migratory necrolytic erythema; glucagonoma syndrome; erythema necrolytica migrans
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Etiology
- glucagonoma
- advanced hepatic cirrhosis
- bronchial carcinoma
- idiopathic* (may be related to nutritional deficiency)
* rare patients without underlying malignancy[2]
Epidemiology
- rare
- middle-aged to elderly
Pathology
- early skin lesions
- band-like upper epidermal necrosis
- retention of pyknotic nuclei
- pale keratinocyte cytoplasm
- electron microscopy
- vacuolar degeneration & lysis of organelles
Clinical manifestations
- intertrigous erythema, epidermal scales, erosions[2]
- inflammatory plaques enlarge with central clearing, resulting in large areas that become confluent
- borders with vesiculation to bulla formation, crusting & scaling
- gyrate, circinate, arcuate & annular arrangement of lesions
- distribution:
- flexures
- intertriginous areas
- perioral
- perigenital
- fingertips red, shining, erosive
- mucous membranes
Laboratory
Differential diagnosis
- acrodermatitis enteropathica
- zinc deficiency
- pustular psoriasis
- mucocutaneous candidiasis
- familial pemphigus
Management
- responds poorly to all forms of therapy
- partial response in some cases to zinc replacement
- surgical excision of glucagonoma may improve or resolve symptoms
More general terms
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 512
- ↑ Jump up to: 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018.