{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T12:21:01Z","timestamp":1788351661632,"version":"build-2803163510"},"reference-count":15,"publisher":"BMJ","issue":"6","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,5,27]]},"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:p>We report an adolescent girl with genetically confirmed Bardet\u2013Biedl syndrome complicated by end-stage renal disease, epilepsy, psychomotor delay and cardiac anomalies. During intensive care for postictal coma and presumed sepsis, she received phenobarbital, amoxicillin\u2013clavulanic acid and ceftriaxone. Four days after admission, she developed acute generalised exanthematous pustulosis, marked by febrile erythroderma with numerous sterile, non-follicular pustules predominantly involving major flexural areas. Laboratory findings showed neutrophilic leucocytosis, eosinophilia and profound thrombocytopenia, precluding skin biopsy. The clinical chronology, morphology and biological features supported a definite diagnosis, with a EuroSCAR score of 9. Suspected medications were withdrawn, pharmacovigilance reporting was performed and supportive dermatological care was initiated. The cutaneous eruption improved, with regression of pustules and residual desquamation; however, the patient deteriorated and died of refractory septic shock.<\/jats:p>","DOI":"10.1136\/bcr-2025-271201","type":"journal-article","created":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T11:40:15Z","timestamp":1780918815000},"page":"e271201","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Acute generalised exanthematous pustulosis during fatal refractory shock in Bardet\u2013Biedl syndrome: a complex multidrug reaction in a medically fragile child"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-1101-3137","authenticated-orcid":false,"given":"Chaimae","family":"Bouhamdi","sequence":"first","affiliation":[{"name":"Department of Dermatology and Venereology, Hassan II University Hospital, Faculty of Medicine, Pharmacy and Dental Medicine, Sidi Mohamed Ben Abdellah University, Fez, Morocco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zakia","family":"Douhi","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Hassan II University Hospital, Faculty of Medicine, Pharmacy and Dental Medicine, Sidi Mohamed Ben Abdellah University, Fez, Morocco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meryem","family":"Soughi","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Hassan II University Hospital, Faculty of Medicine, Pharmacy and Dental Medicine, Sidi Mohamed Ben Abdellah University, Fez, Morocco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Elloudi","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Hassan II University Hospital, Faculty of Medicine, Pharmacy and Dental Medicine, Sidi Mohamed Ben Abdellah University, Fez, 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