Cutaneous Signs and Syndromes Associated With Internal Malignancies

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

Disclosures

Skinmed. 2005;4(2):84-92. 

In This Article

Basal Cell Nevus Syndrome and Bazex's Syndrome (Acrokeratosis Paraneoplastica)

Basal Cell Nevus Syndrome (BCNS) is a rare autosomal dominant disorder characterized by multiple basal cell carcinomas of the skin, odontogenic keratocysts of the jaws, and a variety of skeletal anomalies. Other manifestations are palmoplantar pits, typical facies (broad nasal root, frontal bossing, and hypertelorism), epidermal cysts, intracranial calcifications, developmental malformations, and the predisposition to other benign and malignant tumors.[7]

Patients with BCNS have an elevated risk of developing medulloblastoma at young ages with approximately 5% developing this tumor in the first years of life.[8] BCNS has also been associated with meningiomas[9]and in anecdotic case reports with ovarian cancer,[10]salivary gland carcinoma,[11] non-Hodgkin's lymphoma,[12] and nasopharyngeal rhabdomyosarcoma.[13]

Bazex's syndrome is characterized by violaceous erythema and scaling of the hands, feet, nose, aural helices, and scalp. In advanced stages, the elbows, knees, and cheeks may be involved with nail dystrophy common.

Bazex's syndrome is always associated with an underlying neoplasm. The most commonly associated malignancies are squamous cell carcinomas of the head and neck.[14] Bazex's syndrome has also been associated with GI tract and lung cancer. In 63% of cases, skin lesions precede diagnosis of the tumor by approximately 1 year.[14] Paraneoplastic acrokeratosis generally responds to successful treatment of the underlying tumor and fails to improve when the neoplasm persists.

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