Keratosis Pilaris Rubra Faceii
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Keratosis pilaris (KP; also follicular keratosis, lichen pilaris, or colloquially chicken skin) is a common,
autosomal An autosome is any chromosome that is not a sex chromosome. The members of an autosome pair in a diploid cell have the same morphology, unlike those in allosome, allosomal (sex chromosome) pairs, which may have different structures. The DNA in au ...
- dominant, genetic condition of the skin's
hair follicle The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction between h ...
s characterized by the appearance of possibly
itch Itch (also known as pruritus) is a sensation that causes the desire or reflex to scratch. Itch has resisted many attempts to be classified as any one type of sensory experience. Itch has many similarities to pain, and while both are unpleasant ...
y, small, gooseflesh-like bumps, with varying degrees of reddening or inflammation. It most often appears on the outer sides of the upper arms (the forearms can also be affected),
thigh In human anatomy, the thigh is the area between the hip (pelvis) and the knee. Anatomically, it is part of the lower limb. The single bone in the thigh is called the femur. This bone is very thick and strong (due to the high proportion of bone ...
s, face, back, and
buttock The buttocks (singular: buttock) are two rounded portions of the exterior anatomy of most mammals, located on the posterior of the pelvic region. In humans, the buttocks are located between the lower back and the perineum. They are composed ...
s; KP can also occur on the
hands A hand is a prehensile, multi-fingered appendage located at the end of the forearm or forelimb of primates such as humans, chimpanzees, monkeys, and lemurs. A few other vertebrates such as the koala (which has two opposable thumbs on each " ...
, and tops of
leg A leg is a weight-bearing and animal locomotion, locomotive anatomical structure, usually having a columnar shape. During locomotion, legs function as "extensible struts". The combination of movements at all joints can be modeled as a single ...
s, sides, or any body part except glabrous (hairless) skin (like the palms or soles of feet). Often the lesions can appear on the
face The face is the front of an animal's head that features the eyes, nose and mouth, and through which animals express many of their emotions. The face is crucial for human identity, and damage such as scarring or developmental deformities may aff ...
, which may be mistaken for
acne Acne, also known as ''acne vulgaris'', is a long-term Cutaneous condition, skin condition that occurs when Keratinocyte, dead skin cells and Sebum, oil from the skin clog hair follicles. Typical features of the condition include comedo, black ...
or
folliculitis Folliculitis is the infection and inflammation of one or more hair follicles. The condition may occur anywhere on hair-covered skin. The rash may appear as pimples that come to white tips on the face, chest, back, arms, legs, buttocks, or head. A ...
. The several types of KP have been associated with
pregnancy Pregnancy is the time during which one or more offspring develops ( gestates) inside a woman's uterus (womb). A multiple pregnancy involves more than one offspring, such as with twins. Pregnancy usually occurs by sexual intercourse, but ca ...
,
type 1 diabetes mellitus Type 1 diabetes (T1D), formerly known as juvenile diabetes, is an autoimmune disease that originates when cells that make insulin (beta cells) are destroyed by the immune system. Insulin is a hormone required for the cells to use blood sugar for ...
,
obesity Obesity is a medical condition, sometimes considered a disease, in which excess body fat has accumulated to such an extent that it may negatively affect health. People are classified as obese when their body mass index (BMI)—a person's we ...
,
dry skin Xeroderma, xerosis or xerosis cutis, or simply dry skin, is a skin condition characterized by excessively dry skin. The medical term ''xeroderma'', meaning "dry skin", derives from modern Latin, ''xero-'' 'dry' + Greek ''derma'' 'skin'. In most ...
