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

Paraneoplastic Pemphigus and Pityriasis Rotunda

Paraneoplastic Pemphigus (PNP) was described in 1990.[27] It is an acantholytic mucocutaneous syndrome characterized by painful mucosal erosions, ulcerations, and polymorphous skin lesions that progress to blistering eruptions on the trunk and extremities.[27] Histologic findings are vacuolar interface changes, keratinocyte necrosis, and intraepidermal acantholysis. Malignancies associated with PNP are lymphoproliferative disorders such as non-Hodgkin B-cell lymphoma, chronic lymphocytic leukemia, Waldenström's macroglobulinemia, Hodgkin disease, T-cell lymphoma, Castleman's tumor, and thymoma.[28,29] PNP is associated with a high mortality rate (75%-80%).[30]

Pityriasis rotunda is an unusual skin condition characterized by round, scaly, hyperpigmented lesions on the trunk and proximal extremities. The hands, feet, and face are usually spared. It is more commonly seen in deeply pigmented South African and West Indian patients. Histologically, it is similar to ichthyosis vulgaris. Pityriasis rotunda is associated with internal malignancies in 6% of cases, with gastric carcinoma and hepatocarcinomas being the most frequent neoplasms.[31,32]