Pemphigus vulgaris with exclusive involvement of the esophagus: case report and review

Faias, Sandra; Lage, Pedro; Sachse, Fernanda; Pinto, Anabela; Fidalgo, Paulo; Fonseca, Isabel; Nobre‐Leitão, Carlos

doi:10.1016/s0016-5107(04)01605-0

Cited by 54 publications

(16 citation statements)

References 20 publications

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“…As with cutaneous pemphigus vulgaris, acantholysis and intraepidermal vesicles are the characteristic histological features,10 this has been demonstrated in previous cases (figure 3). 2 7 11 Interestingly, acantholysis was not seen in the samples examined in this case, and only inflammatory infiltrates and reactive epithelial changes were present. The definitive diagnosis of pemphigus vulgaris is made by direct immunofluorescence.…”

Section: Discussionmentioning

confidence: 75%

“…Morbidity results from skin failure and steroid side effects. While mucosal involvement is common, esophageal disease is rarely reported and usually occurs in the context of previously diagnosed oral or cutaneous pemphigus vulgaris 2. Only three published cases could be found where esophageal involvement was the sole manifestation of the disease 2–4.…”

Section: Discussionmentioning

confidence: 99%

“…Ultimately, the goal is to induce remission and then minimise the steroid dose to avoid treatment complications and side effects. Three relatively recent cases demonstrated success in symptom control with high-dose oral prednisolone, though in two of these, long-term prednisolone was required for symptom control 2 4 13. Houghton et al used intravenous methylprednisolone first line to induce clinical remission, presumably due to the severity of the clinical presentation and endoscopic findings.…”

Section: Discussionmentioning

confidence: 99%

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Pemphigus vulgaris: a rare cause of dysphagia

Al‐Janabi¹,

Greenfield

2015

BMJ Case Reports

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Pemphigus vulgaris is a rare autoimmune blistering disease of the skin and mucous membranes. The case reported presented unusually with dyspepsia that was not responsive to protein pump inhibitor (PPI) therapy. This progressed to severe dysphagia and odynophagia. An esophagogastroduodenoscopy showed extensive ulceration of the esophagus, and direct immunofluorescence of an esophageal biopsy showed bright intercellular staining with C3 and IgG, confirming the diagnosis of pemphigus vulgaris. Immunological remission was achieved after a number of courses of pulsed intravenous methylprednisolone and cyclophosphamide. The patient has remained in remission for 5 years, but has required regular dilation of esophageal strictures for symptom relief. During this period, a chronic lymphocytosis was incidentally noted on routine blood tests, and chronic lymphocytic leukaemia was diagnosed. It is essential to investigate PPI-resistant symptoms, dysphagia and odynophagia, as they may indicate a serious underlying cause.

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Section: Discussionmentioning

confidence: 75%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pemphigus vulgaris: a rare cause of dysphagia

Al‐Janabi¹,

Greenfield

2015

BMJ Case Reports

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“…The disease may affect all races but may be relatively more common in people of Ashkenazi Jewish, Greek, and Indian descent [1]. The mean age of onset ranges between 40 and 60 [1,2]. Maleto-female ratio is approximately equal [1].…”

Section: Discussionmentioning

confidence: 99%

“…The Nikolsky's sign (i.e., induction of blistering via mechanical pressure at the edge of a blister or on normal skin) can often be elicited but is not specific [3]. Histologically, pemphigus vulgaris is characterized by loss of intercellular attachments evident as acantholysis in the suprabasal layer of the epidermis [1,2]. Direct immunofluorescence usually displays the presence of IgG with or without complement deposits between epidermal cells and within the dermis, whereas indirect immunofluorescence assays of patient serum detect circulating autoantibodies to Dsg3 [1].…”

Section: Discussionmentioning

confidence: 99%

Hard to Swallow: A Rare Cause

et al. 2015

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A 66-year-old woman underwent open-access upper endoscopy indicated for a two-month history of odynophagia and dysphagia for solids and liquids, described as difficulty in initiating a swallow followed by the subsequent sensation of food and liquids ''holding up'' in her mid-chest. Past medical history included type 2 diabetes mellitus, hypertension, and dyslipidemia; medications consisted of aspirin, rosuvastatin, losartan, hydrochlorothiazide, omeprazole, glipizide, metformin, oral potassium chloride, thyroid hormone, and insulin glargine. There had been no recent changes in medications or dosages. A focused physical examination prior to the endoscopy was unremarkable. Laboratory evaluation includes a complete blood cell count and basic metabolic panel that also revealed no abnormality. Endoscopy revealed an area of healing confluent ulceration extending over several centimeters in the hypopharynx (Fig. 1a). There was also severe, circumferential, erosive esophagitis that extended 5 cm proximally from the gastroesophageal junction (Fig. 1b). Biopsies of the distal esophagus were taken due to the possibility of a viral etiology of the severe ulceration.Following one of the esophageal biopsies, a prominent mucosal defect concerning for a superficial tear was noted for which an endoscopic hemoclip was deployed; no other complications of endoscopy occurred. In addition to standard anti-reflux measures, the patient was prescribed omeprazole.The esophageal biopsies were reported as showing a focally ulcerated fragment of squamous mucosa with associated fibropurulent material, consisting of mixed inflammatory cells. No nuclear or cytoplasmic inclusions were reported. After endoscopy, her primary care provider prescribed topical treatments for suspected aphthous hypopharyngeal ulceration. Four weeks after endoscopy, she complained of worsening oral pain without improvement of her initial symptoms of odynophagia and dysphagia. An oropharyngeal examination during a clinic visit revealed exudative hypopharyngeal erythema with cracking and ulceration of the buccal mucosa, consistent with the appearance of severe aphthous stomatitis. In view of these findings, buccal biopsies were obtained, which were reported as suprabasal epidermal acantholysis compatible with pemphigus vulgaris (Fig. 2a). Review of the first esophageal biopsy identified a single mucosal fragment with similar suprabasal acantholysis in the buccal specimen, confirming the diagnosis of esophageal pemphigus vulgaris (Fig. 2b). Direct immunofluorescence stain of the buccal specimen revealed a strong, intercellular, intramucosal deposition of IgG and C3, confirming the diagnosis of pemphigus vulgaris (Fig. 3). ManagementAfter the diagnosis of pemphigus vulgaris was confirmed, additional dermatologic evaluation revealed numerous skin and groin lesions, including a violaceous papule on the inferior surface of her abdominal pannus, and papular

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Endoscopic mucosal biopsy – histopathological interpretation

Montgomery¹,

Kalloo²

2015

Yamada' S Textbook of Gastroenterology

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Pemphigus vulgaris with exclusive involvement of the esophagus: case report and review

Cited by 54 publications

References 20 publications

Pemphigus vulgaris: a rare cause of dysphagia

Pemphigus vulgaris: a rare cause of dysphagia

Hard to Swallow: A Rare Cause

Endoscopic mucosal biopsy – histopathological interpretation

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