Erythema necrolyticum migrans L99.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.07.2021

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Synonym(s)

Erythema necroticans migrans; Fifth obligatory cutaneous paraneoplasia; impetigo circinata; Necrolytic migratory erythema; Paraneoplasia fifth obligate cutaneous; staphylodermia superficialis circinata

History
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Becker, Kahn and Rothmann, 1942

Definition
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Rare dermatological symptoms as partial manifestation of a glucagonoma.

Originally defined as obligatory cutaneous paraneoplasia in glucagonecystic pancreatic tumor(glucagonoma: approx. 80% metastasized at diagnosis) (also known as glucagonoma syndrome). The thesis of "tumor specificity" has been challenged by recent reports after associations with hepatitis B and C, adenocarcinoma, bronchial carcinoma and squamous cell carcinoma have been described.

Thus the erythema necrolyticum migrans is more comprehensively defined as "a polyetiological, monitoring sign of the skin, characterized by a chronic, anular or multi-cellular, scaly, often pustule-bearing or crusty, peripherally progressive erythema".

Etiopathogenesis
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It is postulated that the increased glucagon leads to a catabolic metabolic state with a deficiency of zinc and various amino acids. This hypothesis is supported by the rapid response of the skin changes to amino acid substitutions. It is remarkable that the clinical and histological picture does not differ from skin changes in zinc deficiency syndrome or biotin deficiency.

Manifestation
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Mostly in postmenopausal women.

Localization
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Centrofacial, periorifcial, inguinal and perianal; rarely trunk and thigh.

Clinical features
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Internal:

  • Weight loss, stomatitis, intermittent diarrhea, tendency to thrombosis, diabetic metabolism, hyperglucagonemia, hyperproteinemia, hypokalemia, anemia, glossitis, weight loss.

Dermatologic:

  • Bizarrely configured, centrifugally growing, annular or multibanded, scaly erythema with pustular formation (evidence of Staphylococcus aureus), crusting, and pigmentary shifts.
  • Furthermore angular cheilitis, nail dystrophies.
  • Furthermore, signs of pellagra may be added due to nicotinic acid deficiency (van Beek AP et al 2004).

Note:

  • The skin lesions disappear after complete resection of the mostly malignant A-cell islet cell tumor. However, since a large part of the symptomatic glucagonomas has already metastasized, the success of the surgical intervention with regard to the dermatological symptoms may not be given.
  • In the case of "non-glucagon-induced" necrolytic erythema, the migratory necrolytic erythema heals after substitution with amino acids and zinc (Thomaidou E et al. 2016).

Laboratory
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Hyperglycemia, SGS elevation. Elevated plasma glucagon levels.

Histology
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In the acute stage image of a subcorneal pustule with ballooned, necrobiotic and dyskeratotic keratinocytes. Typical is the detection of pale necrobiosis zones (maturation defect) running through the surface epithelium in a street pattern. Confluent parakeratosis with exoserosis and penetrating neutrophilic leukocytes. Superimposed orthokeratosis.

Differential diagnosis
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Therapy
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Tumor search and repair!

Progression/forecast
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Healing of the skin changes after tumour removal.

Literature
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  1. Becker SW, Kahn D, Rothman S (1942) Cutaneous manifestations of internal malignant tumors. Arch Dermatol Syphilol 45: 1069-1080
  2. Bruce H et al (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98
  3. Leibovici V et al (1987) The glucagonoma syndrome. J Dermatol 14: 491-496
  4. Luber AJ et al (2015) Pediatric Necrolytic Migratory Erythema as a Presenting Sign of Glucagonoma Syndrome. Br J Dermatol doi: 10.1111/bjd.142982:
  5. Carrot Beater M et al (1999) Squamous cell carcinoma - associated necrolytic migratory erythema. dermatologist 50: 988-202
  6. Pfau A et al. (1995) The Glucagonom Syndrome - Clinic and Therapy. Z Hautkr 70: 725-728
  7. Pujol RM et al (2004) Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol 43: 12-18
  8. Rappersberger K et al (1987) The glucagonoma syndrome. Dermatologist 38: 589-598
  9. Thomaidou E et al (2016) Rapid Clearance of Necrolytic Migratory Erythema Following Intravenous Administration of AminoAcids. JAMA Dermatol 152:345-346
  10. van Beek AP et al (2004) The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol 151: 531-537
  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

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Last updated on: 29.07.2021