (Edition Francaise)
Surgical management of eosinophilic fasciitis of the upper extremity
1997, Journal of Hand Surgery: European Volume
Eosinophilic fasciitis is a rare inflammatory disease associated with peripheral eosinophilia, hyper-gammaglobulinaemia and contractures of any joint in the upper extremity. Although conservative treatments are generally advocated, this study reports the results of surgical intervention. Four patients aged from 20 to 48 years underwent fasciectomy followed by oral administration of prednisolone. All presented with contractures of digits, wrist, or elbow due to eosinophilic fasciitis in the upper extremities. Despite one case that required a second operation for recurrence, all patients regained the range of motion of the affected joints a few weeks after surgery. The recovery was much sooner than in previously reported cases treated conservatively, suggesting that surgical management of eosinophilic fasciitis is effective in alleviating symptoms quickly and allows patients to resume activities of daily living sooner.
Eosinophilic fasciitis
1994, Clinics in Dermatology

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^★: Present address: University of Ioannina, Ioannina, Greece.

¹: From the Arthritis and Rheumatism Branch, National Institute of Arthritis, Metabolism and Digestive Diseases; the Laboratory of Pathology, National Cancer Institute; the Clinical Immunology Section, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland.

^†: Present address: St. Jude Children's Research Hospital, Memphis, Tennessee 38101.

²: From the Rheumatology Unit, Evaggelismos Hospital, Athens, Greece.

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Clinical studyDiffuse fasciitis with eosinophilia: A clinicopathologic study

Abstract

Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome? (abstract.)

J Rheum

Diffuse fasciitis with eosinophilia: A new syndrome?

Trans Assoc Am Physicians

Eosinophilic fasciitis: Report of six cases of a newly recognized scleroderma-like syndrome

Arthritis Rheum

Morphea, fasciitis, and scleroderma with eosinophilia: A broad spectrum of disease

Arthritis Rheum

A scleroderma-like syndrome with fasciitis, myositis, and eosinophilia

Ann Intern Med

Fasciitis (eosinophilic) with scleroderma-like changes in the skin

J Fla Med Assoc

Syndrome de Shulman

Ann Med Intern

Scleroderma of Buschke? Eosinophilic fasciitis

Br J Dermatol

Eosinophilic fasciitis: A scleroderma-like illness

JAMA

Clinical study
Diffuse fasciitis with eosinophilia: A clinicopathologic study