Ultrastructural Findings in Acrodermatitis Enteropathica

Abstract

ExtractDuodenal biopsies from patients with acrodermatitis enteropathica were examined by electron microscopy and dissecting microscopy. T h e ultrastructural studies revealed characteristic inclusions in Paneth cells with a maximum length of 7 pm. These findings were constant in four different patients. A follow-up study in one patient showed the same alterations before and after treatment. SpeculationT h e biochemical defect of acrodermatitis enteropathica is unknown and, therefore, typical ultrastructural alterations challenge one to investigate the nature of the included material.

Fibrillary inclusions in neoplastic and fetal acinar cells of the pancreas

“…Some reports have suggested that the inclusion is similar to the cytoplasmic inclusions which are seen in Paneth cells of the bowel in cases of acrodermatitis enteropathica or experimentally induced zinc deficiency [19,24]. However, the fibrillary-like structure of the inclusions did not form a bundle.…”

Acrodermatitis enteropathica

“…The aetiology of the watery diarrhoea is unknown; it may represent an intestinal infection or impaired mucosal function. Several intestinal mucosal lesions have been reported; these include a zinc responsive small intestinal villous atrophy (Kelly et al, 1976), and inclusion bodies and abnormal intracellular organetles in the paneth cells and the enterocytes (Lombeck et al, 1974;Braun et al, 1976;Elm~ et aI., 1982;Neldner et at., 1974). These changes are probably secondary to but not peculiar to zinc deficiency (Baudon et al, 1978;Elmes et al, 1982).…”

Fatty acid composition of plasma lipids in acrodermatitis enteropathica before and after zinc supplementation

“…In zinc-deficient rats, absorption of olive oil and triolein was found to be impaired, while normal TG digestion and therefore no steatorrhoea were present [26]. The resynthesised TG were found to accumulate in the intestinal mucosa as lipid droplets, 24 ttm in size, similar to the Paneth cell granulae found in AE [27]. Like the plasma FA changes in our patients, the inclusion bodies in intestinal epithelial cells receded after zinc supplementation [22].…”

“…Electron microscopy of a small bowel biopsy revealed pleomorphic Paneth cell granulae characteristic for AE [27]. Oral therapy with zinc sulfate, 3 x 50 mg/day, was started at the age of 6 months and a body weight of 4.4 kg, while the child was fed with Alfar6 and small amounts of breast milk.…”