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Diseases of the Skin Appendages. Adam Wray, D.O. September 13, 2005. Non-scarring alopecias. Alopecia areata. Characterized by rapid and complete loss of hair in one or more often several round or oval patches

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Diseases of the skin appendages l.jpg

Diseases of the Skin Appendages

Adam Wray, D.O.

September 13, 2005



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Alopecia areata

  • Characterized by rapid and complete loss of hair in one or more often several round or oval patches

  • “Exclamation point” hairs that are tapered at the base may persist in localized patches

  • Usually on the scalp, bearded areas, eyebrows and eyelashes

  • 1-5 cm

  • May progress to total loss of scalp hair – alopecia totalis, or hair loss involving the entire body – alopecia universalis


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Alopecia Areata

  • Lifetime risk 1.7%

  • Associated with thyroid disease, vitiligo, inflammatory bowel disease

  • AA may be protective against the development of diabetes

    • Frequency of type I diabetes is increased in relatives of patients with alopecia areata



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AA Histo

  • Lymphocytes surround lower portion of follicle in early lesions = “swarm of bees”

  • Increased numbers of miniature telogen and catagen follicles or sometimes early anagen hair follicles in the superficial dermis




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Telogen effluvium

  • Early and excessive loss of normal club hairs from normal resting follicles in the scalp

  • Loss results from traumatization of the normal hair by some stimulus, such as surgery, parturition, fever, drugs, or traction which precipitates the anagen phase into catagen and telogen phases

  • Associated with Fe deficiency and thyroid disease

  • Follicle is not diseased and inflammation is absent



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Telogen effluvium

  • “lots of hairs coming out by the roots”

  • Loss is diffuse and only infrequently causes clinically perceptible thinning of hair

  • Normal telogen count is below 10%

  • Can be estimated by the pull test

  • Grasping 40 hairs firmly between thumb and forefinger, followed by a slow pull

  • Greater than 4-6 club hairs is abnormal


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Telogen effluvium

  • 100 – 200 hairs lost daily

  • 200 – 400 lost in telogen effluvium


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TX

  • No specific therapy

  • In the majority of cases it will stop spontaneously within a few months and the hair will regrow

  • Chronic form may occur

  • 5% minoxidil solution

  • education


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Anagen effluvium

  • Seen frequently following the administration of cancer chemotherapeutic agents, such as antimetabolites, alkylating agents, and mitotic inhibitors

  • Only anagen hairs are involved

  • With cessation of the drug the follicle resumes normal activity within a few weeks

  • Process entirely reversible

  • Topical minoxidil may decrease the period of baldness by an average of 50 days



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Androgenetic alopeciamale-pattern baldness

  • Most prevalent type of hair loss in humans

  • Can be inherited from either or both parents

  • Starts during the 20s to early 30s by gradual loss of hair, chiefly from the vertex and frontotemporal regions

  • Several patterns

  • Rate varies


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Pathogenesis of AGA

  • Related to dihydrotestosterone (DHT)

  • Testosterone converted to DHT by 5alpha – reductase

    • Two isoenzymes, type I and type II

    • Type I 5alpha – reductase predominantly found in sebaceous glands and the liver as well as scalp hair follicles

    • Type II 5alpha – reductase is present in scalp hair follicles, beard, and chest hair, liver, and prostate

  • Genetic absence of type II 5alpha – reductase is protective from the development of male AGA


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AGA TX

  • Minoxidil

    • 2% and 5%

    • Indefinite treatment to maintain a response

    • 1/3 cases grow cosmetically useful hair

  • Finasteride

    • Effective in preventing further hair loss (90% of men) and in increasing the hair counts (65% of men)

  • Hair transplantation


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Androgenetic alopecia in women

  • Generally diffuse hair loss throughout the midscalp, sparing the frontal hairline except for slight recession

  • The midline part is an important clinical clue to the diagnosis, revealing this central thinning by the appearance of the “Christmas tree pattern”

  • The cause is now believed to be a genetic predisposition in combination with an excessive androgen response


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TX

  • Topical minoxidil 2%

  • Spironolactone or finasteride if associated with hyperandrogenemia




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Trichotillomania

  • A neurotic practice of plucking or breaking hair from the scalp, eyelashes, eyebrows, extremities, or pubic region

