Paraneoplastic Scleroderma (Sclerodermiform Paraneoplastic Syndrome)

Cancers reported to be associated with paraneoplastic scleroderma:

(1) colorectal cancer

(2) ovarian cancer

(3) renal cancer

(4) neuroendocrine carcinoma (carcinoid, small cell lung carcinoma)

(5) plasma cell disorders (POEMS, myeloma, etc)

(6) malignant melanoma

(7) breast cancer

(8) hairy cell leukemia

(9) malignant lymphoma

(10) prostate cancer

(11) uterine cancer

Features of paraneoplastic scleroderma:

(1) The onset of scleroderma is temporally related to the onset of cancer. It may precede the onset of cancer by 1 or 2 years.

(2) The severity of the disease tends to be worse when the cancer is untreated or progressing and better following remission.

(3) There are atypical features for scleroderma - older age at onset, unusual antibodies, more rapid progression, etc.

(4) Other causes of a scleroderma-like reaction are unlikely to explain the findings.

Antibodies found in paraneoplastic scleroderma may include:

(1) RNA polymerase I or RNA polymerase III

(2) Jo-1

(3) anti-nuclear antibodies (ANA)

(4) other (see 22.22.24)

Differential diagnosis:

(1) chemotherapy-induced scleroderma-like reaction

(2) cancer arising in a patient with scleroderma