Histiocytoid Sweet syndrome successfully treated with etanercept

ABSTRACT

We report the first case of a 34-year-old woman with histiocytoid Sweet syndrome (HSS) that was successfully treated with etanercept. HSS is a rare histological variant of acute febrile neutrophilic dermatosis that was described by Requena et al in 2005. It is distinguished by dermal infiltration by mononuclear cells with a histiocytic morphology. To date there are three reported cases of the use of etanercept in the treatment of classic febrile neutrophilic dermatosis but none targeting this disease variant. Our patient presented with a 6-month history of scattered erythematous papules on the neck, trunk, and upper and lower limbs bilaterally. Clinical findings and histopathological evaluation were highly suggestive of HSS. After 32 months of refractory disease activity, our patient was initiated on a regimen of etanercept 1 mg/kg subcutaneously twice weekly and topical desoximetasone 0.05% ointment twice daily as required. To date, our patient has achieved 37 months of remission.

KEYWORDS:

• Enbrel
• etanercept
• histiocytoid Sweet syndrome
• neutrophilic dermatosis
• tumor necrosis factor

DISCLOSURE STATEMENT

No funding was provided by any source for this project. Dr. Menter reports grants and honoraria from AbbVie, Allergan, Amgen, Boehringer Ingelheim, Janssen Biotech, Inc., LEO Pharma, Novartis, Pfizer, and Xenoport; grants from Anacor, Celgene, Dermira, Merck, Neothetics, Regeneron, and Symbio/Maruho; and honoraria from Eli-Lily, Galderma, and Vitae, all outside of the submitted work. The remaining authors do not have funding disclosures, financial support, or industrial affiliations to report regarding any materials or methods used in this study or the findings indicated in this article.