Necrolytic migratory erythema: clinicopathologic study of 13 cases.
BACKGROUND
The clinical mucocutaneous manifestations of glucagonoma syndrome are recognized easily when they occur in the classic pattern of acral or periorificial lesions evolving in recurrent crops, with an annular and migratory distribution, in a patient with diabetes mellitus who has had recent weight loss and anemia. Not infrequently, noncharacteristic clinical and histopathologic features are observed and, in these cases, the diagnosis of pancreatic neoplasm may be delayed.


OBJECTIVE
To review the clinical and histopathologic features of cutaneous manifestations of glucagonoma syndrome.


METHODS
The clinicopathologic features of 13 patients (eight women) with widespread or localized cutaneous eruption as a manifestation of islet cell pancreatic carcinoma with marked glucagon secretion (glucagonoma) were reviewed.


RESULTS
The definitive diagnosis of the cutaneous eruption was established at the time of diagnosis of the pancreatic neoplasm (three patients) or afterwards (10 patients). In nine patients, the mucocutaneous manifestations preceded the diagnosis of the pancreatic neoplasm by 1 month to 3 years (mean, 12 months). In only eight biopsy specimens were the histopathologic features considered to be suggestive or characteristic of necrolytic migratory erythema. Diffuse parakeratosis, that occasionally arose abruptly from normal epidermis, was observed in 12 biopsy specimens. By the time necrolytic migratory erythema was diagnosed, the pancreatic carcinoma had metastasized to the liver, regional lymph nodes, or bone in 12 patients.


CONCLUSIONS
Increased awareness of the polymorphic mucocutaneous and nonspecific histopathologic features of glucagonoma syndrome is needed to avoid unnecessary delay in the diagnosis of this syndrome.
Pujol
Ramon M
RM
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Wang
Chin-Yao Eric
CY
el-Azhary
Rokea A
RA
Su
W P Daniel
WP
Gibson
Lawrence E
LE
Schroeter
Arnold L
AL
D000328:Adult; D000368:Aged; D004890:Erythema / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis