British Journal of Rheumatology 1990;29:145-146
CASE REPORT
EOSINOPHILIC FASCIITIS ASSOCIATED WITH INFLAMMATORY NEUTROPHILIC VASCULITIS BY M. MAGARO*, L. ALTOMONTE*, A. ZOLP, L. MIRONE*, G. MASSIf AND F. FEDERICOt * Department of Internal Medicine, Division of Rheumatology and tInstitute of Pathology, Catholic University, Rome
SUMMARY The authors report a case of eosinophilic fasciitis with an histopathological pattern of inflammatory neutrophilic vasculitis associated with the typical inflammatory infiltrate. The presence of this type of vasculitis which may be observed in the initial forms of scleroderma supports the hypothesis of a considerable overlap between these two entities. KEY WORDS: Vasculitis, Scleroderma, Eosinophilia, Skin.
DISCUSSION Eosinophilic fasciitis may be a variant of scleroderma [6-8] or a distinct disease entity [9, 10]. There are several distinguishing clinical features between eosinophilic fasciitis and scleroderma. The latter is more common in females while eosinophilic fasciitis has been reported to be more common in males [9] or
CASE REPORT A 45-year-old man presented with a 3-month history of progressive swelling and stiffness of his arms and legs, arthralgia and lower extremities weakness. Physical examination revealed a normotensive patient with thickness of the skin over the extremities. There was no cutaneous sclerosis on his face and hands and no evidence of skin atrophy or telangiectasia. The hands were diffusely swollen but without periungual telangiectasia, digital ulceration or calcinosis.The patient did not complain of fever, arthritis or purpura. Laboratory evaluation showed a normal haemoglobin and platelet count, a white blood cell count of 10.3 x 109/l with 24% eosinophils and ESR 40 mm/h. The serum electrolytes, creatine phosphokinase, rheumatoid factor, antinuclear antibody, anti-DNA, anti-ENA and complement were normal or negative. Stool culture and examination of stool for protozoa or helminths were negative. Renal and liver function tests, Submitted 9 February; revised version accepted 4 June 1989. Correspondence to Prof. M. Magaro, Istituto di Clinica Medica, Divisione di Reumatologia, Largo A. Gemelli 8, 00168 Roma, Italia.
FIG. 1.—Severe leucocytoclastic vasculitic lesions with vessel wall damage (H & E x 55). 145
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Glasgow on June 24, 2015
electrocardiogram, oesophageal manometry, spirometry and hand and chest X-ray studies were also normal. Electrophoresis revealed a considerable IgG fraction (2110mg/dl). An en bloc biopsy of the skin, subcutis, fascia and muscle was performed at a clinically involved area. Pathological changes were dominated by dermal sclerosis which extended deeply into the subcutaneous tissues. A perivascular lymphocytic and eosinophilic infiltrate in the lower subcutis and in the mid dermis was present. The aponeurotic fascia appeared thickened and contained a significant inflammatory infiltrate composed of lymphocytes, plasma cells and eosinophils. Lymphocytes and a large number of eosinophils and plasma cells were also present around blood vessels and striated fibres in the muscle biopsy specimen. Neutrophils and nuclear debris were present in and about the walls of damaged vessels, creating a picture of neutrophilic vasculitis with an apparent leucocytoclastic pattern. The patient was treated with an initial dosage of 24 mg 6-methylprednisolone. After 2 weeks of steroid treatment, peripheral eosinophilia and hypergammaglobulinaemia normalized with notable clinical improvement.
200 cases of eosinophilic fasciitis have been reported since Shulman's [1] initial description in 1974. It is a scleroderma-like disease characterized by symmetrical, usually widespread inflammation and sclerosis of the deep fascia, subcutis and dermis. It primarily involves the extremities and is associated with peripheral and tissue eosinophilia, hypergammaglobulinaemia and steroid responsiveness [2]. The syndrome was distinguished clinically from progressive systemic slerosis by the absence of Raynaud's phenomenon, ulcerating fingertips, telangiectasia and visceral involvement and pathologically by abnormal histopathological involvement of lower subcutis and fascia [2]. Recent reports have observed an association between eosinophilic fasciitis and morphoea [3] and an overlap between eosinophilic fasciitis and progressive systemic sclerosis [4]. The possibility that eosinophilic fasciitis may evolve into progressive systemic sclerosis has also been suggested [5, 6]. We report a case of typical eosinophilic fasciitis in which an inflammatory neutrophilic vasculitis was found. OVER
146
BRITISH JOURNAL OF RHEUMATOLOGY VOL. XXIX NO. 2 oderma. Thus it is well known that progressive systemic sclerosis or localized scleroderma may show neutrophilic vasculitis in early biopsy specimens [17]. In conclusion, eosinophilic fasciitis seems to be a scleroderma-like disease which primarily involves the subcutis and fascia in the limbs and is as clinically benign as localized scleroderma. This case underlines the pathogenetic importance of vasculitic damage in the first phase of the syndrome.
