The main symptom of hypertrichosis is hair growth that is excessive. Heterotypic hypertrichosis is an increased amount of hair in unusual places, such as the small of the back. Hair may be lanugo, vellus, or terminal hair, and it can appear in small patches or over the entire body. In cases of congenital ambras syndrome patients can suffer from hypertrichosis lanuginosa, generalized hypertrichosis, terminal hypertrichosis, circumscribed hypertrichosis, localized hypertrichosis or nevoid hypertrichosis. Photo courtesy of brooke army medical teaching file. As discussed elsewhere in this article, git poly posis syndromes, ulcerative colitis, and dermatitis herpetiformis are associated with an. Thefaster thannormal hair growth was reflected by the finding of90% of. All forms of ambras syndrome are associated with excessive hair growth. This form of hypertrichosis was described by barth et al. It is characterized by typical pattern of hair distribution, dysmorphic facial features and a. Fasting growth of lanugo hair after birth, usually appears around the face. Excessive hair growth at inappropriate locations, such as on the extremities, the head, and the back. The term hirsutism is usually referred to patients, mainly women, who show excessive hair growth with male pattern distribution.
The appearance of hair overgrowth all over the body that has not been there before must not be overlooked. Aug 28, 2018 however, the xlinked syndrome of hypertrichosis associated with gingival hyperplasia is often confused with congenital hypertrichosis lanuginosa. Alice elizabeth doherty was the only known person born in the united states with the exceedingly rare condition hypertrichosis lanuginosa or dogfaced hypertrichosis. This is a form of hypertrichosis in which the body is covered with fine hair except for hands and feet. We report three cases occurring after administration of cytotoxic chemotherapy for cancer. Acquired hypertrichosis lanuginosa is a rare disorder of which only 55 cases have been reported. Congenital generalized hypertrichosis, gingival hyperplasia, a. Congenital hypertrichosis lanuginosa, here showing hypertrichosis over back and upper extremities. Heterogeneous hypertrichosis occurs in girls and women.
Disorders of hair in infants and children other than alopecia clinics. Hypertrichosis is an unusual pathological condition in which there is excessive growth of hairs in areas where usually there is no hair growth. Apr 26, 2017 hypertrichosis, also known as werewolf syndrome, is a condition characterized by excessive hair growth anywhere on a persons body. Congenital forms are present at birth, but excessive hair growth, thickness andor pigmentation may not be noticeable until. It is characterized by excessive growth of fine, downy, hypopigmented lanugolike hair on the face, trunk and limbs. Hypertrichinosis definition of hypertrichinosis by the. In this case of hypertrichosis, the face area is commonly affected including the upper lip, chin and the cheeks. This concept does not include hirsutism which is an androgendependent excess hair growth in. Judge mr, khaw pt, rice ns, christopher a, holmstrom g, harper ji. Get a printable copy pdf file of the complete article 565k, or click on a page image below to browse page by page. In congenital hypertrichosis lanuginosa, most of the body is covered with fine, blond or nonpigmented hair at birth. A thick dark vellus hair appears on your upper extremities.
Hypertrichosis is classified according to age of onset. It may be a congenital, autosomal dominant disorder in which there is excessive hair distributed over the entire body throughout life, usually in association with other congenital anomalies. We report a series of children with hypertrichosis. Dec 10, 2019 symptoms of ambras syndrome are dependent on the specific type of hypertrichosis the patient suffers from. Heterochronous hypertrichosis is the premature appearance of hair as a secondary sex characteristic the appearance of a beard, moustache, or pubic hair in children. Hypertrichosis can be partial, local, or distributed over the whole body. These hairs may reach a length of 10 cm and blend with the darker terminal. This may be a sign of having a malignancy in the present or in the future. Request pdf inherited hypertrichoses hypertrichosis is a rare condition. There are several types of congenital werewolfsyndrome, such as hypertrichosis lanuginosa, generalized hypertrichosis,terminal hypertrichosis, localized hypertrichosis, nevoid hypertrichosis,circumscribed hypertrichosis and several others. It is characterized by excessive lanugo hair present at birth covering the entire body surface except the mucosae, palms, and soles. Omim entry % 145700 hypertrichosis lanuginosa congenita. Mar 25, 2019 the first case involved a father, two daughters, a son and a grandchild. Indian j dermatol venereol leprol serial online 2010 cited 2020 may 11.
Hypertrichosis lanuginosa acquisita following cytotoxic. She was shaved infrequently until aged 9 months, when spontaneous loss of. Hypertrichosis, excessive, abnormal hairiness that may be localized or cover the entire body. Acquired generalized hypertrichosis could be associated. Hypertrichosis is defined as an excessive growth in body hair beyond the normal variation compared with individuals of the same age, race and sex and affecting areas not predominantly androgendependent. Localised hypertrichosis is commonly seen over a naevus or spina bifida. Ambras syndrome, a form of congenital hypertrichosis lanuginosa, is extremely rare in neonates. The appearance of hair overgrowth all over the body that has. Acquired forms of the condition include hypertrichosis lanuginosa, generalized hypertrichosis, patterned hypertrichosis and localized hypertrichosis. Hypertrichosis the excessive growth of hair on a persons body where only downy hairs usually grow. Know the classification, causes, symptoms, treatment of hypertrichosis.
