LETTERS TO THE EDITOR

 

Ménétrier's disease and Kaposi's sarcoma in a HIV-positive patient

Enfermedad de Ménétrier y sarcoma de Kaposi en un paciente VIH positivo

 

 

---

Key words: Ménétrier's disease. Kaposi's sarcoma. HIV.

Palabras clave: Enfermedad de Menetrier. Sarcoma de Kaposi. VIH.

---

 

 

Dear Editor,

Ménétrier's disease is an uncommon disease whose etiology remains unknown (1). Its association with Kaposi's sarcoma (KS) in patients with human immunodeficiency virus (HIV) infection has only been reported in two patients by diSibio et al. (2). Here we report one more case of this exotic association.

 

Case report

A 39-year-old male presents with burning epigastric pain, nausea, early satiety, vomiting, soft stools, 7 kg weight loss, asthenia, and adynamia for four months. Two weeks before his admission to hospital he had progressive edema in the lower extremities, and an episode of hematemesis. He had a history of syphilis that was treated 18 years ago. Upon admission he had anasarca and mucocutaneous pallor. Examination revealed epigastric tenderness with fluid wave and no masses or visceromegalies. Purple, vascular-looking, non-bleeding macules were seen on the hard palate, tongue floor, and glans, with laceration of the balano-preputial sulcus and grade-III edema in the lower limbs. ELISA found HIV positivity, which was confirmed with Western Blot, in addition to hypoproteinemia of unclear origin; cardiac, hepatic, renal, and nutritional causes were excluded.

Contrast-enhanced abdominal tomography revealed a polypoid thickening of gastric walls, and upper digestive endoscopy demonstrated an extensive, diffuse, multinodular, exophytic gastric lesion predominant in the body and fundus. The histopathologic study of the gastric biopsy showed severe foveolar cell proliferation resulting in deep invaginations and replacement of the original oxyntic mucosa. A KS was seen at the lamina propria, which was confirmed by immunolabeling for human herpes virus, type 8 (HHV8) (Fig. 1). Antiretroviral therapy was initiated, and subsequent chemotherapy with anthracyclines was scheduled.

 

 

Discussion

Ménétrier's disease is an idiopathic proliferative disorder of the mucosecretory epithelium in the gastric fundus and body, in association with hypoproteinemia and edema. Mucus hypersecretion leads to nutrient, basic electrolyte, and vitamin malabsorption at the intestinal level, and manifests as a protein-losing gastropathy (3). It is an uncommon condition with only a few hundreds of cases reported in the literature (4), and its joint presentation with HIV infection and KS is even rarer.

To our knowledge, this rare presentation has only been described on two occasions by diSibio et al. (2), who also reported the first association between Ménétrier's disease and KS. Additionally, DuPrey et al. (5) and Sánchez et al. (6) described two cases in patients with HIV infection and Ménétrier's disease, positing that HIV-1 might stimulate transforming growth factor α (TGF-α) secretion resulting in continuous activation of the gastric epithelium and increased mucus secretion, hence playing an important role in the pathogenesis of Ménétrier's disease. However, further studies are needed to confirm such theory.

The findings described herein are relevant because management for the conditions associated with Ménétrier's disease, in our case infection with HIV and KS, results in regression of the abnormal mucosa and symptom improvement (7,8). Similarly, Ménétrier's disease-related endoscopic and histopathologic findings should prompt screening for comorbid KS and/or HIV infection from now on.

 

Javier Baena-Del-Valle¹, Tatiana Villareal-Velasquez², Ariel Bello-Espinosa², Germán Márquez³,
José Posada-Viana³, Javier Segovia-Fuentes⁴ and César Redondo-Bermúdez^1
1Department of Pathology. School of Medicine. University of Cartagena. Laboratory of Pathology.
Hospital Universitario del Caribe. Cartagena, Colombia.
²Unit of Internal Medicine. Department of Medicine. School of Medicine. University of Cartagena. Cartagena, Colombia.
³Department of Surgery. School of Medicine. University of Cartagena. Cartagena, Colombia.
⁴Unit of Radiology. Department of Diagnosis. School of Medicine. University of Cartagena. Cartagena, Colombia

 

References

1. Famularo G, Sajeva MR, Gasbarrone L. Beyond gastritis and before cancer: The strange case of Ménétrier's disease. Intern Emerg Med 2011;6:369-71.         [ Links ]

2. diSibio G, McPhaul LW, Sarkisian A, Van Pham B, French SW. Ménétrier's disease associated with Kaposi's sarcoma. Exp Mol Pathol 2008;85:160-4.         [ Links ]

3. Lambrecht NWG. Ménétrier's disease of the stomach: A clinical challenge. Curr Gastroenterol Rep 2011;13:513-7.         [ Links ]

4. Scharschmidt BF. The natural history of hypertrophic gastrophy (Menetrier's disease). Report of a case with 16 year follow-up and review of 120 cases from the literature. Am J Med 1977;63:644-52.         [ Links ]

5. Duprey KM, Ahmed S, Mishriki YY. Ménétrier disease in an acquired immunodeficiency syndrome patient. South Med J 2010;103:93-5.         [ Links ]

6. Sanchez C, Brody F, Pucci E, Bashir S. Laparoscopic total gastrectomy for Ménétrier's disease. J Laparoendosc Adv Surg Tech A 2007;17:32-5.         [ Links ]

7. Di Vita G, Patti R, Aragona F, Leo P, Montalto G. Resolution of Ménétrier's disease after Helicobacter pylori eradicating therapy. Dig Dis 2001;19:179-83.         [ Links ]

8. Gadour MOE-H, Salman AH, El Samman ETEW, Tadros NM. Menetrier's disease: An excellent response to octreotide. A case report from the Middle East. Trop Gastroenterol 2005;26:129-31.         [ Links ]