Pseudoglucagonoma Syndrome Following Frey's Surgery: A Case Report of a Rare Presentation

Abstract

Pseudoglucagonoma syndrome is defined as the presence of necrolytic migratory erythema in the absence of a glucagon-secreting tumor. Necrolytic migratory erythema is the hallmark of glucagonoma syndrome but can also occur due to pancreatitis, pancreatic insufficiency, gastrointestinal dysfunction, inflammatory bowel disease, celiac disease, malabsorption disorders, nutritional deficiencies, hepatocellular dysfunction, and hypoalbuminemia. Pseudoglucagonoma syndrome is extremely rare, and the diagnosis is often delayed, resulting in delayed treatment. We report a rare case of pseudoglucagonoma syndrome in a malnourished male patient following Frey's surgery. The patient presented with a skin rash which gradually progressed over 20 days with diffuse hair loss. On cutaneous examination, multiple irregular erythematous and eroded plaques surrounded by a hyperpigmented scaly border were present over the dorsal aspect of the lower limbs, upper limbs, gluteal region, and genitals. Routine investigations showed normocytic normochromic anemia, neutropenia, lymphocytosis, dyslipidemia, and hypoalbuminemia. Rapid resolution of the skin lesions was observed with improved nutrition.

Keywords: enteroglucagon; frey's procedure; improved nutrition; necrolytic migratory erythema; pseudoglucagonoma syndrome.

Figure 1. Multiple irregular erythematous and eroded plaques surrounded by a hyperpigmented scaly border.

Figure 2. Multiple erythematous papules over the upper limb.

Figure 3. Hyperpigmented scaly plaques over the gluteal region.

Figure 4. Hyperpigmented scaly plaques over the genitals.

Figure 5. Epidermis showing hyperkeratosis, parakeratosis, irregular acanthosis, and spongiosis with focal ballooning of keratinocytes.

Figure 6. Dermis showing edema with perivascular lymphocytic and eosinophilic infiltrate.

Figure 7. Improvement of skin lesions over the gluteal region following nutritional supplementation.

Figure 8. Improvement of skin lesions over the lower limbs following nutritional supplementation.