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Giant Cellulitis-like Sweet Syndrome, a New Variant of Neutrophilic Dermatosis

Andre M. Surovy, MD; Nedzmidin Pelivani, MD; Ivan Hegyi, MD; Urs Buettiker, MD; Helmut Beltraminelli, MD; Luca Borradori, MD
JAMA Dermatol. 2013;149(1):79-83. doi:10.1001/2013.jamadermatol.548.
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Background  Neutrophilic dermatoses comprise a wide spectrum of inflammatory diseases with overlapping features characterized histologically by the presence of an aseptic neutrophilic infiltrate in the epidermis, dermis, and/or hypodermis and are often associated with systemic inflammatory and neoplastic disorders.

Observations  We describe 3 patients with an unusual neutrophilic dermatosis characterized by relapsing episodes of fever, widespread infiltrated plaques with bullous appearance, and variable involvement of the arms, legs, abdomen, and/or trunk. Light microscopy studies showed marked edema of the papillary dermis with an inflammatory infiltrate consisting mainly of mature neutrophils. All 3 patients were morbidly obese, and workup revealed underlying cancer in 2 cases: myeloma and breast carcinoma. Management of the underlying disease resulted in long-term remission of the skin disease.

Conclusions  The clinicopathologic features in our 3 cases best correspond to a widespread giant cellulitis-like form of Sweet syndrome. Knowledge of this newly observed unusual variant of Sweet syndrome within the broad spectrum of neutrophilic diseases is important for its prompt and proper management.

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Figures

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Figure 1. Lesions associated with Sweet syndrome in patient 1. A, Erythematous infiltrated plaque with a bullous appearance involving the left leg. B, Erythematous, slightly infiltrated plaques on the abdomen. C, Similar lesions on the back and buttocks.

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Figure 2. Lesions associated with Sweet syndrome in patient 2. A, Erythematous infiltrated plaques on the back and buttocks. B, Erythematous plaques with bullous appearance and a purpuric component. C, Plaque on the right thigh and the knee.

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Figure 3. Light microscopy and histologic examination results in patient 2. A, A dense inflammatory infiltrate of the upper dermis and middermis consists mainly of mature neutrophils and marked edema of the papillary dermis (hematoxylin-eosin, original magnification ×100). B, Neutrophils in perivascular distribution without leukocytoclasia and interstitial edema (hematoxylin-eosin, original magnification ×400).

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Figure 4. Large erythematous infiltrated plaques involving the hip region, the right leg, and the right foot in patient 3.

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