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[Necrolytic migrating erythema without glucagonoma]
H Maillard1, P Celerier, C Maisonneuve
1Service de Dermatologie, Centre Hospitalier General, Le Mans.
Annales De Dermatologie Et De Venereologie
|January 1, 1995
Summary
This case report highlights a patient with cirrhosis who developed erythema necroticans migrans (ENM), a rare skin condition. The findings suggest cirrhosis may play a key role in ENM development, potentially linked to zinc deficiency.
Area of Science:
- Dermatology
- Hepatology
- Endocrinology
Background:
- Glucagonoma syndrome, characterized by erythema necroticans migrans (ENM) and glucagonoma, is a recognized clinical entity.
- Cirrhosis is a significant risk factor for developing skin conditions, including ENM.
- Zinc deficiency can manifest with various dermatological symptoms.
Observation:
- A 43-year-old male with alcoholic cirrhosis and ascites presented with a 4-month history of centrifugal erythematous skin lesions.
- Skin lesions exhibited bullae and desquamation, initially on the hands and spreading widely, sparing periorificial areas.
- Laboratory findings included anemia, hypovitaminosis K, cholestatic liver failure, and low zinc levels.
Findings:
- Skin biopsy revealed a 'Neapolitan trench' image, suggestive of ENM.
- Despite zinc, amino acid, and vitamin supplementation, the patient's liver disease progressed, leading to death.
- The patient's presentation was distinct from Bazex's syndrome, acrodermatitis enteropathica, and other autoimmune blistering conditions.
Implications:
- This case suggests cirrhosis may be a primary driver of ENM, with zinc deficiency being secondary.
- The failure of supplementation underscores the complexity of managing ENM in cirrhotic patients.
- Further research into the role of essential fatty acids in ENM pathogenesis is warranted.