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

Pyoderma Gangrenosum and Quincke's Edema (Angioedema)

Pyoderma Gangrenosum (PG) is characterized by lesions that begin with painful pustules and/or erythematous nodules that break down to form an ulcer that exhibits raised violaceous undermined borders and with hemorrhagic exudate partially covered by necrotic tissue. Up to 50% of patients have an associated condition such as inflammatory bowel diseases, diverticulosis, arthritis, chronic hepatitis, Behçet's syndrome, or hematologic neoplasia. The frequency of concomitant malignant disease in patients with PG is uncertain, but it has been estimated at 4.5%-7%.[33,34] Atypical or bullous PG has been related to hematologic malignancies, acute myelogenous leukemia being the most frequently associated malig-nancy.[35] It has also been reported in chronic myeloid leukemia, myeloma (usually IgA type), Waldenström macroglobulinemia, and in Hodgkin and non-Hodgkin lymphomas as well as in solid tumors such as carcinoid, colon, breast, and bladder carcinoma.[33]

Angioedema is characterized by acute onset of nonpruritic, nonpitting, and circumscribed areas of edema secondary to increased vascular permeability. Angioedema is most apparent in distensible tissues, such as lips, eyes, earlobes, and tongue, and also may involve the larynx, extremities, and genitalia. Angioedema can be classified as hereditary angioedema, angioedema due to acquired deficiency of the inhibitor of the first component of human complement (C1-INH), angioedema associated with allergic reactions, angioedema secondary to drugs, and idiopathic angioedema.[36]

Angioedema due to acquired deficiency of C1-INH is a rare condition. It is characterized by increased consumption of C1-INH that leads to an enhanced complement system cascade reaction resulting in increased vascular permeability and edema. C1-INH deficiency angioedema is suspected in patients with a history of recurrent angioedema without urticaria, in or after their fourth decade of life, with a negative family history of angioedema, and with C1-INH functional levels below 50% of normal.[37] This type of angioedema has been associated with chronic lymphocytic leukemia, lymphosarcoma, non-Hodgkin lymphoma, lymphocytic lymphoma, myeloma, and Waldenström macroglobulinemia. The risk for lymphatic malignancy is 35% and for other malignancies 8%, based on a study of 128 patients.[37]