EP11 An unusual presentation of Sweet syndrome

Case Reports in Immunology, 2014

A 7-year-old boy with high grade fever (39 ∘ C) and warm, erythematous, and indurated plaque above the left knee was referred. According to the previous records of this patient, these indurated plaques had been changed toward abscesses formation and then spontaneous drainage had occurred after about 6 to 7 days, and finally these lesions healed with scars. In multiple previous admissions, high grade fever, leukocytosis, and a noticeable increase in erythrocyte sedimentation rate and C-reactive protein were noted. After that, until 7th year of age, he had shoulder, gluteal, splenic, kidney, and left thigh lesions and pneumonia. The methylprednisolone pulse (30 mg/kg) was initiated with the diagnosis of Sweet's syndrome. After about 10-14 days, almost all of the laboratory data regressed to nearly normal limits. After about 5 months, he was admitted again with tachypnea and high grade fever and leukocytosis. After infusion of one methylprednisolone pulse, the fever and tachypnea resolved rapidly in about 24 hours. In this admission, colchicine (1 mg/kg) was added to the oral prednisolone after discharge. In the periodic fever and neutrophilic dermatosis, the rheumatologist should search for sterile abscesses in other organs.

2016

Sweet syndrome is a rare inflammatory condition that was first described by Douglas Sweet in 1964 as an acute febrile neutrophilic dermatosis. It can be associated with infections, inflammatory conditions,pregnancy, drugs, and malignancy. It is usually divided into three subtypes based on etiology: classical(idiopathic); malignancy-associated; and drug-induced. We describe a patient with classical Sweet syndrome who had a dramatic response to corticosteroids.Our patient met the major criteria for diagnosis (positive histopathology and an abrupt onset of a painful rash), along with 4 minor criteria (fever, preceding upper respiratory tract infection, dramatic response to steroids, and leukocytosis).

Baylor University Medical Center Proceedings, 2021

We present a rare case of Sweet’s syndrome. A 39-year-old woman with subjective fevers, polyarthralgia, and malaise presented with worsening painful erythematous plaques on the trunk, arms, and legs. Further examination with biopsy revealed a diagnosis of acute febrile neutrophilic dermatosis, or Sweet’s syndrome. Diagnosis by skin biopsy is crucial, and onset requires prompt evaluation for serious associated disorders such as leukemias, inflammatory bowel disease, thyroid disease, sarcoidosis, and infectious etiologies. In general, symptoms and cutaneous manifestations of Sweet syndrome respond rapidly to treatment with systemic corticosteroids or potassium iodide.

A 53-year-old Greek housewife was admitted to hospital because of painful erythematous lesions on the lower (Fig. 1) and upper (Fig. 2) extremities in association with painful ocular hyperemia. These symptoms were preceded by an upper respiratory infection with persistent high-grade fever for about 1 week. Erythematous lesions had appeared 3 days previous to admission. Her medical history included two previous episodes of erythema nodosum (unknown etiology), essential hypertension treated with perindopril 4 mg / day, diabetes mellitus type II under diet only, and multinodular goiter under treatment with thyroxine 0.1 mg / day. Examination of the skin revealed tender, violet-red, subcutaneous erythematous nodules, measuring 2-3 cm in diameter, located on the anterior and posterior surfaces of the legs (Fig. 1), and tender, violaceous papulo-vesicular lesions located on the face and forearms (Fig. 2). Ocular examination revealed diffuse anterior scleritis. Laboratory tests gave the following values: white blood cell count, 12,600 cells / mm 3 (neutrophils 78%, lymphocytes 16%, and monocytes 4%); erythrocyte sedimentation rate (ESR), 109 mm / h; C-reactive protein (CRP), 13 mg / dL (normal range: 0.08 -0.8 mg / dL); normal biochemical parameters and urinalysis. An electrocardiogram, chest X-ray, gastroscopy, colonoscopy and abdominal ultrasound scan were within normal limits. The antistreptolysin (ASTO) level and thyroid function tests gave results within the normal range. A tuberculin skin test, and immunological and serological tests, such as tests for viruses, were negative. Histological examination of a biopsy specimen obtained from an erythematous nodule on the left leg showed panniculitis involving inflammation of the septa in the subcutaneous fat tissue without signs of vasculitis.

Sweet's syndrome is rare in children. Usually, heralded by tender polymorphous skin lesions, typically accompanied by fever and peripheral neutrophilia, can easily be mistaken for infection and lead to many antibiotic administration. Such therapy does not improve disease, which is thought to represent an immunologic reaction to agents as diverse as infection, neoplasia, or drugs. We report two boys, six and nine-year-old who suffered from acute febrile neutrophilic dermatosis. Both were febrile, had leukocytosis, and exhibited characteristic clinical and histopathologic lesions. They had an antecedent trauma, and responded to systemic corticosteroids. Our evaluation and long term follow-up of these children failed to reveal evidence of underlying malignancy or a chronic systemic illness typically encountered in Sweet's syndrome.

Annals of Allergy, Asthma & Immunology, 2010

Case Reports, 2011

BACKGROUND Sweet's syndrome (SS) is the most common neutrophilic dermatosis (ND). It is most often idiopathic but may be associated with a wide range of diseases, especially malignant conditions, infectious diseases or infl ammatory diseases. CASE PRESENTATION We report the case of a 28-year-old woman with a 2-year history of seropositive and destructive rheumatoid arthritis (RA) associated with a secondary Sjögren's syndrome, who was treated by methotrexate (15 mg/week) and lowdose prednisone (5 mg/day). She presented with a widespread cutaneous eruption that involved the face, trunk and four limbs, associated with fever, arthralgia, bilateral conjunctivitis and deterioration of general condition. This symptomatology occurs 5 days after a nasopharyngitis. Clinical examination objectifi ed the fever at 38.5°C and demonstrated well-demarcated erythematous papules, with centrifugal extension involving the face, arm, back Unusual association of diseases/symptoms

Current Health Sciences Journal, 2020

Introduction. Sweet syndrome (SS), also denominated as acute febrile neutrophilic dermatosis, is a rare disease characterized by the sudden onset of painful, erythematous, firm skin lesions (papules, plaques, and nodules) which show, upon histologic examination, the presence of a diffuse infiltrate of mature neutrophils. The cutaneous manifestation typically involves the face, neck, trunk, and upper limbs and is associated with fever, general malaise, arthralgia. Case report. A female patient, 60 years old, attended the Dermatology Clinic due to the appearance of violaceous erythematous-oedematous infiltrated plaques, located on the face, neck, upper limbs, trunk and knees. The onset of the cutaneous manifestation had occurred 2 months prior, accompanied by pain, chills, flares of fever and arthralgia. The onset coincided with the surgical treatment of an umbilical hernia. From the medical history we note that the patient was diagnosed in 2014 with histiocytoid SS. She followed a tr...

Journal of Investigative Medicine High Impact Case Reports

Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder that typically presents with rapid appearance of tender skin lesions accompanied by fever and leukocytosis with neutrophilia. Its pathogenesis is not fully understood. The syndrome is generally classified into classical, malignancy-associated, and drug-induced categories, each of which has its specific characteristics. In this article, we present a case of classical Sweet’s syndrome in a woman who presented with an acute viral illness.

British Journal of Dermatology, 1991

We report a case of Sweet's syndrome in childhood. Our patient suffered from repeated fevers, chest infections and had characteristic erythematous plaques. The severity of his illness increased with age and warranted oral steroid treatment. We review lf> cases reported to date in the literature and comment on the management and prognosis of the disease in childhood.