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Woolly hair nevus (alternatively spelled "Wooly hair nevus") is a congenital condition in which hair in a circumscribed area of the scalp is kinked or woolly.
Congenital forms of hypertrichosis are caused by genetic mutations, and are extremely rare, unlike acquired forms. Congenital hypertrichosis is always present at birth.
- Hypertrichosis lanuginosa
Congenital hypertrichosis lanuginosa is noticeable at birth, with the infant completely covered in thin lanugo hair. In normal circumstances, lanugo hair is shed before birth and replaced by vellus hair; however, in a person with congenital hypertrichosis lanuginosa, the lanugo hair remains after birth. The palms of the hands, soles of the feet, and mucous membranes are not affected. As the person ages, the lanugo hair may thin, leaving only limited areas of hypertrichosis.
- Generalized hypertrichosis
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, whereas women exhibit less severe asymmetrical hair distribution. The palms, soles, and mucous membranes are not affected.
- Terminal hypertrichosis
Congenital terminal hypertrichosis is characterized by the presence of fully pigmented terminal hair that covers the entire body. This condition is usually accompanied by gingival hyperplasia. This form is most responsible for the term "werewolf syndrome" because of the thick, dark hair that appears. People with this condition are sometimes performers at circuses because of their unusual appearance.
- Circumscribed hypertrichosis
Congenital circumscribed hypertrichosis is associated with the presence of thick vellus hair on the upper extremities. Circumscribed signifies this type of hypertrichosis is restricted to certain parts of the body, in this case, the extensor surfaces of the upper extremities. Hairy elbow syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows. This type of hypertrichosis is present at birth, becomes more prominent during aging, and regresses at puberty.
- Localized hypertrichosis
Congenital localized hypertrichosis is a localized increase in hair density and length.
- Nevoid hypertrichosis
Nevoid hypertrichosis may be present at birth or appear later in life. It features an isolated area of excessive terminal hair and is usually not related to any other diseases.
Hair follicle nevus (also known as a "Vellus hamartoma") is a cutaneous condition that presents as a small papule from which fine hairs protrude evenly from the surface.
Typical first symptoms of alopecia areata are small bald patches. The underlying skin is unscarred and looks superficially normal. Although these patches can take many shapes, they are usually round or oval. Alopecia areata most often affects the scalp and beard, but may occur on any part of the body with hair. Different areas of the skin may exhibit hair loss and regrowth at the same time. The disease may also go into remission for a time, or may be permanent. It is common in children.
The area of hair loss may tingle or be painful. The hair tends to fall out over a short period of time, with the loss commonly occurring more on one side of the scalp than the other.
Exclamation point hairs, narrower along the length of the strand closer to the base, producing a characteristic "exclamation point" appearance, are often present.
When healthy hair is pulled out, at most a few should come out, and ripped hair should not be distributed evenly across the tugged portion of the scalp. In cases of alopecia areata, hair will tend to pull out more easily along the edge of the patch where the follicles are already being attacked by the body's immune system than away from the patch where they are still healthy.
Nails may have pitting or trachyonychia.
Important diagnoses to consider include female pattern hair loss (FPHL), chronic telogen effluvium (CTE), and alopecia areata (AA). FPHL is a non-scarring progressive miniaturization of the hair follicle with one of three different characteristic patterns. CTE is an idiopathic disease causing increased hair shedding and bi-temporal recession, usually in middle aged women. AA is an autoimmune attack of hair follicles that usually causes hair to fall out in small round patches.
Acquired hypertrichosis appears after birth. The multiple causes include the side effects of drugs, associations with cancer, and possible links with eating disorders. Acquired forms can usually be reduced with various treatments.
- Hypertrichosis lanuginosa
Acquired hypertrichosis lanuginosa is characterized by rapid growth of lanugo hair, particularly on the face. Hair also appears on the trunk and armpits, while palms and soles are unaffected. The excess hair is commonly referred to as malignant down. This hair is very fine and unpigmented.
- Generalized hypertrichosis
Acquired generalized hypertrichosis commonly affects the cheeks, upper lip, and chin. This form also affects the forearms and legs, but is less common in these areas. Another deformity associated with acquired generalized hypertrichosis is multiple hairs occupying the same follicle. It may also include abnormal hair growth patterns as what happens to the eyelashes in a condition known as trichiasis. Oral minoxidil treatments for hypertension are known to cause this condition. Topical minoxidil used for alopecia causes hair growth in the areas where it is applied, however this hair disappears shortly after discontinuing the use of topical minoxidil.
- Patterned hypertrichosis
Acquired patterned hypertrichosis is an increase in hair growth in a pattern formation. It is similar to acquired generalized hypertrichosis and is a sign of internal malignancy.
- Localized hypertrichosis
Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma. This form is restricted to certain areas of the body.
