Glucagonoma syndrome

Editor: unassigned

Introduction:

Glucagonoma syndrome:

Paraneoplastic syndrome associated with glucagon-producing (α-cell) pancreatic islet tumor
In 3% part of MEN1
In 70% presenting syndrome
5 year survival 50%
Hypercatabolic state induced by excessive levels of glucagon (and pancreatic polypeptide) leads to relative deficiency of Zn, free fatty acids, amino acids, vitamins B and perhaps other essential nutrient

Clinical Presentation:

Necrolytic migratory erythema (NME) (erythema, epidermal necrosis, bulla formation, shedding and hyperpigmentation) (B3, free fatty acids, B12)
Diabetes mellitus
Weight loss
Anemia (B12)
Stomatitis, cheilitis, glossitis (B2,B3)
Diarrhoea (B3)

Important considerations:

Glucagonoma syndrome is not the only syndrome associated with clinical features of migratory necrolytic erythema. Additional syndromes include:

Paraglucagonoma
Necrolytic aral erythema

Paraglucagonoma is a multifactorial nutritional deficiency syndrome associated with migratory necrolytic erythema in the context of:

Other pancreatic and non pancreatic cancers
Chronic pancreatitis
Celiac disease
Jejunal adenocarcinoma
Crohn’s disease
Hepatic cirrhosis

Clinical differential diagnosis:

Necrolytic acral erythema is a multifactorial nutritional deficiency syndrome associated with hyperkeratotic necrolytic erythema. Most cases were associated with Hepatitis C infection.

Histological features :

Acanthosis
Parakeratosis
Epidermal pallor
Epidermal cell vacuolization
Later stages: superficial and intraepidermal mixed infiltrate with lymphocytes, eosinophils and neutrophils
Residual: post-inflammatory hyperpigmentation

Histological pearls: