Cutaneous Signs and Syndromes Associated With Internal Malignancies

Claudia C. Ramirez, MD; Brian Berman, MD, PhD


Skinmed. 2005;4(2):84-92. 

In This Article

Extramammary Paget's Disease

Extramammary Paget's Disease (EMPD) usually affects anogenital and axillary skin, both areas in which apocrine glands are present. Women are predominantly affected, but EMPD of the scrotum is not uncommon. Clinically, EMPD is characterized by red, scaly plaques that fail to heal and the disease is often multifocal. Although it is usually painless, it is often pruritic and in advanced stages can become crusted, exudative, and ulcerate. The histopathology is the same as seen in mammary Paget's disease. A literature review of 197 patients with EMPD[21] found that 24% of patients had an underlying cutaneous adnexal adenocarcinoma and 12% had an internal malignancy that was closely related to the location of the skin lesion. Therefore, perianal EMPD may be associated with colorectal adenocarcinoma, whereas genital or groin EMPD may be more often associated with cancers of the uterus, bladder, vagina, and prostate.

Glucagonoma syndrome is characterized by a glucagon-secreting tumor associated with hyperglucagonemia, necrolytic migratory erythema, recent onset of diabetes mellitus, anemia, weight loss, and diarrhea. Necrolytic migratory erythema is widespread, with predilection for the perineum, abdomen, groin, buttocks, and lower extremities. The eruption begins with irregular patches of intense erythema, superficial flaccid vesicles, and bullae that later rupture and produce extensive erosions or exudative crusted plaques. Other mucocutaneous findings are cheilosis, atrophic glossitis, oral mucosal inflammation, and alopecia. Almost all patients with necrolytic migratory erythema have an α-cell tumor of the pancreas and elevated glucagon levels. In one half of patients, there are liver metastases at the time of the diagnosis.[22] With complete resection of the underlying tumor, the cutaneous manifestations rapidly resolve.