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Diana Camarillo, MD; Timothy H. McCalmont, MD; Ilona J. Frieden, MD; Amy E. Gilliam, MD

Arch Dermatol. 2008;144(11):1495-1498.

Background  Nonbullous neutrophilic dermatoses are seen infrequently in association with lupus erythematosus (LE). A recently described histopathologic variant of Sweet syndrome, to our knowledge, histiocytoid Sweet syndrome (HSS) has not been described in either pediatric or adult patients with LE.

Observations  We describe 2 pediatric patients with nonbullous histiocytoid neutrophilic dermatitis in the setting of LE. One case represents the initial presentation of subacute cutaneous LE, while the other case represents a manifestation of established systemic LE. Both cases demonstrate histopathologic findings of HSS.

Conclusions  We believe that the dermatosis observed in these 2 patients represents a nonbullous histiocytoid neutrophilic dermatosis that is best termed HSS. This entity may represent a distinct and important cutaneous manifestation of LE. Additional study is needed to further elucidate the relationship between neutrophilic dermatitis and LE.

Author Affiliations: Departments of Dermatology (Drs Camarillo, McCalmont, Frieden, and Gilliam), Pathology (Dr McCalmont), and Pediatrics (Drs Camarillo, Frieden, and Gilliam), and San Francisco Dermatopathology Service (Dr McCalmont), University of California, San Francisco.

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