  • Usually localized but may be widespread

  • Areas of alopecia characteristically contain hairs of various lengths

  • Seen mostly in girls under 10 and women, may also be seen in boys and adults


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Trichotillomania

  • It has been suggested that one ask the child not if but rather how the removal is done

  • Shave 3 X 3 cm area and watch the hair regrow normally. Hairs in this “skin window” will be too short for plucking

  • Biopsy – high number of catagen hairs, pigmentary defects and casts, trichomalacia and hemorrhage, follicular plugging


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May be a manifestation of obsessive-compulsive disorder

May be assoc with depression or anxiety

TX – psychotherapy, behavioral therapy or an appropriate psychopharmacologic medication

fluoxetine

Trichotillomania



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Traction alopecia

  • Occurs from prolonged tension on the hair

  • Either from the hair tightly braided or in a ponytail



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Hot comb alopecia

  • Develops characteristically on the crown and spreads peripherally to form a large oval area of partial hair loss

  • Initially reported in black women who straightened their hair with hot combs

  • The hot petrolatum used with the iron causes thermal damage to the hair follicle

  • Histo same as for trichotillomania and traction alopecia (follicular degeneration syndrome


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Follicular degeneration syndrome

  • Pigmented casts in follicle

  • Empty follicles

  • Perifollicular lymphocytes, plasma cells, or neutrophils usually sparse or absent

  • Perifollicular hemorrhage sometimes

  • Perifollicular fibrosis if follicle is destroyed


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Alopecia syphilitica

  • May have a typical moth-eaten appearance on the occipital scalp, a generalized thinning, or a combination of the two

  • Eyebrows and lashes, and other body hair may be involved

  • May be the first sign of a syphilis infection




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Tumor alopecia

  • Refers to hair loss in the immediate vicinity of either benign or malignant tumors of the scalp

  • Syringomas, nerve sheath myxomas, and steatocytoma multiplex

  • Alopecia neoplastica – hair loss from metastatic tumors, most commonly breast carcinoma



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Pseudopelade of Brocq

  • Non-inflammatory, intermittently progressive scarring alopecia

  • Unknown origin

  • Lesions typically start on the crown and spread in a “pseudopod-like” fashion at irregular intervals

  • Clinically, pale depressed areas called “footprints in the snow”

  • Histo: development of elastic fibers around the lower part of the follicle and less perifollicular inflammation

  • Tx: difficult; surgery best option



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Follicular mucinosis(alopecia mucinosa)

  • Most commonly occurs on the scalp or beard area

  • Cicatricial alopecia occurs when enough mucin is deposited in the follicular outer root sheath and sebaceous gland to cause extensive follicular disruption

    • Otherwise alopecia may be reversible

  • Primary cases occur either as localized lesions of the head or neck typically resloving within a year


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Follicular mucinosis(alopecia mucinosa)

  • More generalized lesion have a longer course

  • Young people are primarily affected

  • A secondary type – associated with cutaneous T-cell lymphoma, usually more widespread and in older persons



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Inflammatory alopecia

  • May be seen in lichen simplex chronicus and various eczematous changes on the scalp, including kerion

  • DLE, lichen planopilaris, sarcoidosis, and folliculitis decalvans are the commonest inflammatory causes of cicatricial alopecia


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Increased fragility

Trichothiodystrophy

Pili torti

Monilethrix

Trichorrhexis nodosa

Trichorrhexis invaginata

No increased fragility

Loose anagen hair syndrome

Uncombable hair

Woolly hair

Pili multigemini

Pili annulati

Pili bifurcati

HAIR STRUCTURE DEFECTS


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Hair structure defects

Increased fragility


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Autosomal recessive

Brittle hair with markedly reduced sulfur content

Sulfur reduced 50% of normal value

Distinctive features under polarized light and scanning electron microscopy

Trichothiodystrophy


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With light microscopy trichoschisis (clean transverse fractures) may be seen

With polarized microscopy the hair shows alternating bright and dark regions

Trichothiodystrophy


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Trichothiodystrophy fractures) may be seen


