J Rheum Dis.  2014 Dec;21(6):311-313. 10.4078/jrd.2014.21.6.311.

A Case of Behcet's Disease with Both Esophageal and Pharyngeal Ulcers Successfully treated with Steroid and Sulfasalazine

• Yoon HY
• Cho IJ
• Lee H
• Choi YJ
• Lee WS
• Yoo WH

• KMID: 2222935
• DOI: http://doi.org/10.4078/jrd.2014.21.6.311

Abstract

Behcet's disease is a heterogeneous disease that involves variable organ systems. Gastrointestinal Behcet's disease is rare and it generally affects the terminal ileum with propagation toward the cecum and ascending colon. Therefore, esophageal ulcer associated with Behcet's disease has not been reported frequently. We report an unusual case of Behcet's disease associated with both esophageal and pharyngeal ulcers. A 64-year-old man was admitted for an evaluation of pharyngeal and substernal discomfort sustained for 3 months. He had no underlying chronic disease; however, he suffered from recurrent oral and genital ulcers for 20 years and had folliculitis-like skin lesions on the face, scalp and trunk. He was diagnosed with Behcet's disease and gastroesophageal fiberscopy revealed deep ulcers on both the pharynx and upper esophagus. Esophagopharyngeal ulcers were successfully treated with prednisolone, colchicines, dapsone, and sulfasalazine.

Keyword

Esophageal ulcer; Behcet's disease; Steroid; Sulfasalazine

MeSH Terms

Cecum
Chronic Disease
Colon, Ascending
Dapsone
Esophagus
Humans
Ileum
Middle Aged
Pharynx
Prednisolone
Scalp
Skin
Sulfasalazine*
Ulcer*
Dapsone
Prednisolone
Sulfasalazine

Figure

• 

  Figure 1. Pharyngeal (A) and esophageal (B) ulcers in Behcet's disease.

• 

  Figure 2. Histopathologic findings of ulcer lesion (A) ×200, (B) ×100.

Reference

1. Yasuo M, Miyabayashi H, Okano T, Aoki H, Ichikawa K, Hirose Y. Successful treatment with corticosteroid in a case of Behç et's syndrome with multiple esophageal ulcerations. Intern Med. 2003; 42:696–9.

2. Yi SW, Cheon JH, Kim JH, Lee SK, Kim TI, Lee YC, et al. The prevalence and clinical characteristics of esophageal involvement in patients with Behç et's disease: a single center experience in Korea. J Korean Med Sci. 2009; 24:52–6.

3. Maruyama Y, Hisamatsu T, Matsuoka K, Naganuma M, Inoue N, Ogata H, et al. A case of intestinal Behç et's disease treated with infliximab monotherapy who successfully maintained clinical remission and complete mucosal healing for six years. Intern Med. 2012; 51:2125–9.

4. Almoznino G, Ben-Chetrit E. Infliximab for the treatment of resistant oral ulcers in Behç et's disease: a case report and review of the literature. Clin Exp Rheumatol. 2007; 25(4 Suppl 45):S99–102.

5. Sakane T, Takeno M, Suzuki N, Inaba G. Behç et's disease. N Engl J Med. 1999; 341:1284–91.

6. Lehmann H, Stutte HJ, Zierott G, Schlaak M. A case of Behcet's disease with multiple intestinal ulcerations (author's transl). Dtsch Med Wochenschr. 1975; 100:308–11.

7. Arma S, Habibulla KS, Price JJ, Collis JL. Dysphagia in Behç et's syndrome. Thorax. 1971; 26:155–8.

8. Mori S, Yoshihira A, Kawamura H, Takeuchi A, Hashimoto T, Inaba G. Esophageal involvement in Behcet's disease. Am J Gastroenterol. 1983; 78:548–53.

9. Yashiro K, Nagasako K, Hasegawa K, Maruyama M, Suzuki S, Obata H. Esophageal lesions in intestinal Behç et's disease. Endoscopy. 1986; 18:57–60.

10. Glosemeyer R, Deppe H, Dormann AJ, Wigginghaus B, Stolte M, Stadler R, et al. Ulcerative esophagitis and colitis as rare manifestations of Adamantiades-Behcet disease. Z Gastroenterol. 2001; 39:167–71.

11. Lorenzetti ME, Forbes IJ, Roberts-Thomson IC. Oesophageal and ileal ulceration in Behç et's disease. J Gastroenterol Hepatol. 1990; 5:714–7.

12. Ikezawa K, Kashimura H, Hassan M, Nakahara A, Yanaka A, Matsuzaki Y, et al. A case of Behç et's syndrome with esophageal involvement treated with salicylazosulfapyr-idine and prednisolone. Endoscopy. 1998; 30:S52–3.