Pustular variant of Sweet’s syndrome: a case report and review of the literature

Acute febrile neutrophilic dermatosis (Sweet’s syndrome – SS) is an uncommon inflammatory disorder characterized by suddenly appearing fever, tender skin lesions, neutrophilia and dense infiltrate of predominantly mature neutrophils in skin biopsy [1]. Typically purple-red papules, nodules or plaques are described whereas pustular lesions are not a commonly-reported finding [2]. Here we report the case of a 53-year-old woman who presented with SS with predominant pustular lesions and a literature review on selected neutrophilic dermatoses which may present pustular eruptions.
A 53-year-old woman was admitted to the Dermatology Department due to a tender, nonpruritic pustular rash that developed 3 days before, accompanied by fever up to 40°C, muscle pain and malaise. Clinical examination showed pustules varying in size 0.5–2 cm and erythematosus papules on her face, trunk and upper extremities (Figure 1) with no mucous membrane involvement. She was otherwise healthy and did not take any medications, aside from levonorgestrel-releasing intrauterine device for 1 year. Laboratory tests showed raised total white cell count 21.30 (4–10) ´ 109/l with neutrophilia 86% (40–70%), C-reactive protein 130.93 (0–5) mg/l, erythrocyte sedimentation rate (ESR) 70 (2–15) mm/h. Aerobic and mycological culture from the pus was negative, anaerobic showed Cutibacterium acnes growth. The sinus X-ray revealed a haze of the right maxillary and frontal sinus. Upon admission, the patient was empirically started on intravenous cefuroxime. She remained febrile and on the 8th hospital day, a new lesion erupted on the forehead giving the impression of herpes zoster infection (Figure 2). Therefore, acyclovir was added but the lesions kept worsening. Blood and urine culture were negative. Further investigations showed negative results of IgM antibodies for Varicella zoster virus, Herpes simplex virus, Coxsackie A7 and B1 as well as HIV virus, HCV antibodies, HBs antigen, antinuclear antibodies, antineutrophil cytoplasmic antibodies and rheumatoid factor. To rule out bullous diseases a direct immunofluorescence study was made, but we did not detect any deposits.
A biopsy specimen revealed acute inflammatory infiltrate in the upper dermis with neutrophils and papillary dermis oedema with no evidence of vasculitis.
Histological features and laboratory findings were consistent with SS diagnosis so methylprednisolone of 40 mg daily was initiated. After 2 days of...


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