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Trichothiodystrophy fractures) may be seen

  • Different mutations in the ERCC2 DNA repair gene may lead to different phenotypic expressions of the disease

  • ½ are photosensitive

  • Clinical phenotype: PIBIDS (photosensitivity, ichthyosis, brittle hair, impaired intelligence, decreased fertility, short stature)


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Pili torti fractures) may be seen(twisted hairs)

  • A malformation of hair characterized by twisting of the hair shaft on its own axis

  • The hair shaft is segmentally thickened and light and dark segments are seen

  • Scalp, eyebrows, and eyelashes

  • Hairs are brittle and easily broken

  • Typically occurs in childhood and improves by puberty


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Pili torti fractures) may be seen(twisted hairs)

  • May be associated with patchy alopecia

  • Follows a dominant inheritance pattern

  • May be assoc with Bjornstad’s syndrome, citrullinemia, Menkes’ kinky hair syndrome, Bazex’s follicular atrophoderma syndrome trichothiodystrophy

  • Assoc with etretinate and isotretinoin


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Pili torti fractures) may be seen


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Pili torti fractures) may be seen


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Pili torti fractures) may be seen(twisted hairs)


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Menkes’ Kinky Hair Syndrome fractures) may be seen(steely hair disease)

  • X-linked recessive deficiency of serum copper and copper-dependent enzymes

  • Pili torti, and often monilethrix and trichorrhexis nodosa are common

  • Characteristic ivory color of the hair appears between 1 and 5 months


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Menkes’ syndrome fractures) may be seen

  • Pudgy face with “cupid’s bow” upper lip

  • Doughy skin

  • Psychomotor retardation

  • Seizures

  • Majority die in early childhood

  • Mutation in ATP7A, a gene that encodes a copper transporting ATPase


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Menkes’ Kinky Hair Syndrome fractures) may be seen(steely hair disease)


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Monilethrix fractures) may be seen(beaded hairs)

  • Greek and latin derivative: necklace hair

  • Autosomal dominant

  • Short, fragile, broken hair that appears normal at birth

  • Ultrastructure: alternating elliptical nodes and constrictions

  • Scalp usually the only region involved

  • Involvement of the hair may occur during pregnancy and resolve after delivery


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Monilethrix fractures) may be seen(beaded hairs)

  • Improvement may occur with age

  • Seasonal improvement during the summer

  • Has been described in assoc with Menkes’

  • Disease of the hair cortex

  • No effective treatment


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Monilethrix fractures) may be seen(beaded hairs)


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Monilethrix fractures) may be seen(beaded hairs)


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Trichorrhexis nodosa fractures) may be seen

  • Most common of all the structural hair abnormalities

  • Affected hair shafts may have small white nodes arranged at irregular intervals

  • The nodes are the sites of fracture of the hair cortex

  • Hairs soon break at the nodes

  • Found mostly on the scalp

  • Often in a small area or areas

  • Pubic area, axillae and chest may be involved

  • Microscopy: individual cortical cells and their fragments play out at fracture point


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Trichorrhexis nodosa fractures) may be seen


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Trichorrhexis invaginata fractures) may be seen(bamboo hair)

  • Caused by intussusception of the hair shaft at the zone where keratinization begins

  • The invagination is caused by the softness of the cortex in the keratogenous zone

  • patient has nodose ball and socket deformities

  • Associated with Netherton’s syndrome with ichthyosiform erythroderma or ichthyosis linearis circumflexa


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Trichorrhexis invaginata fractures) may be seen


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Trichorrhexis invaginata fractures) may be seen(bamboo hair)


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Netherton’s syndrome fractures) may be seen


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Netherton’s fractures) may be seensyndrome


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Hair shaft abnormalities fractures) may be seen

No increased fragility


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Loose anagen syndrome fractures) may be seen

  • Disorder in which anagen hairs may be pulled from the scalp with little effort

  • Occurs mostly in blonde girls

  • No increased hair fragility

  • Anagen hair can be easily and painlessly pulled from the scalp

  • Improves with age

  • Microscopic exam reveals a ruffled proximal cuticle and absence of the root sheath


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Loose anagen syndrome fractures) may be seen


