CAMEO
ACUTE EEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME) ASSOCIATED WITH BEHgET'S DISEASE OYA OGUZ, M.D., SERVER S E R D A R O G L U , M.D., YALCIN TUZtlN, M.D., NUSRET E R D O G A N , M.D., HASAN YAZICI, M.D., AND HAEIT SAVAJKAN, M.D.
Case 1: A 46-year-old woman suffered from painful, raised, erythematous plaques, initially located on the volar aspects of her hands, which extended to the extensor surfaces of her arms. A year previously, she had been diagnosed as having the complete type of Behget's disease with oral aphthae, ocular involvement, genital ulceration, and a positive pathergy test.''" The last time she was admitted to the hospital, she had only the eruptions mentioned but no other symptoms of Behget's disease. Dermatologic examination showed that these were dark, erythematous almost purplish papules, which tended to coalesce, located on the outer surfaces of her arms. She did not have any systemic findings. Her body temperature was 38.5°C. Hematocrit was 42%, RBC count 3.6x10''/mm^, WBC count 12.2x103/mm^ with 76% neutrophils. Erythrocyte sedimentation rate was 65 mm in the first hour. Cultures from her tonsils and urine were negative. Histopathologic examination of the eruptions showed a moderate papillomatosis and a dense infiltrate composed of mostly neutrophils, lymphocytes, histiocytes, and nuclear dusts. The infiltrate was situated around the vessels in the upper half of the dermis, which is typical for Sweet's syndrome. The epidermis appeared mildly hyperkeratotic in most areas. The pathergy test was positive. She was given 60 mg of prednisolone per day. The body temperature became normal within 24 hours, and considerable Improvement occurred between the second and the fourth days of treatment. The dose was gradually decreased by 10 mg at 10 day intervals. She was symptom free within the first week, and, during the follow-up period of 10 months, no recurrences were observed.
Figure 1. Gase 2. Glinical appearance of inflammatory nodules on cheeks. came positive. His body temperature was 37.5°C. Hematocrit was 45%, RBC count 4.5x10Vmm'', WBC count I.OxiOVmm^ with 64% neutrophils. Erythrocyte sedimentation rate was 64 mm in the first hour. Radiologic examination and pulmonary function tests revealed obstructive changes. Histopathologic findings included marked edema of the upper dermis and a dense perivascular infiltration of neutrophils (Fig. 2). Cultures from his tonsils and urine remained negative. He was given 40 mg/day prednisolone orally for 10 days, and the dose was gradually decreased within 1 month. He improved remarkably in 3 days, and the eruptions cleared over 6 months.
Case 2: A 51-year-old man, who had previously been hospitalized for nodular vasculitis and treated by corticosteroids for some time, was admitted to the hospital with the complaint of raised, painful plaques on his arms. He had a history of oral aphthae, had developed erythema nodosum, and also complained of joint pains. Dermatologic examination revealed dark red and tender inflammatory nodules with some central blisters on his cheeks, behind his auricles, and on the lateral aspects of his neck and arms (Fig. 1). Within a few days oral aphthae and ulcers on his scrotum appeared. The pathergy test, which had been previously negative, be-
DISCUSSION
f
The differential diagnosis of the dermatoses, which are probably itnniunologically based and characterized by dermal infiltration of polytnorphonuclear leukocytes include: acute febrile tieutrophilic dertnatosis, erythema tnultifortne, allergic or necrotizing vasculitis, Behget's disease, facial granulotna, periarteritis nodosa, and erythema elevatutn diutinutn.'""' As these conditions may usually resetiible each other. Sweet's sytidrotne tnay easily be otnitted in atypical cases unless histopathologic confirmatioti is sought. The association of Sweet's syndrome with Behget's disease has previously been reported iti Gertnany** and
From the Departments of Dermatology and Rheumatology, University of Istanbul, Gerrahpa§a Medical School, Istanbul, and the Kartal State Hospital, Istanbul, Turkey. Address for correspondence: Oya Oguz, M.D., Bagdat Gad., No: 386/8, §a5kinbakkal, Suadiye, Istanbul, Turkey. 645
International journal of Dermatology Vol. 31, No. 9, September 1992
2.
