Deep Sweet Syndrome Secondary to Pegfilgrastim

April 2022 | Volume 21 | Issue 4 | Case Reports | 422 | Copyright © April 2022


Published online March 25, 2022

Dillon D. Clarey MD,a Dominick J. DiMaio MD,b Ryan M. Trowbridge MDa

aUniversity of Nebraska Medical Center, Department of Dermatology, Omaha, NE
bUniversity of Nebraska Medical Center, Department of Pathology and Microbiology, Omaha, NE

Abstract
Sweet syndrome, or acute febrile neutrophilic dermatosis, is a skin condition consisting of erythematous papules and plaques in association with fever, neutrophilia, and a neutrophilic infiltrate that typically involves the papillary dermis. Although development is most commonly idiopathic, medications are also frequently associated with the eruption, notably, the granulocyte colony-stimulating factor (G-CSF), filgrastim. Pegylated G-CSF, despite similar activity, is not commonly reported, with only four published cases. We present a case of drug-induced sweet syndrome with unique histologic features (deep inflammatory infiltrate) in association with the usage of pegfilgrastim in the treatment of invasive ductal carcinoma of the breast.

J Drugs Dermatol. 2022;21(4):422-424. doi:10.36849/JDD.4794

INTRODUCTION

Sweet syndrome (SS), first described in 1964, is a disease constellation of pyrexia, neutrophilic leukocytosis, tender erythematous papules/nodules/plaques, and a diffuse papillary dermal infiltrate of mature neutrophils.1 Three common subtypes of SS have been identified: classic (idiopathic), malignancy-associated, and drug-induced.1 The drug-induced form of SS most often arises with the usage of granulocyte colony-stimulating factor (G-CSF) medications, such as filgrastim, with fewer reports associated with pegfilgrastim, but has also been associated with all-trans-retinoic acid, oral contraceptives, antiepileptics, antihypertensives, antibacterials, and vaccines.

CASE REPORT

A 45-year-old Caucasian female presented with fever (39.2° C), chills, and red spots on her right upper arm and right elbow as well as faint red spots on her left arm appearing 3 days prior. White blood cell count was 21.9 x 109/L. Her past medical history included grade 3 invasive ductal carcinoma with BRCA gene mutation (on chemotherapy), dermatomyositis (on hydroxychloroquine), necrotizing fasciitis subsequent to cesarean section, and tobacco abuse. Her last chemotherapy administration consisted of doxorubicin, cyclophosphamide, and pegfilgrastim (6 mg dose subcutaneous once) 14 days prior.

On dermatologic examination, over the arms, there were semi-firm subcutaneous plaques/nodules, some with overlying erythema (Figure 1). The morphology and clinical picture were concerning for infection (fungal favored over bacterial) versus Sweet syndrome secondary to pegfilgrastim administration. A punch biopsy of the right arm demonstrated skin with an unremarkable epidermis. Within the superficial and deep dermis, extending into the subcutaneous tissue, there was a mild perivascular and interstitial mixed inflammatory infiltrate composed of lymphocytes and neutrophils (Figure 2A and 2B). Histochemical stains for fungus (GMS) and acid-fast bacteria (Fite) were negative. Based upon histology, the differential