Oct 23, 2014 hypertrichosis lanuginosa congenita is a congenital present from birth skin disease characterized by excessive lanugo very fine, soft, unpigmented hair covering the entire body, with the exception of the palms, soles, and mucous membranes. Congenital hypertrichosis lanuginosa is a very rare hypertrichotic disorder with an. These hairs may reach up to 10 centimeters and often blend with the darker, terminal hairs of the scalp andor eyebrows. This hair growth, also known as malignant down, is often confined to the face with long fine silky hair noticeable on the nose and eyelids, sites that are normally hairless. Heterotypic hypertrichosis is an increased amount of hair in unusual places, such as the small of the. Acquired hypertrichosis lanuginosa ahl is a rare cutaneous paraneoplastic disease characterized by the presence of excessive lanugotype hair on the glabrous skin of face, neck, trunk and limbs that can be associated with additional clinical features such as burning glossitis, papillary hypertrophy of the tongue, diarrhea, dysgeusia, andor weight loss.
Acquired hypertrichosis lanuginosa is a rare cutaneous paraneoplastic disease characterized by the presence of excessive lanugotype hair on the glabrous. Warewolf syndrome thick dark hair appears all over your body. It is possible to blend the galvanic and alternating currents from a single. Children with generalized hypertrichosis, known as hypertrichosis lanuginosa congenita. Only 50 cases of congenital hypertrichosis lanuginosa have been recorded since the middle ages, and fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media. Congenital hypertrichosis lanuginosa chl is a rare inherited disorder in which an excess of lanugo hair remains over the entire body after birth, sparing only the palms, soles, and mucous membranes fig 5.
It can affect both women and men, but its extremely rare. Limited hypertrichosis can be either hereditary or elicited by prolonged mechanical, chemical, thermal, or physical trauma. Excessive hair growth within or on the ear is known medically as auricular hypertrichosis. Primary generalized and localized hypertrichosis in children hair. It is characterized by excessive growth of fine, downy, hypopigmented lanugolike. When the babies are born with abnormal hair growth,it is referred to as congenital werewolf syndrome. Aug 28, 2018 for hundreds of years, societies have maintained a certain fascination with the bizarre and the unknown. Congenital hypertrichosis is associated with three different mutations, one on the 8q chromosome a dominant autosomal mutation ie a dominant trait not located on the sex chromosome this is a cutaneous disorder called congenital hypertrichosis lanuginosa, a dominant trait on the x chromosome at xq24q27. Hypertrichosis lanugosa definition of hypertrichosis. The only biopsy report of the hairy site in hypertrichosis cubiti3 showed no structural abnormalities ofthe hairfollicles. Acquired hypertrichosis and hirsutism are more common. In both congenital hypertrichosis lanuginosa and ambras syndrome, excessive hair is apparent at birth. Hypertrichosis symptoms, causes, treatment, pictures.
Hypertrichosis lanuginosa acquisita associated with. Congenital hypertrichosis lanuginosa in a father and son. As a result, the density, length, and extent of involvement may decrease. Hypertrichosis lanuginosa acquisita hla refers to abnormal and excessive growth of fine downy hair. Hypertrichosis universalis congenita how is hypertrichosis. Congenital hypertrichosis lanuginosa chl is a rare disorder, with fewer than 50 cases reported in the literature. It can be associated with malignancy, most often adenocarcinoma of the lung or colon. Congenital hypertrichosis lanuginosa may be caused by a mutation on the 8q chromosome. Of interest, julia pastrana 18341860, one of the most famous persons with generalized hypertrichosis, was long thought to have congenital hypertrichosis lanuginosa. In the past, persons with congenital disorders that cause excessive bodyhair growth have been so dramatized and romanticized that individuals with rare hypertrichosis syndromes became crowddrawing moneymaking phenomena in many 19th cent. Hypertrichosis universalis genetic and rare diseases. It follows many of the same patterns of congenital hypertrichosis. Hypertrichosis lanuginosa congenita genetic and rare. Auricular hypertrichosis hypertrichosis lanuginosa acquisita, hypertrichosis pinnae auris is a genetic condition expressed as long and strong hairs growing from the helix of the pinna.
Fish analysis using bac clones revealed a cryptic deletion at the breakpoint of chromosome 17. There are other medical names for congenital hypertrichosis including congenital hypertrichosis lanuginosa, congenital hypertrichosis universalis, hypertrichosis universalis and hypertrichosis lanuginosa universalis. Hypertrichosis lanuginosa acquisita is a rare dermatological disorder sometimes accompanied by lingual papillary hypertrophy or eyelash trichomegaly. Acquired generalized hypertrichosis indicating internal malignancy. Hypertrichosis definition of hypertrichosis by medical. The excess hair is commonly referred to as malignant down. Hypertrichosis universalis congenita listed as htc. Hypertrichosis lanuginosa acquired hypertrichosis lanuginosa is characterized by rapid growth of lanugo hair, particularly on the face.
