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Sweet’s syndrome presenting as palmoplantar pustulosis
Sweet’s syndrome presenting as palmoplantar pustulosis Sabine Sommer, MD, MRCP,a Stephen M. Wilkinson, MD, MRCP,a William J. Merchant, MRCPath, Dip RCPath,b and Victoria Goulden, MRCPa Leeds, England Sweet’s syndrome was initially described as a reactive dermatosis characterized by sudden onset of fever, leucocytosis, and raised erythematous plaques infiltrated with neutrophils, and therefore called acute febrile neutrophilic dermatosis. However, later it became obvious that fever and neutrophilia are variable features, and a number of other characteristics have been described. Although the dorsa of the hands are frequently affected, the palmoplantar involvement mimicking pustulosis observed in our case appears to be unusual. (J Am Acad Dermatol 2000;42:332-4.)
CASE REPORT A 65-year-old woman presented with a 2-week history of pustulosis on her palms and soles (Fig 1). Ten days previously, she had started to develop symptoms of an upper respiratory tract infection with malaise and lethargy, for which her physician had prescribed amoxycillin. There was no previous history of skin disease or family history of psoriasis. She did have ischemic heart disease and also had 2 cerebrovascular accidents in the past. Her regular medication consisted of atenolol, aspirin, nicardipine, and isosorbide mononitrate. One week after her initial presentation to our department, the pustules spread to involve the dorsa of her hands and feet, and she also developed multiple tender erythematous plaques on her trunk and limbs. On examination, she was pyrexial with discrete pustules on her hands and feet. There were multiple raised erythematous plaques covered with vesicles and pustules distributed mainly on the distal aspect of her limbs. Her mucous membranes were unaffected and the further general examination unremarkable. Hematologic investigations showed a raised white cell count with a neutrophilia. Her erythrocyte sedimentation rate was raised to 72 mm/h. Other investigations, including serum biochemistry, immunoglob-
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Departments of Dermatologya and Histopathology,b Leeds General Infirmary. Reprint requests: Dr Sabine Sommer, Leeds General Infirmary Great George Street, Leeds LS 1 3EX, England. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/99533
332
Fig 1. Palmar pustulosis at presentation.
ulins, ASO-titer, viral titers, chest radiograph, abdominal ultrasound, and fecal occult blood tests were normal or negative.
J AM ACAD DERMATOL VOLUME 42, NUMBER 2, PART 2
Sommer et al 333
Fig 2. Histology of lesion on patient’s arm. (Hematoxylin-eosin stain; original magnification, ×16.)
The histology of a biopsy of a typical lesion on her arm showed a dense superficial neutrophilic infiltrate in the dermis, extending into the epidermis, with a mild degree of karyorrhexis (Fig 2). The immunofluorescence was negative for immunoglobulin and complement deposition. These appearances were in keeping with the clinical diagnosis of Sweet’s syndrome. The patient was commenced on oral prednisolone at a dose of 30 mg/day, which led to a rapid improvement of her skin symptoms. This dose was reduced by 5 mg/wk to 10 mg/day, at which stage the cutaneous lesions relapsed. The patient responded again to an increase of the prednisolone to 20 mg/day, but relapsed once more on reduction of the steroids. At this stage, dapsone was added in at a dose of 50 mg/day, which was well tolerated and subsequently increased to 100 mg. In addition, she was treated with mometasone furoate ointment and compression bandaging to her lower legs, and again her rash cleared. The prednisolone was then reduced by 2.5 mg monthly to 7.5 mg/day, and the skin remained controlled for the next 11 months. Unfortunately, the patient then died as a result of a further cerebrovascular accident complicated by a chest infection.