:
P^^'/C^--:•--'
REFERENCES
1. Piette JC, Herson S. Fasciite avec eosinophilie (mal-
-
*
•
-
FIG. 3.—High magnification of Fig. 2.
equal in both sexes [10]. There have been reports of Raynaud's phenomenon in only isolated cases of eosinophilic fasciitis [11] while it is a common finding in scleroderma. Renal disease and accelerated hypertension have never been reported in eosinophilic fasciitis [12]. In recent years there have been reports noting a considerable overlap in the clinical picture and in the microscopic and laboratory findings of patients with eosinophilic fasciitis and scleroderma [7, 13]. Oesophageal, pulmonary and cardiac involvement have been reported in eosinophilic fasciitis, strictly comparable with those observed in progressive systemic sclerosis [5, 14-16]. Moreover, the possibility that eosinophilic fasciitis may be associated with morphoea [3] or may evolve to progressive systemic sclerosis [5, 6] has been suggested. Histopathological findings support the hypothesis of a close association between eosinophilic fasciitis and progressive systemic sclerosis. Except for the eosinophilia and the deeper involvement, collagen homogenization, tissue atrophy and an infiltrate composed of lymphocytes and plasma cells in ebsinophilic fasciitis resemble those observed in scleroderma. Tissue eosinophilia may also be noted in scleroderma specimens [7]. The neutrophilic inflammatory vasculitis observed in our patient with a typical eosinophilic fasciitis corroborates the hypothesis of a strict overlap between eosinophilic fasciitis and scler-
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Glasgow on June 24, 2015
FIG. 2.—The aponeurotic fascia thickened and containing an inflammatory infiltrate with eosinophils.
adie de shulman). Ann Dermatol Venereol 1986;113:1135-7. 2. Moutsopoulos HM, Webber BL, Paulidis NA, Fostiropoulos G, Goules D, Shulman LE. Diffuse fasciitis with eosinophilia. A clinicopathologic study. Am J Med 1980;68:701-9. 3. Caperton EM, Hathaway DE, Dehner LP. Morphea, fasciitis and scleroderma with eosinophilia: a broad spectrum of disease. Arthritis Rheum 1976;19:792-3. 4. Doyle JA. Eosinophilic fasciitis: extracutaneous manifestations and associations. Cuds 1984;34:259-61. 5. Frayma RA, Atiyah F, Karam P, Ahmed ZA, Salman SM. Eosinophilic fasciitis terminating as progressive systemic sclerosis in a child. Dermatologica 1985;171:291-4. 6. Urbano-Marquez A. Eosinophilic fasciitis evolving into scleroderma (letter). Ann Intern Med 1983; 99:412. 7. Fleischmajer R, Agrege BJ, Shore S. Scleroderma, eosinophilia and diffuse fasciitis. Arch Dermatol 1978;114:1320-5. 8. Coyle HE, Chapman RS. Eosinophilic fasciitis (Shulman syndrome) in association with morphea and systemic sclerosis. Ada Dermatol Venereol 1980 ;60:181-2. 9. Moore TL, Zuckner J. Eosinophilic fasciitis. Semin Arthritis Rheum 1980;9:228-35. 10. Rozboril MB, Maricq HR, Rodnan GP, el al. Capillary microscopy in eosinophilic fasciitis. A comparison with systemic sclerosis. Arthritis Rheum 1983;26:617-22. 11. Bennett RM, Heron A, Keogh L. Eosinophilic fasciitis: case report and review of the literature. Ann Rheum Dis 1977;36:354-9. 12. Lackhanpal S, Ginsburg WW, Michet JJ, Doyle JA, Breanndan Moore S. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988;17:221-31. 13. Botet MV, Sanchez SL. The fascia in systemic scleroderma. J Am Acad Dermatol 1980;3:36-42. 14. Barraclough D, Begg MW. Diffuse fasciitis with eosinophilia. Aust NZ J Med 1980; 10:333-5. 15. Caspi D, Fishel R, Varon M, Yona E, Baratz M, Yaron M. Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil 1982;21:218-21. 16. Gottdiener JS, Moutsopoulos HM, Decker JL. Echocardiographic identification of cardiac abnormality in scleroderma and related disorders. Am J Med 1979;66:391-8. 17. Ackerman AB. Histologic diagnosis of inflammatory skin diseases. Beckenham, Kent: Lea & Febiger, 1978:784.
