A 41-year-old female patient presented to our hospital emergency center with the chief complaint of hematemesis that developed 2 hours ago (total amount, >1 L). PV had been pathologically confirmed in this patient 5 years ago through skin biopsy. Her skin lesions had recurrently aggravated with the discontinuation of corticosteroids, and thus she had been on maintenance therapy for >3 years (oral methylprednisolone 10 mg daily and mycophenolate mofetil [MMF] 500 mg twice daily). She had no other medical history.

On physical examination, her initial blood pressure was 90/60 mm Hg, pulse rate was 97 beats per minute, respiratory rate was 20 breaths per minute, and body temperature was 37.5℃. She showed multiple scars on the face and upper chest due to the healing of earlier skin involvement of PV; however, she did not show any active bullae or desquamation. The baseline laboratory results were as follows: hemoglobin level, 10.7 g/dL; white blood cell count, 19,010/µL; and platelet count, 479,000/µL. The serum levels of routine chemistry markers were within the reference limits.

Initial esophagogastroduodenoscopy showed severe mucosal edema and erythema on the oropharynx and laryngeal area, with desquamation (Fig. 1A). From the upper esophageal sphincter to the lower esophagus, there was diffuse exfoliation of the mucosa with multiple linear ulcerations and erosions (Fig. 1B). There was no current active bleeding; however, subepithelial hemorrhage was present, with the friable mucosa bleeding easily on touching (Fig. 1C). The gastric mucosa and duodenal mucosa were well preserved without signs of hemorrhage or mucosal breakage. Because of the concern of additional bleeding risk, esophageal biopsy was not done. Immediately after the endoscopy was done, the patient experienced dyspnea and her oxygen saturation dropped to 88% on pulse oximetry monitoring, owing to severe laryngeal edema. She shortly recovered completely in a few minutes after supplementation with oxygen and administration of 100 mg intravenous hydrocortisone. Thereafter, she was maintained on intravenous hydrocortisone 100 mg twice daily for 6 days, and did not show any signs or symptoms of gastrointestinal bleeding or airway obstruction. After diet build-up and switching to oral methylprednisolone 16 mg daily with MMF 500 mg twice daily, she was discharged on the 11th day of hospitalization. We recommended a follow-up evaluation of esophagogastroduodenoscopy for pathologic confirmation of the diagnosis; however, the patient refused. She is currently in remission status on MMF 500 mg twice daily and methylprednisolone 8 mg daily, without recurrent hematemesis.

A 30-year-old female patient was referred to our hospital emergency center with hematemesis that started several hours before her presentation. The hematemesis had a total amount of 500 mL, had the color of fresh blood, and was combined with sloughed tissue material. PV had been diagnosed in this patient 11 months previously. Two weeks previously, her dermatologist had decided to increase the quantity of her medication because of new formation of vesicles on her skin. After switching from oral methylprednisolone 2 mg daily, cyclosporine 50 mg twice daily, and MMF 1,000 mg twice daily to oral methylprednisolone 4 mg daily, cyclosporine 150 mg twice daily, and MMF 1,000 mg twice daily, the skin lesions had shown improvement at the time of admission.

On physical examination, her initial blood pressure was 140/90 mm Hg, pulse rate was 118 beats per minute, respiratory rate was 20 breaths per minute, and body temperature was 36.5℃. She did not show any newly or recently developed skin lesions but had chronic scars due to PV on the trunk, inguinal, and perioral skin areas. The baseline laboratory results were as follows: hemoglobin level, 10.0 g/dL; white blood cell count, 16,940/µL; and platelet count, 262,000/µL. The serum levels of routine chemistry markers were within the reference limits.

Initial esophagogastroduodenoscopy results revealed multiple mucosal abrasions in the oral cavity (Fig. 2A), with combined laryngeal edema (Fig. 2B). In the upper esophagus, there was a longitudinal ulceration covered with exudate, and diffuse mucosal friability and erythema that was consistent with the findings of exfoliative esophagitis (Fig. 2C). There was no abnormality in the lower esophagus, stomach, and duodenum. After the administration of intravenous hydrocortisone 100 mg twice daily for 2 days, the patient did not show any other symptoms, and we were able to rapidly taper off the corticosteroids. On the third day of hospitalization, she underwent a follow-up esophagogastroduodenoscopy, which showed markedly improved mucosal exfoliative lesions in the oral cavity (Fig. 3A) and upper esophagus (Fig. 3B), for which biopsy was done. The final pathology report indicated detached squamous epithelium, with acute and chronic inflammation and necroinflammatory exudates. She was discharged on the sixth day of hospitalization with MMF 1,000 mg twice daily. Thereafter, she was referred back to a local clinic, and has not returned to our center.