DISCUSSION Sweet first described a syndrome that he called acute neutrophilic dermatosis in 1964.1 He observed fever, leucocytosis, and tender red plaques, which could occur anywhere on the body. On histology, a dermal infiltrate of neutrophils was seen, many of which showed nuclear fragmentation.2 Sweet’s syndrome may be associated with a num-
ber of systemic diseases and has been divided into 4 broad subgroups, which are as follows: idiopathic; parainflammatory, including infections and autoimmune diseases; paraneoplastic, including hemoproliferative diseases and solid tumors; and those associated with pregnancy.3 Lesions tend to be multiple, but isolated lesions have also been reported. The typical distribution includes the face, neck, upper trunk, as well as the limbs. Plaques usually measure a few centimeters, but larger lesions up to 20 cm in diameter have been described. Discrete pustules may occur adjacent to more typical lesions. Mucous membranes may be affected. Systemic involvement may include the lungs, liver, kidneys, and central nervous system. The diagnostic criteria proposed in 1986 have generally been accepted.4 For the diagnosis of Sweet’s syndrome, 2 major criteria consisting of typical skin lesions and a predominantly neutrophilic infiltration of the dermis without leukocytoclastic vasculitis have to be present, in addition to 2 of 4 minor criteria: (1) preceding nonspecific respiratory or gastrointestinal infection, vaccination, hematoproliferative disorder, solid tumor, pregnancy, or inflammatory disorder, such as chronic infection or autoimmune disease; (2) periods of fever or malaise; (3) raised erythrocyte sedimentation rate, C-reactive protein, leukocytosis, or neutrophilia; and (4) response to systemic steroid treatment. It is generally accepted that Sweet’s syndrome is a reactive dermatosis, although the pathogenesis remains unclear. Abnormal neutrophilic function has been reported, including abnormal lysosomal enzyme activity, decreased oxidative burst, and both increased and decreased neutrophil chemotaxis.3
334 Sommer et al
Plantar pustulosis preceding Sweet’s syndrome by several years has been reported once before, and the author speculated on a common underlying intrinsic abnormality of neutrophil function, although this was not further investigated.5 In Sweet’s syndrome, similar to psoriasis, the epidermal interleukin 8 immunoreactivity was diminished, despite normal serum levels.3 A pustular condition limited to the dorsa of fingers and hands, clinically resembling the plaques of Sweet’s syndrome, has been described.6 However, on histology, in addition to features of Sweet’s syndrome, there was also a small vessel vasculitis. The lesions proved resistant to systemic antibiotics, but responded to steroid treatment. Strutton et al6 proposed the term “pustular vasculitis of the hands” for this condition. Clinically, this appears to be different from the pustular eruption observed by us, which consisted of small pustules in a palmoplantar distribution. Palmar involvement in Sweet’s syndrome has been described before, although this was in the form of tender red plaques.7 In our patient there was no previous history of palmoplantar pustulosis, and the pustules were dis-
J AM ACAD DERMATOL FEBRUARY 2000
tributed on the dorsum of the hands as well as the palms. The onset, resolution, and relapse after steroid withdrawal paralleled the more typical Sweet’s syndrome lesions on her trunk and limbs. We conclude that palmoplantar pustulosis should be regarded as a rare manifestation of Sweet’s syndrome. REFERENCES 1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;74:349-56. 2. Kemmett D, Hunter JAA. Sweet’s syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990;23:5037. 3. Von der Driesch P. Sweet’s syndrome (acute neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56. 4. Siu WPD, Liu HNH. Diagnostic criteria for Sweet’s syndrome. Cutis 1986;37:167-74. 5. Keefe M, Kerr REI. Sweet’s syndrome, plantar pustulosis and vulval pustules. Clin Exp Dermatol 1988;13:344-6. 6. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995;32:192-8. 7. Gunawardena DA, Gunawardena KA, Ratnayaka RMRS, Vasanthanathan NS.The clinical spectrum of Sweet’s syndrome (acute febrile neutrophilic dermatosis)—a report of eighteen cases. Br J Dermatol 1975;92:363-73.
CASE REPORT A 65-year-old woman presented with a 2-week history of pustulosis on her palms and soles (Fig 1). Ten days previously, she had started to develop symptoms of an upper respiratory tract infection with malaise and lethargy, for which her physician had prescribed amoxycillin. There was no previous history of skin disease or family history of psoriasis. She did have ischemic heart disease and also had 2 cerebrovascular accidents in the past. Her regular medication consisted of atenolol, aspirin, nicardipine, and isosorbide mononitrate. One week after her initial presentation to our department, the pustules spread to involve the dorsa of her hands and feet, and she also developed multiple tender erythematous plaques on her trunk and limbs. On examination, she was pyrexial with discrete pustules on her hands and feet. There were multiple raised erythematous plaques covered with vesicles and pustules distributed mainly on the distal aspect of her limbs. Her mucous membranes were unaffected and the further general examination unremarkable. Hematologic investigations showed a raised white cell count with a neutrophilia. Her erythrocyte sedimentation rate was raised to 72 mm/h. Other investigations, including serum biochemistry, immunoglob-
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Departments of Dermatologya and Histopathology,b Leeds General Infirmary. Reprint requests: Dr Sabine Sommer, Leeds General Infirmary Great George Street, Leeds LS 1 3EX, England. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/99533
332
Fig 1. Palmar pustulosis at presentation.
ulins, ASO-titer, viral titers, chest radiograph, abdominal ultrasound, and fecal occult blood tests were normal or negative.
J AM ACAD DERMATOL VOLUME 42, NUMBER 2, PART 2
Sommer et al 333
Fig 2. Histology of lesion on patient’s arm. (Hematoxylin-eosin stain; original magnification, ×16.)
