This group of disorders, in an untreated state, is characterized by infiltrates of neutrophils within the skin. In addition, these dermatoses lack an identifiable infectious etiology, despite the presence of neutrophils (Fig. 21.1). There can be significant overlap in the clinical presentations of the neutrophilic dermatoses; for example, in a patient with acute myelo­genous leukemia, bullous pyoderma gangrenosum may be difficult to distinguish from Sweet’s syndrome. In addition, infiltrates of neutrophils can occur in other organs, particularly the joints, eyes, lungs, and bones. Bone involvement raises the possibility of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.

Sweet’s Syndrome (Acute Febrile Neutrophilic Dermatosis)

• Acute onset of erythematous edematous papules and plaques that are tender, but not pruritic; if the edema is intense, the lesions may become bullous and, occasionally, they resemble erysipelas; favored sites are the face, neck, upper trunk, and upper extremities (Fig. 21.2).

Fig. 21.2 Spectrum of cutaneous findings in Sweet’s syndrome. A Edematous erythematous papules and plaques on the neck of a patient with acute myelogenous leukemia. B Markedly edematous plaques on the upper back, some of which are pseudovesicular while others are becoming bullous. C Some of the edematous lesions have a targetoid appearance. D Central hemorrhagic crusts within facial plaques. B, D, Courtesy, Kalman Watsky, MD; C, Courtesy, Mark Davis, MD.

• Less commonly, nodules develop due to neutrophilic panniculitis or pustules form within the plaques; a variant occurs on the dorsal aspect of the hands and is referred to as ‘neutrophilic dermatosis of the dorsal hands’ (Fig. 21.3).

Fig. 21.3 Neutrophilic dermatosis of the dorsal hands. This entity has overlapping clinical and histologic features with both Sweet’s syndrome and pyoderma gangrenosum. Some patients have an associated pustular vasculitis. Courtesy, Jean L. Bolognia, MD.

• Associated systemic findings include fever, malaise, and arthralgias, and a peripheral leukocytosis is commonly observed (Table 21.1); some patients also develop systemic manifestations, including ocular, pulmonary, and skeletal involvement (Fig. 21.4, Table 21.2).

Table 21.1

Criteria for the diagnosis of Sweet’s syndrome.

Both of the major criteria and two of the minor criteria are needed for the diagnosis.

From Su WPD, Liu HNH. Diagnostic criteria for Sweet’s syndrome. Cutis 1986;37:167–174.

Fig. 21.4 Ocular involvement in Sweet’s syndrome. Erythema and hemorrhage of the sclera and conjunctiva. Courtesy, Kalman Watsky, MD.

Table 21.2

Systemic manifestations of Sweet’s syndrome.

SAPHO, synovitis, acne, pustulosis, hyperostosis, osteitis.

• Most often seen in adults, with a female:male ratio of 4 : 1 (except in the case of malignancy-associated disease); may first appear or flare during pregnancy; idiopathic in up to 50% of patients.

• Underlying disorders include: (1) infections – upper respiratory tract (e.g. viral, streptococcal) or gastrointestinal (e.g. yersinosis) > HIV or atypical mycobacteria; (2) hematologic malignancies (10–20% of patients), particularly acute myelogenous leukemia (AML) but also myelodysplasia and myeloproliferative disorders; (3) inflammatory bowel disease; (4) autoimmune connective tissue disease, particularly systemic lupus erythematosus (SLE); (5) drugs – G-CSF, all-trans-retinoic acid > furosemide, minocycline; and (6) carcinomas – genitourinary, breast, colon.

• The vesiculobullous form is more often associated with AML, and in malignancy-associated Sweet’s syndrome, the lesions tend to be more widespread, including within the oral cavity.

• Histologically, diffuse infiltrates of neutrophils are seen within the dermis and occasionally the subcutaneous fat; leukocytoclastic vasculitis is absent or minimal.

• DDx: bullous pyoderma gangrenosum, neutrophilic eccrine hidradenitis, erysipelas, erythema multiforme, causes of pseudocellulitis (see Table 61.2), infectious cellulitis, vasculitis (urticarial, small vessel, septic), halogenoderma, periodic fever syndromes, as well as additional entities in Fig. 21.1.

• There are differing opinions regarding whether the development of a nonbullous neutrophilic dermatosis in the setting of LE is a distinct entity or is simply Sweet’s syndrome associated with LE.

• Rx:

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