What is Nevoid hypermelanosis?

Overview. Linear and whorled nevoid hypermelanosis (LWNH) is a rare benign condition affecting skin pigment occurring in newborns or early infancy. The primary symptom is swirling streaks of darkened (hyperpigmented) skin, mainly on the trunk and limbs.

What is Incontinentia Pigmenti disorder?

Incontinentia pigmenti (IP) is a genetic disorder with distinctive skin rashes and lesions seen at birth or within the first few weeks. The majority of children with IP don’t have complications and may be only mildly affected, if at all. But around 20% develop neurological problems that can range from mild to severe.

What is Hypomelanotic?

Hypomelanosis of Ito is a rare condition characterized by distinctive skin changes, in which areas of the body lack skin color (hypopigmentation). These skin changes may present as patches, streaks or spiral-shaped (whorled) areas.

Why is it called Incontinentia Pigmenti?

It is named from its appearance under a microscope. This condition is inherited in an X-linked dominant manner. The disease is characterized by skin abnormalities that begin in childhood, usually a blistering rash which heals, followed by the development of harder skin growths.

What is Beckers nevus?

Becker’s nevus is a non-cancerous, large, brown birthmark occurring mostly in males. It can be present at birth, but is usually first noticed around puberty. It typically occurs on one shoulder and upper trunk but occasionally occurs elsewhere on the body.

What is Hypomelanosis of Ito?

Hypomelanosis of Ito (HMI) is a very rare birth defect that causes unusual patches of light-colored (hypopigmented) skin and may be associated with eye, nervous system, and skeletal problems.

What is Goltz syndrome?

Focal dermal hypoplasia (FDH; MIM #305600), also known as Goltz syndrome or Goltz-Gorlin syndrome, is an X-linked dominant multisystem disorder that is lethal in utero in males [1]. The primary feature of FDH is patchy dermal hypoplasia, with herniation of fat through defects in the dermis.

What is nevus depigmentosus?

Abstract. Background: Nevus depigmentosus is defined as a congenital nonprogressive hypopigmented macule or patch that is stable in its relative size and distribution throughout life. The pathogenesis and histopathologic characteristics of nevus depigmentosus is not yet fully established.

What is nevus Anemicus?

Nevus anemicus is an uncommon, congenital vascular malformation resulting in hypopigmented cutaneous macules and/or patches that characteristically do not become erythematous in response to trauma, heat, or cold. Nevus anemicus typically presents as an isolated lesion with no other systemic manifestations.

What is nevus Comedonicus?

Nevus comedonicus is an uncommon skin abnormality first described in 1895 by Kofmann who used the term “comedo nevus.” It comprises of groups of pits filled with black keratinous plugs resembling blackheads, with inflammatory acne lesions developing later.

Is there a cure for Incontinentia Pigmenti?

IP TREATMENT While there is no known cure for incontinentia pigmenti (IP), there are treatment protocols and recommended medical specialists for affected areas of the body like skin, hair, eyes and more.

What is a Lentigine?

Solar lentigines (len-TIJ-ih-neez) are flat spots of increased pigmentation. They are usually tan, brown or dark brown and darker than freckles. Solar lentigines have oval to round shapes and vary in size. They usually appear on areas most exposed to the sun, such as the scalp, face, hands, arms and upper trunk.

What is Acrofacial vitiligo?

Acrofacial vitiligo is a clinical form of vitiligo characterized by macules in distal digits, periorificial-facial and ano-genital areas.

What is Achromic nevus?

Achromic naevus is an uncommon birthmark (naevus) characterised by a well-defined pale patch. This is usually several centimetres in diameter, with an irregular but well-defined border. Shape and size varies. Often, smaller hypopigmented macules arise around the edges, resembling a splash of paint.

What is Malan syndrome?

What is Malan Syndrome? Malan syndrome is a rare genetic disorder that is the result of a change in the NFIX gene and is characterized by overgrowth, intellectual disability, vision and/or hearing impairment, skeletal anomalies, epilepsy and anxiety.

What is linear and whorled nevoid hypermelanosis?

Linear and whorled nevoid hypermelanosis (LWNHM) is a reticulate pigmentary disorder with a sporadic occurrence, representing genetic mosaicism. It is characterised by hyperpigmented macules in a reticulate pattern along Blaschko’s lines, sparing the mucous membranes and stabilising after one to two years.

Is hypomelanosis hereditary from the mother or father?

Inheritance Inheritance. Usually hypomelanosis of Ito is sporadic. “Sporadic” denotes either a genetic disorder that occurs for the first time in a family due to a new mutation or the chance occurrence of a non-genetic disorder or abnormality that is not likely to recur in a family.

What are the symptoms of hypomelanosis?

Other symptoms may include varying degrees of learning disability, seizures, increased body hair, scoliosis, and strabismus . While the exact cause is not known, hypomelanosis of Ito syndrome is strongly linked to its genetics and many patients have chormosomal abnormalities.

Is hypomelanosis of Ito syndrome inherited?

Many people with Hypomelanosis of Ito syndrome have cells that have the normal chromosomes and some cells with abnormal chromosomes. This is known as chromosomal mosaicism. This condition is not inherited in families. Girls tend to be affected more commonly than boys.