Gooper PH, Innes DJ, Greer KE. Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. Gancer 1983; 51:1518-1526. 3. Butron JL. Sweet's syndrome, pyoderma gangrenosum and acute leukemia letter. BrJ Dermatol 1980; 102:239. 4. Prystowsky SD, Eye KH, Goette KD, Daniels TE. Acute febrile neutrophilic dermatosis associated with Sjogren's syndrome. Arch Dermatol 1978; 114:1234. 5. Trentham DE, Masi AT, Baker GF. Arthritis with an inflammatory dermatosis resembling Sweet's syndrome. Report of a unique case and review of the literature on arthritis associated with the inflammatory dermatoses. Am J Med 1976; 61:424-431. 6. Sebastian FV, Jurita JMG, Diez LI. Sweet's syndrome. A clinical and pathological study of 28 cases. 17th World Gongress of Dermatology, May, 1987. 7. Mizoguchi M, Ghikakane K, Goh K, et al. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in Behget's disease. BrJ Dermatol 1987; 116:727-734. 8. Meters HG. Akute febrile neutrophile Dermatose, Ubersicht und Kasuistik. Hautarzt 1972; 23:111. 9. Klock JG, Oken RL. Eebrile neutrophilic dermatosis in acute myelogenous leukemia. Gancer 1976; Yl•.922.-9X7. 10. Tiiziin Y, Yazici H, Pazarli H, et al. The usefulness of the nonspecific skin hyperreactivity (the pathergy test) in Behget's disease in Turkey. Acta Derm Venereol (Stockh) 1979; 59:77-79. 11. Sweet RD. Further ohservations on acute febrile neutrophilic dermatosis. BrJ Dermatol 1968; 80:800-805. 12. Moschella SL. Diseases of the peripheral vessels and their contents: acute febrile neutrophilic dermatosis. In: Moschella SL, Hurley HJ, eds. Dermatology. 2nd Ed. Vol 1. Philadelphia: WB Saunders, 1987; 486.
Figure 2. Gase 2. Lesional biopsy showing edema of the upper dermis and infiltration of neutrophils. (original magnification X 150)
Japan.^ Recently, Japanese authors have pointed out that Behget's disease, a systemic vasculitis,'^ may be one of the conditions associated with Sweet's syndrome.^'"* In a recent study, the incidence of septal panniculitis was recorded as 8% and focal necrotizing vasculitis as 11% in patients with Sweet's syndrome;** however, it has also been shown that Japatiese patients with Sweet's syndrome and Behget's disease did not have similar HLA associations.'"
13.
14.
15.
Both of our patients fulfilled the new defined international criteria for Behget's disease.'''' One patient had active disease at the onset of Sweet's syndrome, while the other had longstanding Behget's disease with no active lesions other than those associated with Sweet's syndrome at the time of onset of the latter. It has been suggested that immunologic events take place at early stages of Sweet's syndrome, that eventually proceed to vasculitis and vascular datnage.''"''^'^" Prospective studies are needed to establish whether a formal association exists between Behget's disease and Sweet's syndrome.
16.
17.
18. 19. REFERENCES 1.
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964; 76:349-356.
20.
646
Greer KW, Pruitt JL, Bishop GF. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Arch Dermatol 1975; 111:1461-1463. Shapiro L, Ghores SD, Leonard LR. Sweet's syndrome (acute febrile neutrophilic dermatosis). Arch Dermatol 1971; 103:81. Honigsmann H, Wollf K. Acute febrile neutrophilic dermatosis (Sweet's syndrome). In: Fitzpatrick TB, Eisen AZ, Freedberg IM, Austen KF, eds. Dermatology in general medicine. 3rd Ed. Vol 1. New York: McGrawHill, 1987; 1323. Hazen PG, Kark EG, Davis BR, et al. Acute febrile neutrophilic dermatosis in children. Arch Dermatol 1983; 119:998-1002. Miiftiioglu AU, Yurdakul S, Yazici H, et al. Vascular involvement in Behget's disease—a review of 129 cases. In: Lehner T, Barnes GG, eds. Recent advances in Behget's disease. London: Royal Society of Medicine Services, 1986:255. Mizushima Y. Recent research into Behget's disease in Japan. Int J Tiss Reac 1 988;X(2):59. International Study Group for Behget's Disease. Griteria for diagnosis of Behget's disease. Lancet 1990; 335: 1078-1080. Storer JS, Nesbitt LT, Galen WK, Deleo VA. Sweet's syndrome. Int J Dermatol 1983; 22:8-12.