It is considered to be an autosomal dominant disorder, but sporadic presentations are also reported. Patients with congenital hypertrichosis lanuginosa have growth of the lanugo hair, which increases in length and extent of involvement from birth to approximately age 2 years range, 18 y. This causes your body to be fully in terminal hair. Hypertrichosis medical definition, flashcards and icd10 codes. Hypertrichosis lanuginosa article about hypertrichosis. What is hypertrichosis, know its causes, symptom, treatment. Hypertrichosis lanuginosa congenita is a rare congenital skin disease characterized by the presence of 3 to 5cm long lanugotype hair on the entire body, with. Hypertrichosis article about hypertrichosis by the free. Acquired hypertrichosis lanuginosa kumar p, pinto j, kamath. The hair is usually silvery gray to blond in color and grows. Hypertrichosis medigoo health medical tests information. Hypertrichosis lanuginosa congenita is a congenital present from birth skin disease characterized by excessive lanugo very fine, soft, unpigmented hair covering the entire body, with the exception of the palms, soles, and mucous membranes.
Hypertrichosis lanuginosa as a sign of internal malignancy. Hypertrichosis is associated with disorders such as anorexia, repeated skin trauma, systemic illness, metabolic disorders, and exposure to certain drugs and chemicals. Hypertrichosis pictures, causes and treatment 2020. It is important that clinicians watch for, ask about, and completely investigate any new hair growth. Congenital hypertrichosis lanuginosa and congenital glaucoma. Acquired hypertrichosis lanuginosa kumar p, pinto j. Avoidance of anabolic steroids by weight lifters and body builders can prevent acquired hypertrichosis from occurring. Hypertrichosis stature journal of medical genetics. Congenital hypertrichosis this is because of a defect that has been present since they were born or as a result of a congenital defect and is a rare class. This is noticeable at birth and becomes more prominant as you get older. It is caused by genetic or acquired factors, and is an androgenindependent process.
The patient had additional complaints of a burning sensation on the tip of the tongue and the sudden appearance of several seborrheic keratoses on hands and forearms. Vintage real life sideshow photos in frames around the house sidenote. Congenital hypertrichosis lanuginosa chl chl is characterized by the presence of fine, blond hair of lanugo type, distributed all over the body except for the palms, soles and mucous membranes 6, 11. It is caused by a developmental anomaly or a disease of the endocrine glands. In normal individuals, lanugo hairs are often present at birth and tend to gradually disappear over the first months of life. Hypertrichosis is defined as an excessive growth of hair on skin not normally hairy. Karyotype analysis of parents and sibs showed no abnormalities. Hair also appears on the trunk and armpits, while palms and soles are unaffected. Congenital hypertrichosis lanuginosa refers to a condition in which hypertrichosis occurs as a generalized and confluent presentation of hair across the body except in regions where hair is not normally found at all glans penis, labia minora, lips, tips of fingers, palms and soles.
Some men, particularly in the male population of india, have coarse hair growth along the lower portion of the helix, a condition referred to as having hairy pinnae hypertrichosis lanuginosa acquisita. It is so rare that there have only been fifty cases verified. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Acquired hypertrichosis can be prevented by not using certain drugs that cause the condition. A newborn baby presented with overgrowth of lanugo hair and a precocious tooth. Acquired hypertrichosis lanuginosa is a paraneoplastic dermatosis 2. Congenital hypertrichosis lanuginosa gupta lk, khare ak. Cutaneous manifestations of gastrointestinal disorders. Acquired hypertrichosis lanuginosa sometimes occurs in people who at a later stage are diagnosed with a cancer of some form. Acquired hypertrichosis lanuginosa is considered to be a paraneoplastic phenomenon, and in certain instances precedes the diagnosis of. Hypertrichosis is not to be confused with hirsutism.
Hypertrichosis, also known as werewolf syndrome, is a condition characterized by excessive hair growth anywhere on a persons body. The first case involved a father, two daughters, a son and a grandchild. Hypertrichosis lanuginosa acquisita was observed in a female patient with smallcell follicular lymphoma stage ivb. Causes and management of hypertrichosis request pdf. Congenital hypertrichosis lanuginosa clinical presentation. Microscopic examination of head and beard hair samples from her mummy confirmed that the hairs were normal terminal scalp and face hair, i. In a mexican female infant with generalized hypertrichosis, destefano et al. This condition is also called as werewolf syndrome. Hl may also be inherited, more commonly ad than ar. Hypertrichinosis synonyms, hypertrichinosis pronunciation, hypertrichinosis translation, english dictionary definition of hypertrichinosis. It is a rare condition which has been reported in association with both malignancy and certain drug therapy. However, the xlinked syndrome of hypertrichosis associated with gingival hyperplasia is often confused with congenital hypertrichosis lanuginosa. This form of hypertrichosis is associated with a well defined set of anomalies, including redundant skin and enlarged, pink, pebbly gingival nodules.