Clinicopathologic manifestations of 36 korean patients with acute generalized exanthematous pustulosis: a case series and review of the literature

Abstract

Background: Acute generalized exanthematous pustulosis (AGEP) is a rare and severe subtype of drug eruption, characterized by acute, extensive, non-follicular, sterile pustules on an erythematous background, accompanied by fever and leukocytosis.

Objective: The purpose of this study was to characterize AGEP in Korean patients in terms of clinical, laboratory, and pathologic findings.

Methods: Thirty-six patients (M:F=17:19) with AGEP were identified from an extensive review of medical records over a 15 year period. All patient cases were confirmed by biopsy and fulfilled the diagnostic criteria.

Results: The patient ages ranged from 4~80 years (37.6+/-19.4). The incubation period was 1~23 days. The duration of disease was 5~14 days. Neutrophilia (36/36), high CRP (14/36), and eosinophilia (30/36) were common laboratory findings. A history of drug administration existed in 23 of 36 patients; herbal medications, lacquers and radiocontrast media were the unique causative drugs. Spongioform subcorneal or intraepidermal pustules in the epidermis was observed in all patients. Thirty-six patients were subdivided into 2 groups: group A (n=23) was strongly associated with known agents; and group B (n=13) had no identified causative agents. There was no significant difference between the 2 groups.

Conclusion: OUR RESULTS DEMONSTRATE THE CHARACTERISTIC FEATURES OF AGEP IN KOREAN PATIENTS AS FOLLOWS: lower identification of causative agents; herbal medications, lacquers, and radiocontrast media were the main causative agents; and no significant differences existed between the 2 groups.

Keywords: Acute generalized exanthematous pustulosis; Clinicopathologic manifestation; Korean.

Fig. 1

Multiple, pin-head sized, non-follicular pustules with an erythematous base on the trunk in case 36 (A) and the big folds of lower extremities in case 10 (B).

Fig. 2

Several, sterile and small pustules with edematous erythema and exfoliations in case 7.

Fig. 3

Marked edema of face and non erosive oral mucosal involvement in case 10.

Fig. 4

(A) Spongioform subcorneal pustules consisted of polymorphonuclear leukocytes in the epidermis (H&E, ×100, case 7). (B) Leukocytoclastic vasculitis in the dermis (H&E, ×100, case 1).