A Rare Case of Pemphigus Vulgaris With Esophageal Involvement: Spontaneous Expulsion of a 50-cm Mucosal Lumenoid Fragment

To the Editor: A 54-year-old man presented to the hospital with a chief complaint of oral ulcers and erosions persisting for >2 months, accompanied by blisters on the scalp and trunk for 1 month. Two and a half months before presentation, he developed multiple idiopathic oral ulcers. Despite combined use of cephalosporin antibiotics and topical Traditional Chinese Medicine (TCM), skin lesions remained unresponsive and worsened progressively. One month before, the patient presented with nonpruritic, painless vesicles on the head and face, without arthrodynia or discomfort in swallowing. The patient visited our stomatology department 12 days ago. With a clinical suspicion of pemphigus vulgaris (PV), 15 mg prednisone was prescribed daily. Notably, new trunk blisters appeared. The patient has a 3-year history of hypertension with poor blood pressure control. On physical examination, symmetric multifocal erosions and ulcers of different sizes on bilateral buccal mucosa and oral commissures, with white to yellowish-white pseudomembranous exudate (Figs. 1A, B). On the head, face, and trunk, mung bean- to peanut-sized blisters on erythematous macules are scattered (Fig. 1C). Blisters are clear, with thin, slightly tense walls. Nikolsky sign is negative. Most lesions have eroded and crusted. Penile examination reveals diffuse erythematous macules, vesiculoerosions, and crusted lesions (Fig. 1D).FIGURE 1.: A, B, Symmetric multifocal erosions and ulcers on bilateral buccal mucosa and oral commissures. C, Several blisters on the trunk, some were eroded and crusted. D, Diffuse erythematous macules, vesiculoerosions, and crusted lesions on the penile. E, Skin biopsy shows intraepidermal blisters with suprabasal acantholysis (100×).Indirect immunofluorescence results indicate a positive desmoglein 1 (Dsg1) antibody level of 150 U/mL, a positive desmoglein 3 (Dsg3) antibody level of 249 U/mL, and a negative bullous pemphigoid 180 (BP180) antibody. Esophagoscopy showed normal mucosa (Figs. 2A, B). Histopathology revealed active chronic moderate superficial gastritis with focal intestinal metaplasia in gastric antral mucosa and positive Helicobacter pylori. Histopathology of right buttock skin biopsy shows intraepidermal blisters with suprabasal acantholysis (Fig. 1E).FIGURE 2.: A, B, Esophagoscopy showed normal mucosa. C, A 50-cm mucosal, lumen-like tissue fragment was expelled through emesis by the patient. D, Histopathology of the mucosal, lumen-like tissue fragment revealed extensive acantholysis and squamous epithelial exfoliation (100×).Postesophagogastroscopy, the patient experienced acute oropharyngeal obstruction while swallowing a Caltrate D tablet. After coughing and retching, a 50-cm mucosal, lumen-like tissue fragment (Fig. 2C) was expelled. Histopathology revealed extensive acantholysis and squamous epithelial exfoliation (Fig. 2D). Macroscopic examination suggested esophageal origin, while microscopic findings were consistent with pemphigus vulgaris. The patient was diagnosed with esophageal PV. After treatment with systemic glucocorticoids, fasting, gastric mucosal protection, and fluid replacement, the patient recovered normal diet tolerance without complications. DISCUSSION PV is an autoimmune blistering disorder, distinguished by the presence of autoantibodies that target desmoglein (Dsg) 3 and 1.1 Mucosal membrane involvement is prevalent among patients with PV, and emerging research indicates a notably high prevalence (46%–87%) of esophageal involvement.2 Esophagogastroscopy is crucial for assessing pharyngeal–esophageal involvement in PV, detecting cryptic lesions during remission and screening for gastrointestinal neoplasms.2 However, fragile mucosa of patients with PV makes esophagogastroscopy risky.2–5 The literature shows that upper endoscopy revealed normal esophageal mucosa during initial passage, but diffuse hyperemia, erosions, and mucosal sloughing were observed on withdrawal.4 Thus, endoscopic procedures must be executed precisely to avoid mucosal iatrogenic injury. In severe cases, this can lead to life-threatening complications, especially acute gastrointestinal bleeding. This patient with PV exhibited typical manifestations, beginning with oral mucosal erosions and ulcers, then developing extensive blistering. Notably, gastrointestinal symptoms were absent, and endoscopy showed no esophageal involvement. However, a rare manifestation of mucosal avulsion occurred after the endoscopy. That owing to the patient retching after swallowing a large, lodged calcium tablet, indicating pre-existing esophageal mucosal fragility. It implies that esophageal mucosal involvement in pemphigus vulgaris may be underdiagnosed. The absence of gastrointestinal manifestations often eludes clinical detection. Even with symptom remission, cryptic esophageal involvement may persist.1 Besides, postendoscopic nursing is essential for optimizing patient outcomes and minimizing risks. CONCLUSIONS Esophagogastroscopy is indispensable for diagnosing esophageal involvement in the treatment of patients with PV, regardless of gastrointestinal symptoms. And to safeguard the delicate gastrointestinal mucosa, patients must avoid abrasive, large, or highly irritating foods postprocedure.