Its clinical features are polymorphic mucocutaneous manifestations, encompassing multiple annular erythematous scaling and crusting patches with hyperpigmentation. These lesions can affect the entire body with a predilection for the perineum, buttocks, groin, abdomen, and limbs — in other words, areas that are subject to increased pressure and friction. The only two reported oral findings are angular cheilitis and atrophic glossitis.
Bullous, erosive oral mucositis with areas having lichenoid features are visible on the tongue and the upper and lower lips (Panel A) and inside the right cheek (Panel B). The patient's entire body, including the back, is covered with purplish, confluent erythematopapulous scaling and crusting lesions with a keratotic surface (Panel C). Histologic analysis of the skin (Panel D, left) shows hyperparakeratosis and spongiosis, accompanied by the presence of necrotic keratinocytes, and vascular proliferation. Histologic analysis of the oral mucosa (Panel D, right) shows a prominent neutrophilic, eosinophilic, and plasmacellular infiltrate (hematoxylin and eosin).
The patient's nutritional status was normal. Routine hematologic tests revealed mild hyperglycemia, hypoalbuminemia, a glucagon level of 73 pg per milliliter (normal range, 25 to 250), and a zinc level of 134 μg per deciliter (normal range, 60 to 250). Tumor markers showed a CA-125 level of 232 U per milliliter (normal range, 0 to 35) and a β2-microglobulin level of 2924 ng per milliliter (normal range, 600 to 2600). Total-body computed tomography revealed the presence of an ovoid mass measuring 40 mm in diameter in the upper lobe of the right lung, with prominent striae hooking the apical and costal homolateral pleura. No evidence of a pancreatic tumor was detected. Needle biopsy of the lung with ultrasonographic guidance revealed a small-cell lung cancer. A diagnosis of nonglucagonoma-associated necrolytic migratory erythema was made. The patient died 2 months later.