Acute febrile neutrophilic dermatosis (Sweet's syndrome) with erythema nodosum and anterior scleritis. A case report

Abstract

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.Subcutaneous erythematous nodules on the anterior and posterior surfaces of the legsimageErythematous lesions on the forearmsimageExamination 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/mm3 (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. Histopathology of a biopsy specimen obtained from a lesion on the right forearm revealed remarkable inflammatory neutrophilic (predominantly) infiltration throughout the entire dermis admixed with some mononuclear cells (lymphocytes and histiocytes) and sparse eosinophils. Endothelial cells of small vessels showed swelling, without signs of vasculitis. Papillary dermis was edematous (Fig. 3a and b).(a) Edema of the papillary dermis and a dense inflammatory infiltrate of the lower dermis with focal edema (hematoxylin and eosin, ×60). (b) Dense infiltrate of the dermis composed largely of neutrophils, intermixed with mononuclear cells. Some nuclear debris (nuclear dust) is evident. Endothelial cells of capillaries show some swelling. No vasculitis is apparent (hematoxylin and eosin, ×300)imageThe patient was treated with prednisolone (initial dose 0.5 mg/kg for 1 week), reduced gradually and suspended after 4 weeks. Five days after the initiation of treatment, the skin lesions vanished and ocular manifestations improved. Leukocyte and neutrophil counts, ESR and CRP returned to normal on suspension of therapy. There was no recurrence at follow‐up 6 months and 1 year later.