Symptoms of hair loss include hair loss in patches usually in circular patterns, dandruff, skin lesions, and scarring. Alopecia areata (mild – medium level) usually shows in unusual hair loss areas e.g. eyebrows, backside of the head or above the ears where usually the male pattern baldness does not affect. In male-pattern hair loss, loss and thinning begin at the temples and the crown and either thins out or falls out. Female-pattern hair loss occurs at the frontal and parietal.
People have between 100,000 and 150,000 hairs on their head. The number of strands normally lost in a day varies, but on average is 100. In order to maintain a normal volume, hair must be replaced at the same rate at which it is lost. The first signs of hair thinning that people will often notice are more hairs than usual left in the hairbrush after brushing or in the basin after shampooing. Styling can also reveal areas of thinning, such as a wider parting or a thinning crown.
Perifollicular erythema and scarring white patches are seen on dermoscopy. On scalp biopsy, lymphocytic and granulomatous perifolliculitis with eccentric atrophy of follicular epithelia and perifollicular fibrosis are visualized.
The presentation may be of alopecia (baldness). Individuals vary in severity of symptoms. Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.
Baldness is the partial or complete lack of hair growth, and part of the wider topic of "hair thinning". The degree and pattern of baldness varies, but its most common cause is androgenic hair loss, "alopecia androgenetica", or "alopecia seborrheica", with the last term primarily used in Europe.
Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss.
It can be caused by a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. A variety of distributions are possible. In some cases, hair loss is gradual, without symptoms, and is unnoticed for long periods. In other cases, hair loss is associated with severe itching, burning and pain and is rapidly progressive. The inflammation that destroys the follicle is below the skin surface and there is usually no "scar" seen on the scalp. Affected areas of the scalp may show little signs of inflammation, or have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Scarring hair loss occurs in otherwise healthy men and women of all ages and is seen worldwide.
Nevus comedonicus (also known as a "comedo nevus") is characterized by closely arranged, grouped, often linear, slightly elevated papules that have at their center keratinous plugs resembling comedones.
Telogen effluvium is a scalp disorder characterized by the thinning or shedding of hair resulting from the early entry of hair in the telogen phase (the resting phase of the hair follicle). Emotional or physiological stress may result in an alteration of the normal hair cycle and cause the disorder, with potential causes including eating disorders, fever, childbirth, chronic illness, major surgery, anemia, severe emotional disorders, crash diets, hypothyroidism, and drugs.
Diagnostic tests, which may be performed to verify the diagnosis, include a trichogram, trichoscopy and biopsy. Effluvium can present with similar appearance to alopecia totalis, with further distinction by clinical course, microscopic examination of plucked follicles, or biopsy of the scalp. Histology would show telogen hair follicles in the dermis with minimal inflammation in effluvium, and dense peribulbar lymphocytic infiltrate in alopecia totalis.
Vitamin D levels may also play a role in normal hair cycle.
It is important to continue to watch for symptoms and signs of active disease during and after treatment to ensure that the disease is responding adequately and has not re-activated after therapy has been discontinued. Response to therapy may be indicated by the resolution of scalp symptoms such as itching, pain, tenderness, or burning, by improvement in the signs of scalp inflammation such as decreased redness, scaling or pustules, and by halting or slowing the progression of hair loss. A dermatologist can follow your cicatricial alopecia using these guidelines, and with the pull test. Photographs of the scalp may be useful in monitoring the course of the disease and response to treatment.
Symptoms include rash, itching skin, and hair which remains in spite of shaving. The site of the ingrown hair will form a reddish, raised bump, similar in appearance to a pimple.
Typical features of acne include increased secretion of oily sebum by the skin, microcomedones, comedones, papules, nodules (large papules), pustules, and often results in scarring. The appearance of acne varies with skin color. It may result in psychological and social problems.
Hypermelanotic nevi must be differentiated from other types of pigmented skin lesions, including:
- Lentigo simplex
- Solar lentigo
- Café au lait macule
- Ink-spot lentigo
- Mucosal melanotic macule
- Mongolian spot (dermal melanocytosis)
Favre–Racouchot syndrome occurs in sun-damaged skin and includes open and closed comedones.
Nevus comedonicus or comedo nevus is a benign hamartoma (birthmark) of the pilosebaceous unit around the oil-producing gland in the skin. It has widened open hair follicles with dark keratin plugs that resemble comedones, but they are not actually comedones.
Dowling-Degos disease is a genetic pigment disorder that includes comedo-like lesions and scars.
Familial dyskeratotic comedones is a rare autosomal dominant genetic condition, with keratotic (tough) papules and comedo-like lesions.
Linear verrucous epidermal nevus (also known as a "Linear epidermal nevus," and "Verrucous epidermal nevus") is a skin lesion characterized by a verrucous skin-colored, dirty-gray or brown papule. Generally, multiple papules present simultaneously, and coalesce to form a serpiginous plaque. When this nevus covers a diffuse or extensive portion of the body's surface area, it may be referred to as a systematized epidermal nevus, when it involved only one-half of the body it is called a nevus unius lateris.
