Vitiligo-associated jejunal xanthomatosis leading to small bowel obstruction

Abstract

Introduction: Xanthomatosis is characterized by the accumulation of lipid-laden-foamy macrophages, leading to plaque or nodule formation. The most common causes of intestinal obstruction are postoperative adhesion, hernia, and tumors. While cases of jejunal xanthomatosis leading to small bowel obstruction have been rarely documented in the literature, no prior reports have described its occurrence in association with vitiligo.

Case presentation: A 32-year-old male presented with features of small bowel obstruction. Per abdominal examination and digital rectal examination didn't reveal any positive findings. Although there was no evidence of cutaneous xanthomas, multiple hypo-pigmented macular lesions were present over body, suggestive of vitiligo. Contrast-enhanced computed tomography (CECT) of abdomen and pelvis showed dilated duodenum and proximal jejunum, with transition point in proximal jejunum. Exploratory laparotomy revealed a conglomerated mass located 20 cm distal to duodeno-jejunal (DJ) flexure, adherent to the transverse colon, causing complete obstruction of the jejunum. Resection anastomosis of the involved segments of jejunum and transverse colon was done. Later on, histopathology revealed the mass as jejunal xanthomatosis.

Discussion: Although jejunal xanthomatosis is asymptomatic, it may rarely present with features of intestinal obstruction. It may be associated even with vitiligo. In vitiligo, elevated levels of cytokines such as IL-6 and TNF-α increase vascular permeability, leading to the deposition of lipids and macrophages in the interstitium, which may contribute to the formation of xanthomas or xanthomatosis.

Conclusion: Intestinal xanthomatosis, though rare, can be considered as one of the differential diagnoses in patients presenting with features of bowel obstruction.

Keywords: jejunal xanthomatosis; obstruction; small bowel; vitiligo; xanthoma.

Figure 1.

Hypo-pigmented macular lesion on anterior abdominal wall, chest wall, dorsal surface of hand and foot.

Figure 2.

Black arrow showing dilated duodenum and yellow arrow showing dilated proximal jejunum.

Figure 3.

Black arrow showing jejunal segment, blue arrow showing segment having stricture along with jejunal xanthomatosis, green arrow showing transverse colon firmly adhered to jejunal segment.

Figure 4.

Xanthomatous histiocytes (H&E 40×).

Figure 5.

(a) Xanthomatous histiocytes involving the serosal layer (H&E 10×). (b) Full-thickness jejunal wall xanthomatosis in muscularis and serosal layer (H&E 4×).