Necrolytic migratory erythema
Definition & Pathogenesis
- Necrolytic migratory erythema (NME) = obligate paraneoplastic dermatosis of the glucagonoma (neuroendocrine pancreatic tumor, α-cell tumor), first described as "Sweet & Mallinson glucagonoma syndrome" 1974.
- Pathogenesis: Mechanism is hyperglucagonemia + hypoaminoacidemia + zinc/niacin/tryptophan deficiency → epidermal necrolysis with defect of keratinocyte energy supply.
- Tumor: Causative tumor is glucagonoma (neuroendocrine α-cell pancreatic tumor).
- Pseudo-glucagonoma syndrome: NME without glucagonoma in liver cirrhosis, pancreatitis, celiac disease, malabsorption (pseudo-NME) — important DD.
Clinical Presentation
- Skin triad: Characteristic efflorescences are migratory, annular/circinate confluent erythemas + central bullae/erosions + marginal scaling + postinflammatory hyperpigmentation; perigenital + perianal + intertrigines + acra.
- Mucosa: glossitis (red-smooth tongue), angular cheilitis, stomatitis.
- System signs: Classic 4-D tetrad is Dermatitis (NME) + Diabetes mellitus + Drastic weight loss + Depression.
- Laboratory triad: hyperglucagonemia + hypoaminoacidemia + normochromic normocytic anemia.
- Thrombosis risk: ↑ in glucagonoma (paraneoplastic hypercoagulability).
Diagnostics
- Skin biopsy: Histological hallmark is necrolytic upper epidermal layer ("pale upper epidermis" with ballooning + confluence of necrotic keratinocytes) + parakeratosis; similar to pellagra / acrodermatitis enteropathica / necrolytic acral erythema.
- Laboratory: Guiding lab findings are plasma glucagon ↑↑ (often > 500 pg/ml, mostly > 1000 pg/ml) + amino acid levels ↓ + zinc/niacin often ↓ + hyperglycemia + normochromic anemia.
- Imaging: Recommended imaging is abdomen CT/MRI with contrast + somatostatin receptor PET/CT (⁶⁸Ga-DOTATATE) for tumor localization + staging.
- Genetics: rule out MEN-1 (multiple endocrine neoplasia type 1) in younger patients / family history.
Differential diagnoses
- Erythema ab igne
- Psoriasis vulgaris (plaque psoriasis)
- Nummular eczema
- Necrobiosis lipoidica
- Pemphigus vulgaris
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