Erythema necroticans migrans

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 to exclude diabetes mellitus
  • Blood count to exclude a normocytic normochromic anaemia
  • Serum proteins to exclude hyperproteinemia
  • Potassium to exclude 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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2. Tanner, M., J. Brasch, and E. Christophers, [Erythema necroticans migrans without glucagonoma]. Hautarzt, 1994. 45(7): p. 480-3.
3. Rockl, H., J. Metz, and C. Ackermann-Schopf, [Staphylodermia superficialis circinata. The 5th obligatory cutaneous paraneoplasia]. Hautarzt, 1977. 28(4): p. 178-84.
4. Bruce H et al. (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98
5. Leibovici V et al. (1987) The glucagonoma syndrome. J Dermatol 14: 491-496
6. Möhrenschläger M et al. (1999) Plattenepithelkarzinom - assoziiertes nekrolytisches migratorisches Erythem. Hautarzt 50: 988-202
7. Maillard, H., et al., [Necrolytic migrating erythema without glucagonoma]. Ann Dermatol Venereol, 1995. 122(11-12): p. 786-8.
8. Pfau A et al. (1995) Das Glukagonom-Syndrom - Klinik und Therapie. Z Hautkr 70: 725-728
9. Pujol RM et al. (2004) Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol 43: 12-18
10. Rappersberger K et al. (1987) Das Glukagonom-Syndrom. Hautarzt 38: 589-598
11. Vogl A et al. (2005) Skin and alcohol. J Dtsch Dermatol Ges 3: 788-790
12. Zeng J et al. (2003) Glucagonoma syndrome: diagnosis and treatment. J Am Acad Dermatol 48: 297-298
13. Wilde, O., et al., [Erythema necroticans migrans in non-glucagon-secreting pancreas tumor]. Hautarzt, 2007. 58(12): p. 1014-6.