Linear atrophoderma of Moulin | |
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Other names | Moulin atrophoderma linearis, atrophoderma of Moulin |
Specialty | Dermatology |
Linear atrophoderma of Moulin, also known as Moulin atrophoderma linearis, [1] ) is an acquired unilateral dermatitis localized along the Blaschko lines. It affects children or adolescents of both genders, involving the trunk and the limbs. It is, presumably, a rare cutaneous form of mosaicism. [2] This disease was first referred to as atrophoderma of Moulin after Dr. Moulin who first reported it in 1992 [3] then was renamed as linear atrophoderma of Moulin. [4] Only a few dozen cases have been described; for this reason, LAM is considered to be a very rare disorder. [5] [6] [7]
A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment.
Incontinentia pigmenti (IP) is a rare X-linked dominant genetic disorder that affects the skin, hair, teeth, nails and central nervous system. It is named from its appearance under a microscope.
Cold urticaria is a disorder in which large red welts called hives (urticaria) form on the skin after exposure to a cold stimulus. The hives are usually itchy and often the hands, feet and other parts of the body will become itchy and swollen as well. Hives vary in size from about 7 mm in diameter to as big as about 27 mm or larger.
Palmoplantar keratodermas are a heterogeneous group of skin disorders characterized by abnormal thickening (scleroderma) of the stratum corneum of the palms and soles.
Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by reduction in blood flow (ischemia) through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration (cyanosis). This can be a secondary effect of a condition that increases a person's risk of forming blood clots (thrombosis), including a wide array of pathological and nonpathological conditions. Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.
Morphea is a form of scleroderma that mainly involves isolated patches of hardened skin on the face, hands, and feet, or anywhere else on the body, usually with no internal organ involvement. However, in Deep Morphea inflammation and sclerosis can be found in the deep dermis, panniculus, fascia, superficial muscle and bone.
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 skin condition or in chimeras where different cell lines contain different genes. These lines may express different amounts of melanin, or become visible due to a differing susceptibility to disease. In such individuals, they can become apparent as whorls, patches, streaks or lines in a linear or segmental distribution over the skin. They follow a V shape over the back, S-shaped whirls over the chest and sides, and wavy shapes on the head. Not all mosaic skin conditions follow Blaschko's lines.
The Koebner phenomenon or Köbner phenomenon, also called the Koebner response or the isomorphic response, attributed to Heinrich Köbner, is the appearance of skin lesions on lines of trauma. The Koebner phenomenon may result from either a linear exposure or irritation. Conditions demonstrating linear lesions after a linear exposure to a causative agent include: molluscum contagiosum, warts and toxicodendron dermatitis. Warts and molluscum contagiosum lesions can be spread in linear patterns by self-scratching ("auto-inoculation"). Toxicodendron dermatitis lesions are often linear from brushing up against the plant. Causes of the Koebner phenomenon that are secondary to scratching rather than an infective or chemical cause include vitiligo, psoriasis, lichen planus, lichen nitidus, pityriasis rubra pilaris, and keratosis follicularis.
Conradi–Hünermann syndrome is a rare type of chondrodysplasia punctata. It is associated with the EBP gene and affects between one in 100,000 and one in 200,000 babies.
Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaque.
Anetoderma is a benign but uncommon disorder that causes localized areas of flaccid or herniated sac-like skin due to a focal reduction of dermal elastic tissue. Anetoderma is subclassified as primary anetoderma, secondary anetoderma, iatrogenic anetoderma of prematurity, congenital anetoderma, familial anetoderma, and drug-induced anetoderma.
Unilateral nevoid telangiectasia presents with fine thread veins, typically over a segment of skin supplied by a particular nerve on one side of the body. It most frequently involves the trigeminal, C3 and C4, or nearby areas. The condition was named in 1970 by Victor Selmanowitz.
Trichorrhexis invaginata is a distinctive hair shaft abnormality that may occur sporadically, either in normal hair or with other hair shaft abnormalities, or regularly as a marker for Netherton syndrome. The primary defect appears to be abnormal keratinization of the hair shaft in the keratogenous zone, allowing for intussusception of the fully keratinized and hard distal shaft into the incompletely keratinized and soft proximal portion of the shaft.
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:
Acrokeratosis verruciformis is a rare autosomal dominant disorder appearing at birth or in early childhood, characterized by skin lesions that are small, verrucous, flat papules resembling warts along with palmoplantar punctate keratoses and pits. However sporadic forms, whose less than 10 cases have been reported, presents at a later age, usually after the first decade and generally lack palmoplantar keratoses. Whether acrokeratosis verruciformis and Darier disease are related or distinct entities has been controversial, like Darier's disease, it is associated with defects in the ATP2A2 gene. however the specific mutations found in the ATP2A2 gene in acrokeratosis verruciformis have never been found in Darier's disease.
Phakomatosis pigmentokeratotica is a rare neurocutanous condition characterized by the combination of an organoid sebaceous nevus and speckled lentiginous nevus. It is an unusual variant of epidermal naevus syndrome. It was first described by Happle et al. It is often associated with neurological or skeletal anomalies such as hemiatrophy, dysaesthesia and hyperhidrosis in a segmental pattern, mild mental retardation, seizures, deafness, ptosis and strabismus.
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. There is no cure, but different medical treatments can alleviate the symptoms.
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a skin lesion that resembles a comedonal nevus, but it occurs on the palms and soles where pilosebaceous follicles are normally absent. It is probably transmitted by paradominant transmission.
Microphthalmia–dermal aplasia–sclerocornea syndrome is a condition characterized by linear skin lesions. MLS is a rare X-linked dominant male-lethal disease characterized by unilateral or bilateral microphthalmia and linear skin defects in affected females, and in utero lethality for affected males. It can be associated with HCCS, mutations in it cause microphthalmia with Linear Skin Defects Syndrome.
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