Track: Case Reports

Topic: Inflammatory Diseases

Abstract:

Cutaneous hemophagocytosis can be a manifestation of primary hemophagocytic lymphohistiocytosis (HLH), which typically presents with fever, hepatosplenomegaly, cytopenia and coagulopathy. An ineffective pathogen elimination has been postulated which is compensated by excessive macrophage activation. A 38-year-old female patient presented with a painful skin rash and a right swollen knee joint that occurred two weeks after onset of streptococcal angina treated with amoxicillin and acetylsalicylic acid. Skin lesions presented as succulent livid red plaques from the forehead to the extensor sides of the upper arms. Medical history was unremarkable. Clinically, a classical Sweet syndrome was diagnosed by post-infectious onset, distribution, morphology of typical skin lesions, and abnormal laboratory values including neutrophilic leukocytosis. Surprisingly, skin biopsy revealed not only a neutrophilic, Sweet-typical inflammation with interstitial neutrophils, eosinophils, superficial band-like neutrophils and a marked papillary edema, but also a striking phagocytosis of neutrophils by macrophages. In addition, immunohistochemistry marked histiocytoid cells (MPO- and CD68-positive). There was a rapid regression of the complaints and skin lesions under systemic high-dose prednisone therapy. Cutaneous hemophagocytosis (CH) and Sweet syndrome are both rare and independent of each other. Clinically inevident CH in the setting of SS is extremely rare, with only one other case documented in the literature. Cutaneous hemophagocytosis restricted to the dermis has been suggested a localized, abortive form of HLH, here triggered by a previous respiratory streptococcal infection and possible drug cofactors. Extensive clarification is recommended only if indicators for a primary HLH are present.

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Pinar Avci, MD