The term nevus is included in the names of multiple dermatologic syndromes:
- Basal cell nevus syndrome
- Blue rubber bleb nevus syndrome
- Dysplastic nevus syndrome
- Epidermal nevus syndrome
- Linear nevus sebaceous syndrome
Becker's nevus (also known as "Becker's melanosis", "Becker's pigmentary hamartoma", "nevoid melanosis", and "pigmented hairy epidermal nevus") is a skin disorder predominantly affecting males. The nevus can be present at birth, but more often shows up around puberty. It generally first appears as an irregular pigmentation (melanosis or hyperpigmentation) on the torso or upper arm (though other areas of the body can be affected), and gradually enlarges irregularly, becoming thickened and often hairy (hypertrichosis). The nevus is due to an overgrowth of the epidermis, pigment cells (melanocytes), and hair follicles. This form of nevus was first documented in 1948 by American dermatologist Samuel William Becker (1894–1964).
A comedo is a clogged hair follicle (pore) in the skin. Keratin (skin debris) combines with oil to block the follicle. A comedo can be open (blackhead) or closed by skin (whitehead), and occur with or without acne. The word comedo comes from the Latin "comedere", meaning "to eat up", and was historically used to describe parasitic worms; in modern medical terminology, it is used to suggest the worm-like appearance of the expressed material.
The chronic inflammatory condition that usually includes both comedones and inflamed papules and pustules (pimples) is called acne. Infection causes inflammation and the development of pus. Whether a skin condition classifies as acne depends on the amount of comedones and infection. Comedones should not be confused with sebaceous filaments.
Comedo-type ductal carcinoma in situ (DCIS) is not related to the skin conditions discussed here. DCIS is a non-invasive form of breast cancer, but comedo-type DCIS may be more aggressive and so may be more likely to become invasive.
Acne scars are caused by inflammation within the dermal layer of skin and are estimated to affect 95% of people with acne vulgaris. The scar is created by abnormal healing following this dermal inflammation. Scarring is most likely to take place with severe acne, but may occur with any form of acne vulgaris. Acne scars are classified based on whether the abnormal healing response following dermal inflammation leads to excess collagen deposition or loss at the site of the acne lesion.
Atrophic acne scars have lost collagen from the healing response and are the most common type of acne scar (account for approximately 75% of all acne scars). They may be further classified as ice-pick scars, boxcar scars, and rolling scars. Ice-pick scars are narrow (less than 2 mm across), deep scars that extend into the dermis. Boxcar scars are round or ovoid indented scars with sharp borders and vary in size from 1.5–4 mm across. Rolling scars are wider than icepick and boxcar scars (4–5 mm across) and have a wave-like pattern of depth in the skin.
Hypertrophic scars are uncommon, and are characterized by increased collagen content after the abnormal healing response. They are described as firm and raised from the skin. Hypertrophic scars remain within the original margins of the wound, whereas keloid scars can form scar tissue outside of these borders. Keloid scars from acne occur more often in men and people with darker skin, and usually occur on the trunk of the body.
Ingrown hair is a condition where 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) or "razor bumps" (pseudofolliculitis barbae), which vary in size. While ingrown hair most commonly appears in areas where the skin is shaved or waxed (beard, legs, pubic region), it can appear anywhere. Anything which causes the hair to be broken off unevenly with a sharp tip can cause ingrown hairs. Ingrown hairs are also caused because of lack of natural exfoliation in the skin.
Blue nevi may be divided into the following types:
- A "patch blue nevus" (also known as an "acquired dermal melanocytosis", and "dermal melanocyte hamartoma") is a cutaneous condition characterized by a diffusely gray-blue area that may have superimposed darker macules.
- A "blue nevus of Jadassohn–Tièche" (also known as a "common blue nevus", and "nevus ceruleus") is a cutaneous condition characterized by a steel-blue papule or nodule.
- A "cellular blue nevus" is a cutaneous condition characterized by large, firm, blue or blue-black nodules.
- An "epithelioid blue nevus" is a cutaneous condition most commonly seen in patients with the Carney complex.
- A "deep penetrating nevus" is a type of benign melanocytic skin tumor characterized, as its name suggests, by penetration into the deep dermis and/or subcutis. Smudged chromatic is a typical finding. In some cases mitotic figures or atypical melanocytic cytology are seen, potentially mimicking a malignant melanoma. Evaluation by an expert skin pathologist is advisable in some cases to help differentiate from invasive melanoma.
- An "amelanotic blue nevus" (also known as a "hypomelanotic blue nevus") is a cutaneous condition characterized by mild atypia and pleomorphism.
- A "malignant blue nevus" is a cutaneous condition characterized by a sheet-like growth pattern, mitoses, necrosis, and cellular atypia.