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