Incontinentia pigmenti with secondary Raynaud's phenomenon: A case report and review of the literature
نویسندگان
چکیده
Purpose To describe a patient with incontinentia pigmenti (IP) and Raynaud's phenomenon (RP). Observations A 5 year-old girl with history of IP was noted to have RP. Visual acuity was unaffected in both eyes, and fundus examination demonstrated regressed peripheral neovascularization. Photos of the patient's hands demonstrated pale discoloration associated with exposure to cold. Conclusions and importance IP, known to affect small cerebral and retinal blood vessels, can also affect the small blood vessels in the extremities, resulting in secondary RP.
منابع مشابه
Incontinentia Pigmenti
Incontinentia pigmenti (IP) is an uncommon X-linked dominant genodermatosis characterized by four cutaneous stages and frequent association with dental (90%), central nervous system (33%) and ocular (35%) anomalies. The exact pathogenesis of this disorder remains unknown.Herein, we report a newborn girl with inflammatory vesiculobullous and warty skin lesions and a positive family history of IP...
متن کاملA Case Report of Incontinentia Pigmenti in a Newborn with Positive Family History Extending Over Three Generations
Background: Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis that presents at the time of birth or soon after birth with cutaneous manifestation. This disorder may also affect the ectodermal tissues, such as the central nervous system, skeletal system, eyes, hair, nails, and teeth. The dermatological findings occur in four successi...
متن کاملA Case Study of Hypomelanosis of Ito
Among nevoid causes of hypopigmentations; incontinentia pigmenti achromians (Hypomelanosis of Ito) is a neurocutaneous syndromes characterized by distinctive macular, linear or irregular whorls or swirls of Hypopigmentations. Unrelated to incontinentia pigmenti, the loss of pigment begins spontaneously during infancy or early childhood and is of Particular importance because of the falt that m...
متن کاملSecondary or Symptomatic Hemifacial Spasm caused by a Tumor of Cerebellopontine Angle Case Report and Review of the Literature
Background & Importance: Primary hemifacial spasm is usually caused by microvascular compression of the facial nerve at its root exit zone at brainstem without any space-occupying pathology. Secondary or so-called symptomatic hemifacial spasm has an additional underlying pathology e.g. tumors leading to the microvascular compression. We review and discuss the pathophysiology and the literature ...
متن کاملIncontinentia pigmenti: a newborn with characteristic skin lesions and bilateral optic atrophy: case report and review of literature.
Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder with skin, eye, central nervous system (CNS) and tooth abnormalities. According to the reported cases, it is estimated that there have been nearly 900-1200 affected individuals. In this article, the literature is reviewed and a case of IP with characteristic skin lesions and optic atrophy is presented.
متن کامل