Necrolytic migratory erythema
| Necrolytic migratory erythema | |
|---|---|
| Classification and external resources | |
| Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
| DiseasesDB | 8833 |
| eMedicine | derm/168 |
| Patient UK | Necrolytic migratory erythema |
Necrolytic migratory erythema (NME) is a red, blistering rash that spreads across the skin. It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.
Contents
Appearance
Clinical features
NME features a characteristic skin eruption of red patches with irregular borders, intact and ruptured vesicles, and crust formation.[1] It commonly affects the limbs and skin surrounding the lips, although less commonly the abdomen, perineum, thighs, buttocks, and groin may be affected.[1] Frequently these areas may be left dry or fissured as a result.[1] All stages of lesion development may be observed synchronously.[2] The initial eruption may be exacerbated by pressure or trauma to the affected areas.[1]
Histology
The histopathologic features of NME are nonspecific[3] and include:[4]
- epidermal necrosis
- subcorneal pustules
- confluent parakeratosis, epidermal hyperplasia, and marked papillary dermal hyperplasia in a psoriasiform pattern
- angioplasia of papillary dermis
- suppurative folliculitis
The vacuolated, pale, swollen epidermal cells and necrosis of the superficial epidermis are most characteristic.[2] Immunofluorescence is usually negative.[2]
Cause
The etiology of NME is unknown, although various mechanisms have been suggested. These include hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease.[2] In addition to the etiology being unknown, the pathogenesis is also unknown.
Associated conditions
William Becker first described an association between NME and glucagonoma in 1942[2][5] and since then, NME has been described in as many as 70% of individuals with a glucagonoma.[6] NME is considered part of the glucagonoma syndrome,[7] which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia.[2] When NME is identified in the absence of a glucagonoma, it may be considered "pseudoglucagonoma syndrome".[8] Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:[9]
- Celiac disease
- Ulcerative colitis
- Crohn's disease
- Hepatic cirrhosis
- Hepatocellular carcinoma
- Lung cancer, including small cell lung cancer
- Tumors that secrete insulin- or insulin-like growth factor 2
- Duodenal cancer
See also
References
- ↑ 1.0 1.1 1.2 1.3 Lua error in package.lua at line 80: module 'strict' not found.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
