%608068
ICD+
| |||||||||||||||||||||||||||
| NEUTROPHILIC DERMATOSIS, ACUTE FEBRILE | |||||||||||||||||||||||||||
| Alternative titles; symbols | |||||||||||||||||||||||||||
| AFND SWEET SYNDROME; SS GOMM-BUTTON DISEASE | |||||||||||||||||||||||||||
| TEXT | |||||||||||||||||||||||||||
| Clinical Features | |||||||||||||||||||||||||||
| Sweet (1964) described 8 patients, all women, with an acute dermatosis characterized by fever, neutrophilia, painful erythematous plaques, a dense dermal infiltrate of mature neutrophils, and rapid response to corticosteroids. In 7 of the patients the condition was preceded by an illness, usually infective. Large, tender, raised dark red plaques occurred most commonly on the face, neck, arms, and legs. Pustules, when present, were sterile. No evidence of infection was found. Uncommon systemic manifestations of Sweet syndrome involve sterile neutrophilic infiltration in organs other than skin, such as lungs, bones, joints, central nervous system, liver, gastrointestinal tract, lymph nodes, spleen, cardiovascular system, and eyes (von den Driesch, 1994; Vignon-Pennamen and Wallach, 1995). Von den Driesch (1994) stated that Sweet syndrome can be subdivided into 4 broad groups: classic/idiopathic, parainflammatory, paraneoplastic, and pregnancy-associated. Parsapour et al. (2003) delineated 2 forms of Sweet syndrome, idiopathic and associated with malignancy. The malignancy-associated form is less common, accounting for 20% of cases, and gender-independent. Malignancies associated with Sweet syndrome are usually hemoproliferative disorders or solid tumors. They stated that although Sweet syndrome is seen most commonly in the middle-aged female population, pediatric cases account for about 8% of reported cases and are generally gender-nonspecific. Behcet disease (109650) and Sweet syndrome have overlapping symptoms, including eye lesions, oral aphthae, genital ulcers, and skin lesions (Mizoguchi et al., 1987). The incidence of Behcet disease is very high in Japan (1 per 15,000 people); although no cases of Sweet syndrome with Behcet syndrome had been reported outside Japan, several Japanese patients with Behcet disease also had features of Sweet syndrome. Mizoguchi et al. (1987) reported a case of Sweet syndrome associated with Behcet disease and discussed the relationship between the 2 disorders. They came to the conclusion that Sweet syndrome may be associated with several underlying disorders, of which Behcet disease is one. | |||||||||||||||||||||||||||
| Diagnosis | |||||||||||||||||||||||||||
| Su and Liu (1986) proposed 2 major and 4 minor criteria for diagnosis of Sweet syndrome. They suggested fulfillment of both of the major criteria (abrupt onset of tender or painful erythematous or violaceous plaques or nodules; predominantly neutrophilic infiltration in the dermis without leukocytoclastic vasculitis) and 2 of the minor criteria (onset preceded by fever or infections; condition accompanied by fever, arthralgia, conjunctivitis, or underlying malignancy; leukocytosis; good response to systemic steroids but not to antibiotics) as indicative of a definite diagnosis. Von den Driesch (1994) presented a revised version of the minor criteria to include onset preceded by vaccination or associated with inflammatory diseases such as chronic autoimmune disorders, or associated with hemoproliferative disorders, solid malignant tumors, or pregnancy; they also suggested laboratory values during onset. | |||||||||||||||||||||||||||
| Clinical Management | |||||||||||||||||||||||||||
| Von den Driesch (1994) stated that oral administration of corticosteroids represents the standard treatment for Sweet syndrome; potassium iodide is as effective but may cause severe vasculitis. Because recurrence is common, the long-term use and subsequent effects of corticosteroid therapy become major obstacles in the pediatric population (Parsapour et al., 2003). | |||||||||||||||||||||||||||
| Molecular Genetics | |||||||||||||||||||||||||||
| Mizoguchi et al. (1988) reported a Japanese patient with Sweet syndrome with symptoms similar to those of Behcet disease and performed HLA typing on 28 patients with Sweet syndrome and 49 patients with Behcet disease. They found that the frequency of HLA-Bw54 was significantly higher in patients with Sweet syndrome than in controls, while there was no significant difference in the incidence of HLA-Bw54 between patients with Behcet disease and controls. No differences in HLA antigen frequencies were seen in patients with Sweet syndrome without Behcet disease symptoms and controls. The frequency of HLA-Bw54 is higher in Japanese (17.9%) compared with white (0.6%) or black (0%) populations and suggests a genetic predisposition among Japanese for Sweet syndrome. Parsapour et al. (2003) performed a comprehensive review of the literature on pediatric Sweet syndrome and reported 2 Caucasian male sibs with idiopathic neonatal Sweet syndrome, providing further evidence for a genetic component. | |||||||||||||||||||||||||||
| Nomenclature | |||||||||||||||||||||||||||
| Sweet (1964) referred to the disorder as acute febrile neutrophilic dermatosis and noted that it had in his department been referred to as Gomm-Button disease, in eponymous honor of the first 2 patients. Whittle et al. (1968) and Crow et al. (1969) were the first to use the designation 'Sweet syndrome' in their case descriptions. | |||||||||||||||||||||||||||
| REFERENCES | |||||||||||||||||||||||||||
| |||||||||||||||||||||||||||
| |||||||||||||||||||||||||||