migratory necrolytic erythema; glucagonoma syndrome; erythema necrolytica migrans

Etiology

• glucagonoma
• advanced hepatic cirrhosis
• bronchial carcinoma
• idiopathic* (may be related to nutritional deficiency)

* rare patients without underlying malignancy

Epidemiology

• rare
• middle-aged to elderly

Pathology

• early skin lesions
  • band-like upper epidermal necrosis
  • retention of pyknotic nuclei
  • pale keratinocyte cytoplasm
• electron microscopy
  • vacuolar degeneration & lysis of organelles

Clinical manifestations

• intertrigous erythema, epidermal scales, erosions
• inflammatory plaques enlarge with central clearing, resulting in large areas that become confluent
• borders with vesiculation to bulla formation, crusting & scaling
• gyrate, circinate, arcuate & annular arrangement of lesions
• distribution:
  • flexures
  • intertriginous areas
  • perioral
  • perigenital
  • fingertips red, shining, erosive
• mucous membranes
  • glossitis
  • angular cheilitis
  • blepharitis

Laboratory

• serum chemistries
  • hyperglycemia
  • glucagon
  • serum zinc
• skin biopsy

Differential diagnosis

• acrodermatitis enteropathica
• zinc deficiency
• pustular psoriasis
• mucocutaneous candidiasis
• familial pemphigus

Management

• responds poorly to all forms of therapy
• partial response in some cases to zinc replacement
• surgical excision of glucagonoma may improve or resolve symptoms

More general terms

• paraneoplastic dermatosis