Acta Dermato-Venereologica issue 50:1 98-1CompleteContent | Page 37

138 SHORT COMMUNICATION Staphylococcal Scalded Skin Syndrome Caused by a Rare Variant of Exfoliative-toxin-A + S. aureus in an Adult Immunocompromised Woman Simona SALUZZO 1 , Franziska LAYER 2 , Georg STINGL 1# and Georg STARY 1# Department of Dermatology, Division of Immunology, Allergy and Infectious Diseases, Medical University of Vienna, AT-1090 Vienna, Austria, and 2 Department of Infectious Diseases, National Reference Centre for Staphylococci and Enterococci, Division Nosocomial Pathogens and Antibiotic Resistances, Robert Koch Institute, Wernigerode, Germany. E-mail: [email protected] # These authors contributed equally. 1 Accepted Aug 29, 2017; Epub ahead of print Aug 30, 2017 Staphylococci may cause severe infections in children and immunocompromised adults (1). Staphylococ- cal scalded skin syndrome (SSSS), for example, is a potentially life-threatening disease caused by the hae- matogenous spread of exfoliative toxins (ET) of Stap- hylococc us aureus termed ET-A, ET-B or ET-D (2) and encoded by the respective chromosomal (eta, etd) and plasmid located genes (etb) (3); bullous impetigo (BI), on the other hand, results from the local impact of the very same exfoliatins. We report here a case of a SSSS caused by a rare ET-A-producing S. aureus strain in an adult woman affected by chronic lymphocytic B-cell leukaemia (CLBCL). with Ibrutinib. The patient had vesiculobullous lesions on an inflamed basis on her face, neck, trunk and left arm (Fig. 1A, B). Only a few blisters were intact; most were ruptured. Differential diagnoses included SSSS, a generalized variant of BI, toxic epidermal necrolysis (TEN), pemphigus vulgaris (PV) and toxic erythema of chemotherapy (TEC). Physical examination revealed fever (38°C) and cre- pitation on thorax auscultation. A chest X-ray was diag- nostic for pneumonia of the left lower lobe. The Tzanck test from a bullous lesion showed acantholytic cells with no sign of necrosis, making TEN unlikely. The Nikolsky sign was positive on clinically normal-appearing skin. Primary laboratory findings revealed elevated C-reactive protein of 20.88 mg/dl (normal < 0.5) and 9% relative CASE REPORT band neutrophils (normal 3.0–5.0%). We observed hy- poimmunoglobulinaemia, with IgG 340.0 mg/dl (normal A 63-year-old woman presented with fever and a bul- 700–160), IgA 28.5 mg/dl (normal 70–400) and IgM 6.6 lous skin eruption. She reported the intake of antibiotics mg/dl (NR, 40–230). one week earlier (amoxicillin and sulbactam) because Perilesional histology of a fresh bulla showed a neu- of an upper airway infection. Her past medical history trophilic infiltrate in the upper dermis, spongiosis and was remarkable for CLBCL, currently being treated acantholytic split formation in the stratum granulosum (Fig. 2A). A search for pemphigus antibodies yielded negative results. A gram stain of lesional skin revealed gram+ cocci in the split (Fig 2B). Bacterial cultures from blood and sputum, as well as swabs of the nose and lesional skin, were positive for methicillin-sensitive S. aureus (MSSA). DNA sequencing of the S. aureus-specific staphy- lococcal protein A (spa) and multi-locus sequence-typing (MLST) revealed spa-type t729, sequence type (ST) 88 for all isolates. Strains were positive for the gene encoding the exfo- liative toxin A (eta). A diagno- sis of SSSS was made and the patient received intravenous piperacillin-sulbactam (4.5 g i.v. Fig. 1. Clinical course. (A) Flaccid bullae formation of face, neck, trunk and left arm. (B) Twenty days 3 times daily for 10 days) until after initiation of antimicrobial therapy, lesions were healed without scarring. A written permission from the patient is given. complete remission (Fig. 1B). doi: 10.2340/00015555-2778 Acta Derm Venereol 2018; 98: 138–139 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.