, allergic diseases (e.g.,
atopic dermatitis Atopic dermatitis (AD), also known as atopic eczema, is a long-term type of inflammation of the skin (dermatitis). It results in puritis, itchy, red, swollen, and cracked skin. Clear fluid may come from the affected areas, which often thickens o ...
), and rarely
cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
. Many rarer types of the disorder are part of inherited genetic syndromes. The cause of KP is not completely understood. As of 2018, KP is thought to be due to abnormalities in the process of depositing the protein
keratin Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. Alpha-keratin (α-keratin) is a type of keratin found in vertebrates. It is the key structural material making up scales, hair, nails, feathers, ho ...
in hair follicles, abnormalities in the hair shaft, or both. KP is usually diagnosed by a medical professional based on the appearance of the skin, but
dermoscopy Dermatoscopy also known as dermoscopy or epiluminescence microscopy, is the examination of skin lesions with a dermatoscope. It is a tool similar to a camera to allow for inspection of skin lesions unobstructed by skin surface reflections. The de ...
can be used, as well, if the diagnosis is unclear. Variants of the ''
ABCA12 ATP-binding cassette sub-family A member 12 also known as ATP-binding cassette transporter 12 is a protein that in humans is encoded by the ''ABCA12'' gene. ABCA12 belongs to a group of genes called the ATP-binding cassette family, which makes p ...
''
gene In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...
have been associated with KP. KP is the most common disorder of the hair follicle in children. How common KP is in adults is unclear, with estimates ranging from 0.75 to 34% of the population. No single approach has been found to completely cure KP, but treatments can improve the cosmetic appearance of the condition. Treatment includes the application of topical preparations of
moisturizers A moisturizer, or emollient, is a cosmetic preparation used for protecting, moisturizing, and lubricating the skin. These functions are normally performed by sebum produced by healthy skin. The word "emollient" is derived from the Latin verb ''mo ...
and medications such as
glycolic acid Glycolic acid (or hydroxyacetic acid; chemical formula HOCH2CO2H) is a colorless, odorless and hygroscopic crystalline solid, highly soluble in water. It is used in various skin-care products. Glycolic acid is widespread in nature. A glycolate (so ...
,
lactic acid Lactic acid is an organic acid. It has a molecular formula . It is white in the solid state and it is miscible with water. When in the dissolved state, it forms a colorless solution. Production includes both artificial synthesis as well as natu ...
,
salicylic acid Salicylic acid is an organic compound with the formula HOC6H4CO2H. A colorless, bitter-tasting solid, it is a precursor to and a metabolite of aspirin (acetylsalicylic acid). It is a plant hormone, and has been listed by the EPA Toxic Substance ...
,
urea Urea, also known as carbamide, is an organic compound with chemical formula . This amide has two amino groups (–) joined by a carbonyl functional group (–C(=O)–). It is thus the simplest amide of carbamic acid. Urea serves an important r ...
, or
retinoid The retinoids are a class of chemical compounds that are vitamers of vitamin A or are chemically related to it. Retinoids have found use in medicine where they regulate epithelial cell growth. Retinoids have many important functions throughout t ...
s to the skin. Fractional
carbon dioxide laser The carbon-dioxide laser (CO2 laser) was one of the earliest gas lasers to be developed. It was invented by Kumar Patel of Bell Labs in 1964 and is still one of the most useful types of laser. Carbon-dioxide lasers are the highest-power continuou ...
s and Nd:YAG laser therapies are also effective.


