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When dermatology meets gastroenterology: a case of odynophagia
  1. Grigoriy E Gurvits
  1. Correspondence to Grigoriy E Gurvits, Department of Gastroenterology, New York University Medical Center, 530 First Avenue, SKI–9N New York, NY 10016, USA; g_gurvits{at}hotmail.com

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Clinical presentation

A 60-year-old woman with a long standing history of cutaneous pemphigus vulgaris (PV), was admitted to our hospital complaining of progressive dysphagia and odynophagia to both liquids and solids over a 2-month duration. Patient suffered from limited oral caloric intake and a 30-pound weight loss. Current medication included oral Prednisone and recently started Azathioprine. On physical examination, she had multiple denuded hyperaemic lesions involving soft palate, gingiva, uvula, and oropharynx. Numerous hyperpigmented cutaneous macules and crusted erosions in different stages of healing were appreciated, however, no skin bullae were seen. Upper endoscopy confirmed oral and pharyngeal findings and showed several friable zigzagging superficial ulcerations in the proximal and mid-oesophagus (figure 1A) with easy exfoliation of the mucosa on gentle passage of the gastroscope (figure 1B).

Figure 1

Oesophageal findings on endoscopy.

Question

What is the diagnosis?

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Answer

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Histological examination of biopsy specimen in our patient revealed acantholysis. No Candida hyphae were evident, and immunostains for cytomegalovirus and herpes simplex virus were negative. Direct immunoflorescence confirmed the suspected diagnosis of oesophageal PV (figure 2). Patient's symptoms significantly improved on high dose intravenous steroids and oral Azathioprine and she was discharged home tolerating soft diet.

Figure 2

Direct immunoflorescence of the oesophagus.

Diagnosis of oesophageal PV in absence of active cutaneous lesions is challenging and requires high index of suspicion. Although silent involvement of the oesophageal mucosa is very common and can be immunohistologically evident in up to 95% of PV patients,1 debilitating digestive disease symptoms are quite rare. Earlier studies have suggested its unique predilection to middle aged women who were in cutaneous remission.2 Multidisciplinary approach with gastrointestinal evaluation is essential and prompt endoscopy with biopsies will establish correct diagnosis and rule out other common causes of dysphagia and odynophagia, including infections. Nicholsky sign (figure 1B) in the oesophagus (easy exfoliation of the epithelium on slight rubbing with passage of an endoscope) is a classic phenomenon of the PV and is highly suggestive of the diagnosis.3 Dose escalation of immunosuppression is crucial and results in appropriate disease control and symptom resolution.

Acknowledgments

Dr Laura Barisoni for immunoflorescence slide review.

References

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; internally peer reviewed.