The histology of a biopsy of a typical lesion on her arm showed a dense superficial neutrophilic infiltrate in the dermis, extending into the epidermis, with a mild degree of karyorrhexis (Fig 2). The immunofluorescence was negative for immunoglobulin and complement deposition. These appearances were in keeping with the clinical diagnosis of Sweet’s syndrome. The patient was commenced on oral prednisolone at a dose of 30 mg/day, which led to a rapid improvement of her skin symptoms. This dose was reduced by 5 mg/wk to 10 mg/day, at which stage the cutaneous lesions relapsed. The patient responded again to an increase of the prednisolone to 20 mg/day, but relapsed once more on reduction of the steroids. At this stage, dapsone was added in at a dose of 50 mg/day, which was well tolerated and subsequently increased to 100 mg. In addition, she was treated with mometasone furoate ointment and compression bandaging to her lower legs, and again her rash cleared. The prednisolone was then reduced by 2.5 mg monthly to 7.5 mg/day, and the skin remained controlled for the next 11 months. Unfortunately, the patient then died as a result of a further cerebrovascular accident complicated by a chest infection.
DISCUSSION Sweet first described a syndrome that he called acute neutrophilic dermatosis in 1964.1 He observed fever, leucocytosis, and tender red plaques, which could occur anywhere on the body. On histology, a dermal infiltrate of neutrophils was seen, many of which showed nuclear fragmentation.2 Sweet’s syndrome may be associated with a num-
ber of systemic diseases and has been divided into 4 broad subgroups, which are as follows: idiopathic; parainflammatory, including infections and autoimmune diseases; paraneoplastic, including hemoproliferative diseases and solid tumors; and those associated with pregnancy.3 Lesions tend to be multiple, but isolated lesions have also been reported. The typical distribution includes the face, neck, upper trunk, as well as the limbs. Plaques usually measure a few centimeters, but larger lesions up to 20 cm in diameter have been described. Discrete pustules may occur adjacent to more typical lesions. Mucous membranes may be affected. Systemic involvement may include the lungs, liver, kidneys, and central nervous system. The diagnostic criteria proposed in 1986 have generally been accepted.4 For the diagnosis of Sweet’s syndrome, 2 major criteria consisting of typical skin lesions and a predominantly neutrophilic infiltration of the dermis without leukocytoclastic vasculitis have to be present, in addition to 2 of 4 minor criteria: (1) preceding nonspecific respiratory or gastrointestinal infection, vaccination, hematoproliferative disorder, solid tumor, pregnancy, or inflammatory disorder, such as chronic infection or autoimmune disease; (2) periods of fever or malaise; (3) raised erythrocyte sedimentation rate, C-reactive protein, leukocytosis, or neutrophilia; and (4) response to systemic steroid treatment. It is generally accepted that Sweet’s syndrome is a reactive dermatosis, although the pathogenesis remains unclear. Abnormal neutrophilic function has been reported, including abnormal lysosomal enzyme activity, decreased oxidative burst, and both increased and decreased neutrophil chemotaxis.3
334 Sommer et al
Plantar pustulosis preceding Sweet’s syndrome by several years has been reported once before, and the author speculated on a common underlying intrinsic abnormality of neutrophil function, although this was not further investigated.5 In Sweet’s syndrome, similar to psoriasis, the epidermal interleukin 8 immunoreactivity was diminished, despite normal serum levels.3 A pustular condition limited to the dorsa of fingers and hands, clinically resembling the plaques of Sweet’s syndrome, has been described.6 However, on histology, in addition to features of Sweet’s syndrome, there was also a small vessel vasculitis. The lesions proved resistant to systemic antibiotics, but responded to steroid treatment. Strutton et al6 proposed the term “pustular vasculitis of the hands” for this condition. Clinically, this appears to be different from the pustular eruption observed by us, which consisted of small pustules in a palmoplantar distribution. Palmar involvement in Sweet’s syndrome has been described before, although this was in the form of tender red plaques.7 In our patient there was no previous history of palmoplantar pustulosis, and the pustules were dis-
J AM ACAD DERMATOL FEBRUARY 2000
tributed on the dorsum of the hands as well as the palms. The onset, resolution, and relapse after steroid withdrawal paralleled the more typical Sweet’s syndrome lesions on her trunk and limbs. We conclude that palmoplantar pustulosis should be regarded as a rare manifestation of Sweet’s syndrome. REFERENCES 1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;74:349-56. 2. Kemmett D, Hunter JAA. Sweet’s syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990;23:5037. 3. Von der Driesch P. Sweet’s syndrome (acute neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56. 4. Siu WPD, Liu HNH. Diagnostic criteria for Sweet’s syndrome. Cutis 1986;37:167-74. 5. Keefe M, Kerr REI. Sweet’s syndrome, plantar pustulosis and vulval pustules. Clin Exp Dermatol 1988;13:344-6. 6. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995;32:192-8. 7. Gunawardena DA, Gunawardena KA, Ratnayaka RMRS, Vasanthanathan NS.The clinical spectrum of Sweet’s syndrome (acute febrile neutrophilic dermatosis)—a report of eighteen cases. Br J Dermatol 1975;92:363-73.