CASE REPORT
EOSINOPHILIC FASCIITIS ASSOCIATED WITH INFLAMMATORY NEUTROPHILIC VASCULITIS BY M. MAGARO*, L. ALTOMONTE*, A. ZOLP, L. MIRONE*, G. MASSIf AND F. FEDERICOt * Department of Internal Medicine, Division of Rheumatology and tInstitute of Pathology, Catholic University, Rome
SUMMARY The authors report a case of eosinophilic fasciitis with an histopathological pattern of inflammatory neutrophilic vasculitis associated with the typical inflammatory infiltrate. The presence of this type of vasculitis which may be observed in the initial forms of scleroderma supports the hypothesis of a considerable overlap between these two entities. KEY WORDS: Vasculitis, Scleroderma, Eosinophilia, Skin.
DISCUSSION Eosinophilic fasciitis may be a variant of scleroderma [6-8] or a distinct disease entity [9, 10]. There are several distinguishing clinical features between eosinophilic fasciitis and scleroderma. The latter is more common in females while eosinophilic fasciitis has been reported to be more common in males [9] or
CASE REPORT A 45-year-old man presented with a 3-month history of progressive swelling and stiffness of his arms and legs, arthralgia and lower extremities weakness. Physical examination revealed a normotensive patient with thickness of the skin over the extremities. There was no cutaneous sclerosis on his face and hands and no evidence of skin atrophy or telangiectasia. The hands were diffusely swollen but without periungual telangiectasia, digital ulceration or calcinosis.The patient did not complain of fever, arthritis or purpura. Laboratory evaluation showed a normal haemoglobin and platelet count, a white blood cell count of 10.3 x 109/l with 24% eosinophils and ESR 40 mm/h. The serum electrolytes, creatine phosphokinase, rheumatoid factor, antinuclear antibody, anti-DNA, anti-ENA and complement were normal or negative. Stool culture and examination of stool for protozoa or helminths were negative. Renal and liver function tests, Submitted 9 February; revised version accepted 4 June 1989. Correspondence to Prof. M. Magaro, Istituto di Clinica Medica, Divisione di Reumatologia, Largo A. Gemelli 8, 00168 Roma, Italia.
FIG. 1.—Severe leucocytoclastic vasculitic lesions with vessel wall damage (H & E x 55). 145
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Glasgow on June 24, 2015
electrocardiogram, oesophageal manometry, spirometry and hand and chest X-ray studies were also normal. Electrophoresis revealed a considerable IgG fraction (2110mg/dl). An en bloc biopsy of the skin, subcutis, fascia and muscle was performed at a clinically involved area. Pathological changes were dominated by dermal sclerosis which extended deeply into the subcutaneous tissues. A perivascular lymphocytic and eosinophilic infiltrate in the lower subcutis and in the mid dermis was present. The aponeurotic fascia appeared thickened and contained a significant inflammatory infiltrate composed of lymphocytes, plasma cells and eosinophils. Lymphocytes and a large number of eosinophils and plasma cells were also present around blood vessels and striated fibres in the muscle biopsy specimen. Neutrophils and nuclear debris were present in and about the walls of damaged vessels, creating a picture of neutrophilic vasculitis with an apparent leucocytoclastic pattern. The patient was treated with an initial dosage of 24 mg 6-methylprednisolone. After 2 weeks of steroid treatment, peripheral eosinophilia and hypergammaglobulinaemia normalized with notable clinical improvement.
200 cases of eosinophilic fasciitis have been reported since Shulman's [1] initial description in 1974. It is a scleroderma-like disease characterized by symmetrical, usually widespread inflammation and sclerosis of the deep fascia, subcutis and dermis. It primarily involves the extremities and is associated with peripheral and tissue eosinophilia, hypergammaglobulinaemia and steroid responsiveness [2]. The syndrome was distinguished clinically from progressive systemic slerosis by the absence of Raynaud's phenomenon, ulcerating fingertips, telangiectasia and visceral involvement and pathologically by abnormal histopathological involvement of lower subcutis and fascia [2]. Recent reports have observed an association between eosinophilic fasciitis and morphoea [3] and an overlap between eosinophilic fasciitis and progressive systemic sclerosis [4]. The possibility that eosinophilic fasciitis may evolve into progressive systemic sclerosis has also been suggested [5, 6]. We report a case of typical eosinophilic fasciitis in which an inflammatory neutrophilic vasculitis was found. OVER
146
BRITISH JOURNAL OF RHEUMATOLOGY VOL. XXIX NO. 2 oderma. Thus it is well known that progressive systemic sclerosis or localized scleroderma may show neutrophilic vasculitis in early biopsy specimens [17]. In conclusion, eosinophilic fasciitis seems to be a scleroderma-like disease which primarily involves the subcutis and fascia in the limbs and is as clinically benign as localized scleroderma. This case underlines the pathogenetic importance of vasculitic damage in the first phase of the syndrome.
