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Inflammatory Linear Verrucous Epidermal Nevus is a rare disease of the skin that presents as multiple, discrete, red papules that tend to coalesce into linear plaques that follow the Lines of Blaschko. The plaques can be slightly warty (psoriaform) or scaly (eczema-like). ILVEN is caused by somatic mutations that result in genetic mosaicism.
Schimmelpenning syndrome. Schimmelpenning syndrome is a neurocutaneous condition characterized by one or more sebaceous nevi, usually appearing on the face or scalp, [1] associated with anomalies of the central nervous system, ocular system, skeletal system, cardiovascular system and genitourinary system. [2]
Blaschko's lines. Blaschko's lines, also called the lines of Blaschko, are lines of normal cell development in the skin. These lines are only visible in those with a mosaic [1][2][3] skin condition or in chimeras where different cell lines contain different genes. These lines may express different amounts of melanin, [4] or become visible due ...
Epidermal nevus syndrome, also known as Feuerstein and Mims syndrome, [1][2] and Solomon's syndrome[1]: 775 [3] is a rare disease that was first described in 1968 and consists of extensive epidermal nevi with abnormalities of the central nervous system (CNS), skeleton, skin, cardiovascular system, genitourinary system and eyes. [2]: 634 However ...
The hypothesized pathomechanism of the entity appears to be genetic mosaicism and a possible eccrine or restricted epidermal aberration of keratinization. [7] Recent research suggests that PEODDN is a mosaic variant of keratitis ichthyosis deafness (KID) syndrome caused by a somatic mutation in GJB2 , which encodes a gap junction protein called ...
Linear verrucous epidermal nevus is a skin lesion characterized by a verrucous skin-colored, dirty-gray or brown papule. [ 2]: 771 [ 3]: 633 Generally, multiple papules present simultaneously, and coalesce to form a serpiginous plaque. [ 3]: 633 When this nevus covers a diffuse or extensive portion of the body's surface area, it may be referred ...
Phakomatosis pigmentokeratotica is a rare neurocutanous condition characterized by the combination of an organoid sebaceous nevus and speckled lentiginous nevus. [1]: 634–5 [2]: 776 It is an unusual variant of epidermal naevus syndrome. [3] It was first described by Happle et al. [4] It is often associated with neurological or skeletal ...
Pigmented hairy epidermal nevus syndrome. Other names. Becker's naevus syndrome. Specialty. Dermatology. Pigmented hairy epidermal nevus syndrome, also known as Becker's naevus syndrome, [1] is a cutaneous condition characterized by a Becker nevus, ipsilateral hypoplasia of the breast, and skeletal defects such as scoliosis. [2] : 635 [3] : 776.