Signs and symptoms

KP results in small, rough bumps on the surface of the skin. They are skin-colored bumps the size of a grain of sand, many of which are surrounded by a slight pink color in light-skinned people and dark spots in dark-skinned people. Most people with KP do not have symptoms, but the bumps in the skin can occasionally be itchy. Irritation due to scratching KP bumps can result in redness and
inflammation Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...
. Though people with KP experience the condition year-round, the problem can become exacerbated, with the bumps likely to look and feel more pronounced in color and texture, during the colder months, when moisture levels in the air are lower. The symptoms may also worsen during pregnancy or after childbirth. Increased sun exposure might mitigate the symptoms of KP.


Pathophysiology

KP occurs when the human body produces excess amounts of the skin protein keratin, resulting in the formation of small, raised bumps in the skin, often with surrounding redness. The excess keratin, which is the same color as the person's natural skin tone, surrounds and entraps the
hair follicles The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction between h ...
in the pore. This causes the formation of hard plugs (a process known as
hyperkeratinization Hyperkeratinization (American English or hyperkeratinisation in British) is a disorder of the cells lining the inside of a hair follicle. It is the normal function of these cells to detach or slough off ( desquamate) from the skin lining at normal ...
). Many KP bumps contain an
ingrown hair Ingrown hair is a condition where a hair curls back or grows sideways into the skin. The condition is most prevalent among people who have coarse or curly hair. It may or may not be accompanied by an infection of the hair follicle (folliculitis) o ...
that has coiled. This is a result of the keratinized skin's "capping off" the hair follicle, preventing the hair from exiting. The hair grows encapsulated inside the follicle. KP is more common in patients affected by
atopic Atopy is the tendency to produce an exaggerated immunoglobulin E (IgE) immune response to otherwise harmless substances in the environment. Allergic diseases are clinical manifestations of such inappropriate, atopic responses. Atopy may have a ...
diseases such as
allergic rhinitis Allergic rhinitis, of which the seasonal type is called hay fever, is a type of inflammation in the nose that occurs when the immune system overreacts to allergens in the air. Signs and symptoms include a runny or stuffy nose, sneezing, red, i ...
and
atopic dermatitis Atopic dermatitis (AD), also known as atopic eczema, is a long-term type of inflammation of the skin (dermatitis). It results in puritis, itchy, red, swollen, and cracked skin. Clear fluid may come from the affected areas, which often thickens o ...
. KP subtypes are occasionally part of genetically inherited syndromes associated with
intellectual disability Intellectual disability (ID), also known as general learning disability in the United Kingdom and formerly mental retardation,Rosa's Law, Pub. L. 111-256124 Stat. 2643(2010). is a generalized neurodevelopmental disorder characterized by signifi ...
,
neuro-cardio-facial-cutaneous syndromes Neuro-cardio-facial-cutaneous-syndromes (NCFC), (also referred to as ''neuro-craniofacial-cardiac syndromes'') is a group of developmental disorders with a genetic ground, affecting the nervous system, circulatory system, (cranio)facial and cutaneou ...
, RASopathies,
ectodermal dysplasia Ectodermal dysplasia (ED) is a group of genetic syndromes all deriving from abnormalities of the ectodermal structures.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Sau ...
s, and certain
myopathies In medicine, myopathy is a disease of the muscle in which the muscle fibers do not function properly. This results in muscular weakness. ''Myopathy'' means muscle disease (Greek : myo- ''muscle'' + patheia '' -pathy'' : ''suffering''). This meani ...
.


Diagnosis

Physicians can often diagnose KP simply by examining the skin without specialized tests, but a
dermatologist Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medical ...
can use
dermoscopy Dermatoscopy also known as dermoscopy or epiluminescence microscopy, is the examination of skin lesions with a dermatoscope. It is a tool similar to a camera to allow for inspection of skin lesions unobstructed by skin surface reflections. The de ...
to confirm the diagnosis and assess if a person with KP is responding to treatment. Physicians often consider family history and the presence of symptoms when making the diagnosis.Mayo Clinic Staff. "Tests and Diagnosis". ''Mayo Clinic. ''Retrieved 2013-10-31 Those with this condition are generally encouraged to contact a physician if the bumps are bothersome and do not improve with over-the-counter lotions.


Differential diagnosis

Several medications that can cause a skin eruption similar to KP include
cyclosporine Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is a natural product. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease ...
,
BRAF inhibitor BRAF is a human gene that encodes a protein called B-Raf. The gene is also referred to as proto-oncogene B-Raf and v-Raf murine sarcoma viral oncogene homolog B, while the protein is more formally known as serine/threonine-protein kinase B-Raf. T ...
s, and
tyrosine kinase inhibitor A tyrosine kinase inhibitor (TKI) is a pharmaceutical drug that inhibits tyrosine kinases. Tyrosine kinases are enzymes responsible for the activation of many proteins by signal transduction cascades. The proteins are activated by adding a phosph ...
s.


