key: cord-0954427-fl8lis1j authors: Kinariwalla, Neha; London, Ashley O.; Soliman, Yssra S.; Niedt, George W.; Husain, Sameera; Gallitano, Stephanie M. title: A Case of Generalized Sweet’s Syndrome with Vasculitis Triggered by Recent COVID-19 Vaccination date: 2021-11-25 journal: JAAD Case Rep DOI: 10.1016/j.jdcr.2021.11.010 sha: 4ceecaf365ee98d8184e4d37108c62c1decccc89 doc_id: 954427 cord_uid: fl8lis1j nan Sweet's syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon 46 dermatosis characterized by fever, neutrophilia, and multiple erythematous painful plaques. 47 Histologically, it presents with a dense dermal neutrophilic infiltration. SS is often associated 48 with hematologic malignancies, including plasma cell dyscrasias. Herein, we report a rare case of 49 SS with vasculitis and transient IgA monoclonal gammopathy triggered by a Janssen 50 Ad26.COV2.S vaccine. 51 52 Report of a Case 53 A 54-year-old male with no significant past medical history presented to the emergency 54 department in significant pain, with tongue swelling and ulceration. The patient denied fevers, 55 chills, night sweats and weight loss. He received the Janssen Ad26.COV2.S vaccine nine days 56 prior to onset of symptoms. 57 On physical examination, he had an erythematous, fissured tongue with confluent erosions 58 ( Figure 1A ) and dozens of firm, blanching and erythematous and papulonecrotic nodules on the 59 extremities and trunk ( Figure 1B ). The scrotum had flesh colored and erythematous plaques. The 60 plantar feet had erythematous and targetoid macules. ( Figure 1C ).The patient's lesions 61 progressed over one week, and he developed new eroded and crusted plaques on the scalp, trunk, 62 and extremities. The genital ( Figure 1D ) and plantar foot lesions became more targetoid in 63 appearance. 64 Lab work revealed a leukocytosis to 10.84x10 3 /uL with 90% neutrophils and erythrocyte 65 sedimentation rate (ESR) of 29 mL/hour and C-reactive protein (CRP) of 19.6 mg/L. Serum 66 protein electrophoresis (SPEP) resulted in a positive abnormal monoclonal IgA lambda. 67 Additional rheumatologic, including anti-neutrophil cytoplasmic antibodies (ANCA) and 68 antinuclear antibody (ANA), and infectious workup was otherwise negative. The patient was 69 negative for COVID-19 on nasal polymerase chain reaction (PCR) and COVID serologies were 70 negative. Computed tomography of the chest, abdomen, and pelvis were unremarkable. 71 Three 3-millimiter punch biopsies were performed for direct immunofluorescence (DIF), 72 hematoxylin and eosin (H&E), tissue bacterial, fungal and AFB cultures. H&E demonstrated a 73 dense interstitial and perivascular neutrophilic infiltrate with leukocytoclasia and focal fibrin 74 deposition in blood vessel walls (Figure 2A-C) Figure 1: Physical exam revealed an enlarged, erythematous painful tongue with many papules and fissures with overlying 222 exudate (A), erythematous, firm nodules with slightly eroded centers on the dorsal hands and extremities (B), tan 157 158 159 160 161 162 163 164 165 166 167 168 169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185