Erythema necroticans migrans

Last Updated: 2022-01-21

Author(s): Anzengruber, Navarini

Becker et al. 1942

Glucagonoma syndrome, staphylodermia superficialis circinata, fifth obligate cutaneous paraneoplasia.

Tumour (mostly glucagon-secreting) of the pancreas (80% malignant neoplasms) that functions as an obligate cutaneous paraneoplasia. Other possible triggers are hepatitis B, C and other tumours (adenocarcinomas, bronchial carcinomas and squamous cell carcinomas)

Almost exclusively in postmenopausal women.

  • Pancreatic tumour
  • Other epithelial neoplasia
  • Hepatitis B
  • Hepatitis C

Interesting regarding pathogenetic considerations: Zinc and biotin deficiency result in very similar changes.

  • Circular, centrifugally growing, erosive erythema with blisters and pustules appear periorally, on the trunk, perineum and extremities
  • Diarrhoea, weight loss, abdominal pain, glossitis, stomatitis, cheilitis angularis, nail dystrophy
.
  • Anamnesis (increased tendency to thrombosis?)
  • Clinic
  • Laboratory
    • blood sedimentation rate (BSR) elevation
    • Glucagon to rule out hyperglucagonemia
    • Blood glucose values e.g. of diabetes mellitus
    • Blood count e.g. of a normocytic normochromic anaemia
    • Serum proteins e.g. hyperproteinemia
    • Potassium e.g. of hypokalaemia
  • Smear
    • Detection of Staphylococcus aureus from the pustule

Face, genitals around orifices, groin, proximal parts of the body in a broader sense can also be affected.

  • Pale vacuolated keratinocytes in the upper epidermis, leading to confluent necrosis, subcorneal or intraepidermal cleft formation possible. Parakeratosis with exoserosis.
  • Diffuse neutrophilic infiltration of the epidermis may occur concomitantly.

Treatment and - if possible - surgical removal of the tumour. This leads to a healing of the cutaneous symptoms.

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