HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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".
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EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
Mostly in postmenopausal women.
LocalizationThis section has been translated automatically.
Centrofacial, periorifcial, inguinal and perianal; rarely trunk and thigh.
Clinical featuresThis section has been translated automatically.
- Weight loss, stomatitis, intermittent diarrhea, tendency to thrombosis, diabetic metabolic condition, hyperglucagonemia, hyperproteinemia, hypokalemia, anemia, glossitis, weight loss.
- Bizarrely configured, centrifugally growing, annular or multi-arched, scaly erythema with pustular formation (detection of Staphylococcus aureus), incrustations and pigmentary shifts.
- Further angular cheilitis, nail dystrophies
Furthermore, signs of pellagra can be added by nicotinic acid deficiency (van Beek AP et al. 2004)
- The skin changes disappear after a complete resection of the mostly malignant A-cell islet tumor. However, since a large part of the symptomatic glucagonomas have already metastasized, the success of the surgical intervention regarding the dermatological symptoms may not be given.
- In "non-glucagon-induced" necrolytic erythema, after substitution with amino acids and zinc, the migratory necrolytic erythema heals (Thomaidou E et al. 2016).
LaboratoryThis section has been translated automatically.
Hyperglycemia, SGS elevation. Elevated plasma glucagon levels.
HistologyThis section has been translated automatically.
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 diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Tumor search and repair!
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Becker SW, Kahn D, Rothman S (1942) Cutaneous manifestations of internal malignant tumors. Arch Dermatol Syphilol 45: 1069-1080
- Bruce H et al (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98
- Leibovici V et al (1987) The glucagonoma syndrome. J Dermatol 14: 491-496
- Luber AJ et al (2015) Pediatric Necrolytic Migratory Erythema as a Presenting Sign of Glucagonoma Syndrome. Br J Dermatol doi: 10.1111/bjd.142982:
- Carrot Beater M et al (1999) Squamous cell carcinoma - associated necrolytic migratory erythema. dermatologist 50: 988-202
- Pfau A et al. (1995) The Glucagonom Syndrome - Clinic and Therapy. Z Hautkr 70: 725-728
- Pujol RM et al (2004) Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol 43: 12-18
- Rappersberger K et al (1987) The glucagonoma syndrome. Dermatologist 38: 589-598
- Thomaidou E et al (2016) Rapid Clearance of Necrolytic Migratory Erythema Following Intravenous Administration of AminoAcids. JAMA Dermatol 152:345-346
- van Beek AP et al (2004) The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol 151: 531-537
- Vogl A et al (2005) Skin and alcohol. J Dtsch Dermatol Ges 3: 788-790
- Zeng J et al (2003) Glucagonoma syndrome: diagnosis and treatment. J Am Acad Dermatol 48: 297-298
Incoming links (13)Glucagon; Glucagonom; Glucagonoma syndrome; Impetigo circinata; Necrolytic acral erythema; Necrolytic migratory erythema; Necrolytic migratory erythema; Pancreatic diseases skin changes; Paraneoplasia, fifth, obligatory cutaneous; Psoriasis erythema anulare centrifugum-like; ... Show all
Outgoing links (12)Acrodermatitis enteropathica; Biotin; Candidoses; Glucagon; Glucagonom; Paraneoplasia, cutaneous; Pemphigus diseases (overview); Psoriasis erythema anulare centrifugum-like; Pustule; Pustulose subcorneal; ... Show all
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