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Cutaneous leiomyomatosis

Edward W Cowen, MD, MHSc
Section Editor
Jonathan A Dyer, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Cutaneous leiomyomas, also called piloleiomyomas, are uncommon benign smooth muscle tumors derived from the arrector pili muscle, which is responsible for piloerection of hair follicles. Piloerection, commonly known as "goose bumps," is an involuntary process that may occur upon cold exposure as well as a variety of emotional states (eg, fear, pleasure).

Cutaneous leiomyomas may occur sporadically or in multiple numbers as part of an autosomal dominant cancer syndrome called hereditary leiomyomatosis and renal cell cancer (HLRCC, MIM #150800) [1]. The discovery in 2001 of the association between cutaneous leiomyomas, uterine leiomyomas in women, and an aggressive form of renal cell cancer (RCC) underscores the importance of accurate dermatologic diagnosis of CL so that appropriate cancer screening and counseling of patients and at-risk relatives can be instituted [2].

This topic will discuss the pathogenesis, clinical manifestations, diagnosis, and management of HLRCC. The treatment of renal cell cancer is discussed separately. Other hereditary renal cancer syndromes and uterine leiomyomatosis are also discussed separately.

(See "Overview of the treatment of renal cell carcinoma".)

(See "Hereditary kidney cancer syndromes".)


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Literature review current through: Sep 2016. | This topic last updated: Aug 18, 2015.
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