Introduction
A 47-year-old man, with no medical history, presented with a 1-year history of a recurrent skin eruption made of erythematous, erosive, crusty and pruritic plaques involving the scrotum, inguinal and intergluteal skinfolds, trunk and legs (figure 1). Mucosal examination showed glossitis and perleche. He had received topical and systemic antifungal agents without improvement. General examination was normal except a weight loss of 9 kg, without other digestive symptoms and despite a balanced diet. Blood routine tests were normal except a mild normocytic anaemia (11 g/dL) and a hypoalbuminaemia (31 g/L). Nutritional assessment revealed a zinc deficiency (7.37 µmol/L, normal value >12.5 µmol/L). Histological examination of a skin biopsy revealed a psoriasiform acanthosis with a keratinocyte vacuolation.
Figure 1.
Erosive and crusty erythematous plaque of the intergluteal fold.
Question
Which diagnosis should be evoked in the context of this clinical presentation?
Answer
Necrolytic migratory erythema (NME) (glucagonoma syndrome).
Clinical and histological features suggested the diagnosis of an NME or a zinc deficiency (whatever its cause). The blood rate of glucagon was high (1419 ng/L; normal value <74 ng/L). Abdominal CT scan showed a caudal pancreatic mass, confirmed by Ga68-DOTATOC PET/CT (SUVmax 61). Distal pancreatectomy and splenectomy were performed. Histological analysis showed a well-differentiated grade G1 neuroendocrine tumour, consistent with glucagonoma. The patient’s general condition and dermatosis improved after surgery and topical treatment with betamethasone.
Glucagonoma is a rare neuroendocrine tumour (incidence rate ~2.4/100 000 000/year).1 NME is present in 80% of cases.2 NME is characterised by migrating erythematous, erosive and/or crusted annular plaques frequently located in the intertriginous and periorificial areas. An oral mucosal involvement (cheilitis, glossitis) may be associated. NME-like lesions may also be present in zinc deficiency, pellagra, Kwashiorkor, liver dysfunction and cirrhosis.2 3 Histology shows keratinocyte vacuolisation and loss of the granular layer. Nonetheless, the diagnosis delay is often long.3 4 Blood glucose levels are normal in 30% of cases.2
The prognosis is variable, with an overall 5-year all-stage survival rate of 50%.2 Somatostatin analogues lead to the rapid relief of symptoms. The only curative treatment is surgery, feasible in case of localised disease or isolated liver metastases. In other cases, chemotherapy induces a partial response.5 Other treatments include tyrosine kinase and mTOR inhibitors (sunitinib and everolimus).
NME is a suggestive dermatological condition that should evoke the diagnosis of glucagonoma. Physicians should be aware of this eruption to shorten the diagnosis delay and thus improve the prognosis of patients.
Footnotes
Contributors: Submitting author is EA. Corresponding author is SI-H-O. EA and SI-H-O contributed to the conception of the work, collected data and drafted the work. SL, TB, JD, BP, LDM and ES contributed equally to the design of the work, critically reviewed the article. All authors gave final approval of the version to be published and agreed to be accountable for all aspects of the work.
Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests: None declared.
Provenance and peer review: Not commissioned; externally peer reviewed.
Ethics statements
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References
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