A Case of Pellagra Associated With Megaduodenum in a Young Woman

Abstract

Pellagra is a rare condition that has been known for many years to be related to niacin deficiency. Clinically known as the 4 "D" symptoms for dermatitis, diarrhea, dementia, and even death, skin changes remain one of the most important features of this pathology, leading frequently to the diagnosis. Pellagra is mostly seen in poor populations with a deficient diet; still, it is sporadically observed in developed countries, usually in association with digestive disorders. We report a new case of pellagra in a 29-year-old woman in whom the diagnosis of megaduodenum was made. Megaduodenum is a rare condition that can be idiopathic or secondary to visceral myopathy or neuropathy. Parenteral supplementation with niacin has resulted in a quick response of dermatological and psychiatric symptoms. To our knowledge, no case of pellagra due to megaduodenum has been reported in literature.

Pellagra affecting a patient with Crohn's disease

“… 2 The prevalence of the disease might be misleading due to underdiagnosis. 3 Pellagra is divided into two forms: primary pellagra, which results from inadequate niacin intake; and secondary pellagra, which occurs when other conditions interfere with niacin absorption and/or processing. These include chronic alcoholism, prolonged diarrhea, and biochemical impairment of tryptophan metabolism (which can be caused by diseases such as carcinoid syndrome, Crohn's disease, and Hartnup disease).…”

A rare case of idiopathic congenital megaduodenum in adult misinterpreted during childhood: case report and literature review

“…The etiology of primary functional megaduodenum remains unknown; however, some theories divide in 3 main causes as following: (1) paralytic, such as postvagotomy and in patients with Chagas disease; (2) neuropathic, including vitamin deficiency, congenital aganglionosis, porphyria, and diabetes mellitus ; and (3) myopathic, such as collagen diseases [3], [4], [5], [6], [12], [13]. In the presented case, there was no mechanical cause of obstruction and the histopathologic analysis of the duodenum did not demonstrate any significant abnormality in the duodenal wall and the myenteric plexus were normal.…”

“…Table 1 summarizes the main cases of megaduodenum described in the literature [1], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25]. Patients with megaduodenum often have abdominal distention, nausea, vomiting diarrhea, and malnutrition, especially in chronic cases and late presentation.…”

“…In childhood, mechanical megaduodenum is frequently caused by congenital duodenal stenosis; however, there are other causes of mechanical megaduodenum including congenital bands, annular pancreas, and superior mesenteric artery syndrome [1], [2]. Idiopathic megaduodenum in children is a rare disease defined as a massive dilation of the duodenum without mechanical obstruction [2], [3], [4], [5], [6]. Considering its rarity, idiopathic megaduodenum may be misdiagnosed and mimic other intestinal disorders, especially at nonexperienced centers.…”

Chronic diarrhea and pruritic rash related to pellegra after pancreaticoduodenectomy

“…The mechanism of this postoperative complication is not clear. Absorption of niacin occurs mainly in the stomach and upper small intestine 4 ; and pancreaticoduodenectomy, which involves the removal of the duodenum and a part of the stomach, could lead to insufficient niacin absorption. Moreover, the GI surgery seriously affects the function of digestion and absorption over the long term, which makes patients susceptible to develop nutrition deficiency-related skin diseases.…”