Acute generalized exanthematous pustulosis with airway mucosa involvement: A case report

Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by sterile pustules on erythematous skin associated with fever and leukocytosis. The annual incidence of AGEP is estimated to be 1-5 cases per million. Cases of AGEP with oral mucosa involvement have been reported. However, reports of AGEP involving airway mucosa are limited.

We report a 42-year-old woman with serious AGEP involving the airway mucosa. The patient initially developed fever and a small rash on her forehead and face. Over the next 2 d, she developed a diffuse, pustular rash over her trunk and legs. In addition, she complained of a cough with white foam-like sputum, chest tightness and dyspnea. Four days later, due to dyspnea, her mental status started to gradually deteriorate. She became more and more drowsy. Biopsies of the skin and airway mucosa suggested the diagnosis of AGEP. According to the European study of severe cutaneous adverse reactions group's scoring system, the patient scored +6 indicating a probable diagnosis of AGEP. She received intravenous methylprednisolone 120 mg/12 h for 3 d, and was eventually discharged in good condition. This patient had already experienced respiratory failure and airway mucosa involvement on admission; however, the clinicians had an insufficient understanding of AGEP. Glucocorticoid was administered for more than 10 d following onset of the disease, and her overall prognosis was satisfactory.

This case represents a rare clinical feature of AGEP and an important finding for clinicians.