[Show abstract][Hide abstract] ABSTRACT: Acute generalized exanthematous pustulosis (AGEP) is now a well-known clinical entity, characterized by acute onset with associated fever, and numerous non-follicular pin-head sterile pustules on erythematous background. The biopsy evidences subcorneal pustules resembling those of pustular psoriasis. However, polymorphic aspects such as pseudo-erythema multiforme purpuric lesions, and edema are often associated, and with the rapid self-healing course of this impressive pustulosis, allow the differential diagnosis with pustular psoriasis. Most cases of AGEP are drug induced, particularly by antibiotics and mainly beta-lactams. However, a number of other drugs, of which the list is increasing, may be responsible. Few cases are related to other causative factors such as viral infections or ultraviolet radiation.
Seminars in Cutaneous Medicine and Surgery 01/1997; 15(4):244-9. DOI:10.1016/S1085-5629(96)80037-X · 1.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The purpose of the present review is to update knowledge on acute generalized exanthematous pustulosis (AGEP) in terms of epidemiology, pathogenesis, cause, clinical features, diagnosis, and treatment.
AGEP is a rare reaction pattern attributed mainly to drugs. Drug-specific T cells (CD4+ and CD8+) and the production of interleukin-8/CXCL8 play an important role in its pathogenesis. A large-scale case-control study (EuroSCAR study) revealed a broad spectrum of drugs strongly associated with AGEP characterized by different time patterns (latent periods). Recent publications have supported the recognized role of individual drugs in the induction of AGEP and some have reported newly incriminated drugs. Many recent publications on AGEP have used the AGEP validation score (EuroSCAR group criteria) to establish the diagnosis. The value of in-vivo tests (mainly patch tests), in-vitro tests (the lymphocyte transformation test and cytokine release tests), or both for the identification of causative drugs has been demonstrated. Infections do not play a prominent role in the development of AGEP. There is no evidence for the assumption that AGEP is a variant of pustular psoriasis. Unique observations related to AGEP include a marked female predominance, a possible role for seasonality and a causal role for spider bites.
A broad spectrum of drugs is associated with AGEP, a T cell-mediated reaction. Genetic susceptibility and the possible role of other risk factors in AGEP should be further evaluated in larger studies of AGEP patients with a validated diagnosis.
Current Opinion in Allergy and Clinical Immunology 06/2009; 9(4):322-8. DOI:10.1097/ACI.0b013e32832cf64e · 3.57 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background Acute generalized exanthematous pustulosis (AGEP) is a rare severe pustular reaction pattern with a typical clinical picture.
Objectives To characterize the histopathological features of AGEP in a large series of cases with a validated diagnosis.
Methods A multinational retrospective histopathological study was conducted. It included 102 hospitalized patients (recruited within the EuroSCAR and RegiSCAR studies) with a validated diagnosis of probable or definite AGEP. A systematic description of the histopathological features in AGEP was done based on a standardized grading system.
Results Sub/intracorneal pustules (41%), intraepidermal pustules (20%) or combinations of them (38%) were observed in 102 cases. The pustules were usually large (> 15 keratinocytes) (82% and 89%, respectively) and regularly contained eosinophils (36% and 32%, respectively). Spongiform features were less prominent in the sub/intracorneal pustules compared with the intraepidermal pustules (44% and 95%, respectively). The main epidermal features were necrotic keratinocytes (67%), including incidental segmental necrosis (7%), and spongiosis (80%) with neutrophil exocytosis (77%). The main dermal features were papillary oedema (88%) and mixed superficial (100%), interstitial (93%), and mid/deep-dermal infiltrates (95%) containing neutrophils (100%) and eosinophils (81%). Follicular pustules were also seen (23%), but vasculitis generally was absent. Classical features of plaque-type psoriasis were infrequent and usually mild. No significant differences were observed between a subgroup of 16 cases with and 86 cases without psoriasis.
Conclusions The present histopathological study concerns a large series of cases with a validated diagnosis of AGEP. It provides diagnostic clues in favour of AGEP in patients with a pustular eruption.
British Journal of Dermatology 08/2010; 163(6):1245 - 1252. DOI:10.1111/j.1365-2133.2010.09967.x · 4.28 Impact Factor
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