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Multiple cutaneous leiomyomas of pilar origin have long been recognized to have an autosomal dominant inheritance. While the skin tumors are relatively uncommon and benign, women of affected families often develop uterine fibroids with associated infertility, pain and bleeding. In addition, a subset of these families harbors a predisposition to papillary renal cell carcinoma. Germline mutations in a recently identified classical tumor suppressor gene encoding fumarate hydratase are observed in these individuals. Appropriate screening measures for associated disorders are mandatory.

BACKGROUND. The advancement flap involves the linear advancement of tissue in one direction. Despite its straightforwardness and simple concept, it can be used to close a variety of defects, ranging from small defects on the scalp or extremities to large, complicated defects involving cosmetic units on the face.

Syringomas are benign tumors of the eccrine sweat gland that are most common to the periorbital location. Less common and under-recognized is their potential involvement of the vulvar skin, where they may cause significant pruritus. In this location, lesions often develop just prior to or during puberty, with symptoms of pruritus sometimes correlating with pregnancy or menstruation. A hormonal influence has thus been postulated. Reported treatments for symptomatic patients include topical corticosteroids, systemic and topical antihistamines, laser and excisional surgery, cryotherapy, and topical atropine, with variable results. We report a 9-year-old girl who had severe pruritus secondary to syringomas of the vaginal labia, and review the literature on this unusual disorder. Awareness of this condition is important in order that patients receive appropriate evaluation and management of their symptoms.

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