ACUTE EEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME) ASSOCIATED WITH BEHgET'S DISEASE OYA OGUZ, M.D., SERVER S E R D A R O G L U , M.D., YALCIN TUZtlN, M.D., NUSRET E R D O G A N , M.D., HASAN YAZICI, M.D., AND HAEIT SAVAJKAN, M.D.
Case 1: A 46-year-old woman suffered from painful, raised, erythematous plaques, initially located on the volar aspects of her hands, which extended to the extensor surfaces of her arms. A year previously, she had been diagnosed as having the complete type of Behget's disease with oral aphthae, ocular involvement, genital ulceration, and a positive pathergy test.''" The last time she was admitted to the hospital, she had only the eruptions mentioned but no other symptoms of Behget's disease. Dermatologic examination showed that these were dark, erythematous almost purplish papules, which tended to coalesce, located on the outer surfaces of her arms. She did not have any systemic findings. Her body temperature was 38.5°C. Hematocrit was 42%, RBC count 3.6x10''/mm^, WBC count 12.2x103/mm^ with 76% neutrophils. Erythrocyte sedimentation rate was 65 mm in the first hour. Cultures from her tonsils and urine were negative. Histopathologic examination of the eruptions showed a moderate papillomatosis and a dense infiltrate composed of mostly neutrophils, lymphocytes, histiocytes, and nuclear dusts. The infiltrate was situated around the vessels in the upper half of the dermis, which is typical for Sweet's syndrome. The epidermis appeared mildly hyperkeratotic in most areas. The pathergy test was positive. She was given 60 mg of prednisolone per day. The body temperature became normal within 24 hours, and considerable Improvement occurred between the second and the fourth days of treatment. The dose was gradually decreased by 10 mg at 10 day intervals. She was symptom free within the first week, and, during the follow-up period of 10 months, no recurrences were observed.
Figure 1. Gase 2. Glinical appearance of inflammatory nodules on cheeks. came positive. His body temperature was 37.5°C. Hematocrit was 45%, RBC count 4.5x10Vmm'', WBC count I.OxiOVmm^ with 64% neutrophils. Erythrocyte sedimentation rate was 64 mm in the first hour. Radiologic examination and pulmonary function tests revealed obstructive changes. Histopathologic findings included marked edema of the upper dermis and a dense perivascular infiltration of neutrophils (Fig. 2). Cultures from his tonsils and urine remained negative. He was given 40 mg/day prednisolone orally for 10 days, and the dose was gradually decreased within 1 month. He improved remarkably in 3 days, and the eruptions cleared over 6 months.
Case 2: A 51-year-old man, who had previously been hospitalized for nodular vasculitis and treated by corticosteroids for some time, was admitted to the hospital with the complaint of raised, painful plaques on his arms. He had a history of oral aphthae, had developed erythema nodosum, and also complained of joint pains. Dermatologic examination revealed dark red and tender inflammatory nodules with some central blisters on his cheeks, behind his auricles, and on the lateral aspects of his neck and arms (Fig. 1). Within a few days oral aphthae and ulcers on his scrotum appeared. The pathergy test, which had been previously negative, be-
DISCUSSION
f
The differential diagnosis of the dermatoses, which are probably itnniunologically based and characterized by dermal infiltration of polytnorphonuclear leukocytes include: acute febrile tieutrophilic dertnatosis, erythema tnultifortne, allergic or necrotizing vasculitis, Behget's disease, facial granulotna, periarteritis nodosa, and erythema elevatutn diutinutn.'""' As these conditions may usually resetiible each other. Sweet's sytidrotne tnay easily be otnitted in atypical cases unless histopathologic confirmatioti is sought. The association of Sweet's syndrome with Behget's disease has previously been reported iti Gertnany** and
From the Departments of Dermatology and Rheumatology, University of Istanbul, Gerrahpa§a Medical School, Istanbul, and the Kartal State Hospital, Istanbul, Turkey. Address for correspondence: Oya Oguz, M.D., Bagdat Gad., No: 386/8, §a5kinbakkal, Suadiye, Istanbul, Turkey. 645
International journal of Dermatology Vol. 31, No. 9, September 1992
2.