:
P^^'/C^--:•--'
REFERENCES
1. Piette JC, Herson S. Fasciite avec eosinophilie (mal-
-
*
•
-
FIG. 3.—High magnification of Fig. 2.
equal in both sexes [10]. There have been reports of Raynaud's phenomenon in only isolated cases of eosinophilic fasciitis [11] while it is a common finding in scleroderma. Renal disease and accelerated hypertension have never been reported in eosinophilic fasciitis [12]. In recent years there have been reports noting a considerable overlap in the clinical picture and in the microscopic and laboratory findings of patients with eosinophilic fasciitis and scleroderma [7, 13]. Oesophageal, pulmonary and cardiac involvement have been reported in eosinophilic fasciitis, strictly comparable with those observed in progressive systemic sclerosis [5, 14-16]. Moreover, the possibility that eosinophilic fasciitis may be associated with morphoea [3] or may evolve to progressive systemic sclerosis [5, 6] has been suggested. Histopathological findings support the hypothesis of a close association between eosinophilic fasciitis and progressive systemic sclerosis. Except for the eosinophilia and the deeper involvement, collagen homogenization, tissue atrophy and an infiltrate composed of lymphocytes and plasma cells in ebsinophilic fasciitis resemble those observed in scleroderma. Tissue eosinophilia may also be noted in scleroderma specimens [7]. The neutrophilic inflammatory vasculitis observed in our patient with a typical eosinophilic fasciitis corroborates the hypothesis of a strict overlap between eosinophilic fasciitis and scler-
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Glasgow on June 24, 2015
FIG. 2.—The aponeurotic fascia thickened and containing an inflammatory infiltrate with eosinophils.
adie de shulman). Ann Dermatol Venereol 1986;113:1135-7. 2. Moutsopoulos HM, Webber BL, Paulidis NA, Fostiropoulos G, Goules D, Shulman LE. Diffuse fasciitis with eosinophilia. A clinicopathologic study. Am J Med 1980;68:701-9. 3. Caperton EM, Hathaway DE, Dehner LP. Morphea, fasciitis and scleroderma with eosinophilia: a broad spectrum of disease. Arthritis Rheum 1976;19:792-3. 4. Doyle JA. Eosinophilic fasciitis: extracutaneous manifestations and associations. Cuds 1984;34:259-61. 5. Frayma RA, Atiyah F, Karam P, Ahmed ZA, Salman SM. Eosinophilic fasciitis terminating as progressive systemic sclerosis in a child. Dermatologica 1985;171:291-4. 6. Urbano-Marquez A. Eosinophilic fasciitis evolving into scleroderma (letter). Ann Intern Med 1983; 99:412. 7. Fleischmajer R, Agrege BJ, Shore S. Scleroderma, eosinophilia and diffuse fasciitis. Arch Dermatol 1978;114:1320-5. 8. Coyle HE, Chapman RS. Eosinophilic fasciitis (Shulman syndrome) in association with morphea and systemic sclerosis. Ada Dermatol Venereol 1980 ;60:181-2. 9. Moore TL, Zuckner J. Eosinophilic fasciitis. Semin Arthritis Rheum 1980;9:228-35. 10. Rozboril MB, Maricq HR, Rodnan GP, el al. Capillary microscopy in eosinophilic fasciitis. A comparison with systemic sclerosis. Arthritis Rheum 1983;26:617-22. 11. Bennett RM, Heron A, Keogh L. Eosinophilic fasciitis: case report and review of the literature. Ann Rheum Dis 1977;36:354-9. 12. Lackhanpal S, Ginsburg WW, Michet JJ, Doyle JA, Breanndan Moore S. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988;17:221-31. 13. Botet MV, Sanchez SL. The fascia in systemic scleroderma. J Am Acad Dermatol 1980;3:36-42. 14. Barraclough D, Begg MW. Diffuse fasciitis with eosinophilia. Aust NZ J Med 1980; 10:333-5. 15. Caspi D, Fishel R, Varon M, Yona E, Baratz M, Yaron M. Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil 1982;21:218-21. 16. Gottdiener JS, Moutsopoulos HM, Decker JL. Echocardiographic identification of cardiac abnormality in scleroderma and related disorders. Am J Med 1979;66:391-8. 17. Ackerman AB. Histologic diagnosis of inflammatory skin diseases. Beckenham, Kent: Lea & Febiger, 1978:784.