Classification

The several different types of KP include KP rubra (red, inflamed bumps, which can be on arms, head, legs), KP alba (rough, bumpy skin with no irritation), KP rubra faceii (reddish rash on the cheeks), KP atrophicans, keratosis follicularis spinulosa decalvans, atrophoderma vermiculatum, KP atrophicans faciei, erythromelanosis follicularis faciei et colli, and papular profuse precocious KP. KP is commonly described in association with other dry-skin conditions, such as ichthyosis vulgaris, dry skin, and atopic dermatitis, including those of asthma and allergies. KP does not bear any known, long-term health implications, nor is it associated with increased
mortality Mortality is the state of being mortal, or susceptible to death; the opposite of immortality. Mortality may also refer to: * Fish mortality, a parameter used in fisheries population dynamics to account for the loss of fish in a fish stock throug ...
or
morbidity A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that a ...
. and It is not related to
goose bumps Goose bumps, goosebumps or goose-pimples are the bumps on a person's skin at the base of body hairs which may involuntarily develop when a person is tickled, cold or experiencing strong emotions such as fear, euphoria or sexual arousal. The fo ...
, which result from
muscle contractions Muscle contraction is the activation of tension-generating sites within muscle cells. In physiology, muscle contraction does not necessarily mean muscle shortening because muscle tension can be produced without changes in muscle length, such as w ...
, except that both occur in the area where the
hair shaft Hair is a protein filament that grows from follicles found in the dermis. Hair is one of the defining characteristics of mammals. The human body, apart from areas of glabrous skin, is covered in follicles which produce thick terminal and fine ...
exits the skin.


Gallery

File:Keratosis Pilaris on Lower Extremity.jpg, Keratosis pilaris on lower extremity File:KeratosisPilaris.jpg, Keratosis Pilaris on back of upper arm File:Keratosis pilaris arm.jpg, Keratosis pilaris on arm File:Keratosis pilaris skin plugs.jpg, Skin plugs removed from a person with keratosis pilaris


Treatment

KP is medically harmless, but many individuals may seek treatment, as the condition can cause emotional distress. Topical creams and lotions are currently the most commonly used treatment for KP, specifically those consisting of moisturizing or
keratolytic Keratolytic () therapy is a type of medical treatment to remove warts, calluses and other lesions in which the epidermis produces excess skin. In this therapy, acidic topical medicines, such as Whitfield's ointment or Jessner's solution, are ap ...
treatments, including urea, lactic acid, glycolic acid, salicylic acid,
vitamin D Vitamin D is a group of fat-soluble secosteroids responsible for increasing intestinal absorption of calcium, magnesium, and phosphate, and many other biological effects. In humans, the most important compounds in this group are vitamin D3 (c ...
,
fish oil Fish oil is oil derived from the tissues of oily fish. Fish oils contain the omega-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), precursors of certain eicosanoids that are known to reduce inflammation in the body a ...
, or topical retinoids such as
tretinoin Tretinoin, also known as all-''trans'' retinoic acid (ATRA), is a medication used for the treatment of acne and acute promyelocytic leukemia. For acne, it is applied to the skin as a cream, gel or ointment. For leukemia, it is taken by mouth f ...
. Improvement of the skin often takes months, and the bumps are likely to return. Limiting time in the shower and using gentle exfoliation to unplug pores can help. Many products are available that apply abrasive materials, with alpha or beta hydroxy acids to assist with exfoliation. Some cases of KP have been successfully treated with
laser therapy Laser medicine consists in the use of lasers in medical diagnosis, treatments, or therapies, such as laser photodynamic therapy, photorejuvenation, and laser surgery. Lasers Lasers used in medicine include in principle any type of laser, but ...
, which involves passing intense bursts of light into targeted areas of the skin. Depending on the body's response to the treatment, multiple sessions over the course of a few months may be necessary.


Epidemiology

Worldwide, KP affects an estimated 30 to 50% of the adult population, and around 50 to 80% of all
adolescents Adolescence () is a transitional stage of physical and psychological development that generally occurs during the period from puberty to adulthood (typically corresponding to the age of majority). Adolescence is usually associated with the te ...
. It is more common in women than in men, and it is often present in otherwise healthy individuals. The skin condition is prevalent in people of all ethnicities, and no particular ethnicity is at higher risk for developing KP. Although KP may manifest in people of any age, it usually appears within the first decade of life and is more common in young children. In most cases, the condition gradually improves before age 30, but it can persist longer.


See also

*
Ichthyosis linearis circumflexa Ichthyosis linearis circumflexa is a distinctive skin condition of generalized hyperkeratosis and polycyclic and serpiginous erythematous plaques with a characteristic, migratory, double-edged scale at the margins, and is the typical cutaneous man ...


References


External links


The American Academy of DermatologyThe American Osteopathic College of Dermatology
- Article on keratosis pilaris {{Congenital malformations and deformations of integument Genetic diseases and disorders Genodermatoses Dermal and subcutaneous growths