Acute generalized exanthematous pustulosis (AGEP) is a rare entity characterized by fever associated with the sudden appearance of erythematous lesions, on which multiple sterile, non-follicular pustules develop. We describe a case of a 44-year-old healthy male who developed fever and multiple erythematous and edematous lesions with progressive generalization to the entire body, associated with multiple small non-follicular pustules three days after having started flucloxacillin for the treatment of a furuncle. Considering the characteristics of the exanthema, fever, and association with aminopenicillin initiation, AGEP was considered. A skin biopsy revealed subcorneal and superficial epidermal pustules, with foci of spongiosis, papillary edema, and a superficial, perivascular inflammatory cell infiltrate with neutrophils and eosinophils, consistent with the clinical diagnosis of AGEP. The culprit drug was suspended, and prednisolone was started, considering the rash extension, with progressive and complete improvement. Although it is a rare condition, the hypothesis of AGEP should be considered in acute febrile conditions with disseminated pustules. It resolves spontaneously after discontinuation of the offending drug, and the diagnosis is based on clinical presentation and skin biopsy.
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http://dx.doi.org/10.7759/cureus.32073 | DOI Listing |
Acta Dermatovenerol Croat
November 2024
Prof. Marija Jelušić, MD, PhD, Department of Paediatrics, University of Zagreb, School of Medicine, Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, 10 000 Zagreb, Croatia;
Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery.
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