Gooper PH, Innes DJ, Greer KE. Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. Gancer 1983; 51:1518-1526. 3. Butron JL. Sweet's syndrome, pyoderma gangrenosum and acute leukemia letter. BrJ Dermatol 1980; 102:239. 4. Prystowsky SD, Eye KH, Goette KD, Daniels TE. Acute febrile neutrophilic dermatosis associated with Sjogren's syndrome. Arch Dermatol 1978; 114:1234. 5. Trentham DE, Masi AT, Baker GF. Arthritis with an inflammatory dermatosis resembling Sweet's syndrome. Report of a unique case and review of the literature on arthritis associated with the inflammatory dermatoses. Am J Med 1976; 61:424-431. 6. Sebastian FV, Jurita JMG, Diez LI. Sweet's syndrome. A clinical and pathological study of 28 cases. 17th World Gongress of Dermatology, May, 1987. 7. Mizoguchi M, Ghikakane K, Goh K, et al. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in Behget's disease. BrJ Dermatol 1987; 116:727-734. 8. Meters HG. Akute febrile neutrophile Dermatose, Ubersicht und Kasuistik. Hautarzt 1972; 23:111. 9. Klock JG, Oken RL. Eebrile neutrophilic dermatosis in acute myelogenous leukemia. Gancer 1976; Yl•.922.-9X7. 10. Tiiziin Y, Yazici H, Pazarli H, et al. The usefulness of the nonspecific skin hyperreactivity (the pathergy test) in Behget's disease in Turkey. Acta Derm Venereol (Stockh) 1979; 59:77-79. 11. Sweet RD. Further ohservations on acute febrile neutrophilic dermatosis. BrJ Dermatol 1968; 80:800-805. 12. Moschella SL. Diseases of the peripheral vessels and their contents: acute febrile neutrophilic dermatosis. In: Moschella SL, Hurley HJ, eds. Dermatology. 2nd Ed. Vol 1. Philadelphia: WB Saunders, 1987; 486.
Figure 2. Gase 2. Lesional biopsy showing edema of the upper dermis and infiltration of neutrophils. (original magnification X 150)
Japan.^ Recently, Japanese authors have pointed out that Behget's disease, a systemic vasculitis,'^ may be one of the conditions associated with Sweet's syndrome.^'"* In a recent study, the incidence of septal panniculitis was recorded as 8% and focal necrotizing vasculitis as 11% in patients with Sweet's syndrome;** however, it has also been shown that Japatiese patients with Sweet's syndrome and Behget's disease did not have similar HLA associations.'"
13.
14.
15.
Both of our patients fulfilled the new defined international criteria for Behget's disease.'''' One patient had active disease at the onset of Sweet's syndrome, while the other had longstanding Behget's disease with no active lesions other than those associated with Sweet's syndrome at the time of onset of the latter. It has been suggested that immunologic events take place at early stages of Sweet's syndrome, that eventually proceed to vasculitis and vascular datnage.''"''^'^" Prospective studies are needed to establish whether a formal association exists between Behget's disease and Sweet's syndrome.
16.
17.
18. 19. REFERENCES 1.
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964; 76:349-356.
20.
646
Greer KW, Pruitt JL, Bishop GF. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Arch Dermatol 1975; 111:1461-1463. Shapiro L, Ghores SD, Leonard LR. Sweet's syndrome (acute febrile neutrophilic dermatosis). Arch Dermatol 1971; 103:81. Honigsmann H, Wollf K. Acute febrile neutrophilic dermatosis (Sweet's syndrome). In: Fitzpatrick TB, Eisen AZ, Freedberg IM, Austen KF, eds. Dermatology in general medicine. 3rd Ed. Vol 1. New York: McGrawHill, 1987; 1323. Hazen PG, Kark EG, Davis BR, et al. Acute febrile neutrophilic dermatosis in children. Arch Dermatol 1983; 119:998-1002. Miiftiioglu AU, Yurdakul S, Yazici H, et al. Vascular involvement in Behget's disease—a review of 129 cases. In: Lehner T, Barnes GG, eds. Recent advances in Behget's disease. London: Royal Society of Medicine Services, 1986:255. Mizushima Y. Recent research into Behget's disease in Japan. Int J Tiss Reac 1 988;X(2):59. International Study Group for Behget's Disease. Griteria for diagnosis of Behget's disease. Lancet 1990; 335: 1078-1080. Storer JS, Nesbitt LT, Galen WK, Deleo VA. Sweet's syndrome. Int J Dermatol 1983; 22:8-12.
![[Acute febrile neutrophilic dermatosis Sweet's syndrome].](/assets/img/hold.png)
