Necrolytic migratory erythema 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.
Signs and symptoms
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]
Associated conditions
William Becker first described an association between NME and
glucagonoma in 1942[2][3] and since then, NME has been described in as many as 70% of persons with a glucagonoma.[4] NME is considered part of the
glucagonoma syndrome,[5] 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".[6] Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:[7]
The histopathologic features of NME are nonspecific[8] and include:[9]
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]
Management
Managing the original condition,
glucagonoma, by
octreotide or surgery. After resection, the rash typically resolves within days.[10]
^
abcdefPujol RM, Wang CY, el-Azhary RA, Su WP, Gibson LE, Schroeter AL (January 2004). "Necrolytic migratory erythema: clinicopathologic study of 13 cases". International Journal of Dermatology. 43 (1): 12–8.
doi:
10.1111/j.1365-4632.2004.01844.x.
PMID14693015.
S2CID26012738.
^Becker WS, Kahn D, Rothman S (1942). "Cutaneous manifestations of internal malignant tumors". Archives of Dermatology and Syphilology. 45 (6): 1069–1080.
doi:
10.1001/archderm.1942.01500120037004.
^Marinkovich MP, Botella R, Datloff J, Sangueza OP (April 1995). "Necrolytic migratory erythema without glucagonoma in patients with liver disease". Journal of the American Academy of Dermatology. 32 (4): 604–9.
doi:
10.1016/0190-9622(95)90345-3.
PMID7896950.
^Wilkinson DS (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Transactions of the St. John's Hospital Dermatological Society. 59 (2): 244–50.
PMID4793623.
^Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I (July 1986). "Histologic variation in the skin lesions of the glucagonoma syndrome". The American Journal of Surgical Pathology. 10 (7): 445–53.
doi:
10.1097/00000478-198607000-00001.
PMID3014912.
S2CID19879900.