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Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients
C Hotz1, L Valeyrie-Allanore, C Haddad
1Department of Dermatology, Referral Center for Toxic and Autoimmune Blistering Diseases, Université-Paris-Est Créteil Val de Marne (UPEC), Henri-Mondor Hospital, 51, avenue du Maréchal de Tassigny, Créteil CEDEX, 94010, France.
Background:
Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts.
Objectives:
To investigate the frequency and clinical features of AGEP systemic involvement.
Methods:
This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work-up available.
Results:
Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C-reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone-marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments.
Conclusions:
The neutrophil count-systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.
