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We report an adolescent girl with genetically confirmed Bardet–Biedl 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–clavulanic 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 Suspected medications were withdrawn, pharmacovigilance reporting was performed and supportive dermatological care was initiated.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.