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Uncombable fractures) may be seenhair syndrome


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Uncombable hair syndrome fractures) may be seen(pili triangulati et canaliculi)

  • “Spun glass” appearance due to the reflection of light from variably oriented, flattened hair surfaces

  • Noted in the first years of life as dry, blonde, shiny hair that stands straight out from the scalp and cannot be combed

  • Longitudinal grooves make hair rigid

  • Autosomal dominant inheritance

  • Sporadic cases reported

  • Some cases improve spontaneously in childhood


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Uncombable fractures) may be seenhair syndrome


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Pili annulati fractures) may be seen(Ringed Hair)

  • The hair seems banded by alternating segments of light and dark color when seen in reflected light

  • Light bands are caused by clusters of abnormal air-filled cavities which scatter light

  • Hair growth is normal

  • No other assoc abnormalities

  • Autosomal dominant

  • Begins in infancy and requires no treatment


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Pili annulati fractures) may be seen(Ringed Hair)


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Pili pseudoannulati fractures) may be seen

  • Mimics pili annulati

  • Bright segments are caused by reflection and refraction of light by flattened, twisted surfaces of hair

  • Variant of normal hair


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Woolly hair fractures) may be seen

  • Present at birth and usually most severe during childhood

  • Variable amelioration in adulthood

  • Four subgroups

    • Hereditary woolly hair (AD)

    • Familial woolly hair (AR)

    • Woolly hair nevus

    • Naxos disease


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Tend to unite into locks fractures) may be seen

No assoc cutaneous or systemic diseases


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Woolly hair fractures) may be seen


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Woolly hair fractures) may be seenNOT


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Pili multigemini fractures) may be seen

  • Rare malformation of the pilary apparatus

  • Characterized by the presence of bifurcated or multiple divided hair matrices and papillae, giving rise to the formation of multiple hair shafts within the individual follicles

  • No treatment


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Pili multigemini fractures) may be seen


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Pili bifurcati fractures) may be seen

  • Described in a 3 year old seen for hair loss

  • Bifurcation was found in short segments along the shafts of several hairs

  • Anomaly was transient


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Pili bifurcati fractures) may be seen



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Trichostasis spinulosa fractures) may be seen

  • A common disorder of the hair follicles that clinically gives the impression of follicular keratosis

  • Follicles are filled with funnel-shaped horny plugs within which are bundles of vellus hairs

  • Hairs are round at their proximal ends and are shredded distally

  • Occurs on the nose and forehead of elderly

  • Shoulders and back


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Trichostasis spinulosa fractures) may be seen

  • Results from retention of telogen hairs

  • Treatment with keratolytics after using a wax depilatory

  • Tretinoin 0.05% solution


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Trichostasis spinulosa fractures) may be seen


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Trichostasis spinulosa fractures) may be seen


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Microscopically the tufts are made up of several dozen fine, stubbly hairs

Trichostasis spinulosathe removed plug


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Trichostasis spinulosa stubbly hairs

  • Numerous vellus hairs within a follicle


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Hypertrichosis stubbly hairs

  • An overgrowth of hair not localized to the androgen-dependent areas of the skin.

    • As opposed to hirsutism which is excessive growth of terminal hairs in a “male pattern”

  • Several forms exist


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Localized acquired hypertrichosis stubbly hairs

  • Systemic disease

    • PCT

  • After repeated trauma, friction, irritation or inflammation; hair may become longer and thicker

    • On back in sack carriers, affected limb after plaster cast


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PCT stubbly hairs


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Congenital localized hypertrichosis stubbly hairs

  • Congenital melanocytic nevi

    • Hair may be present at birth, but usually increases in infancy

  • Becker’s nevi

    • Hamartoma, not a true nevus

    • Associated SNUB syndrome (supernummary nipples, uropathies, Becker’s nevus)

  • Nevoid hypertrichosis

    • Alteration characterized by growth of terminal hairs in a circumscribed area

  • Spinal dysraphism

    • Alteration in the formation of a fold or elevation which is constituted in the midline of the body as a consequence of the union of two lateral portions

    • “faun tail” mark as in spina bifida


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Becker’s nevus stubbly hairs


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Becker’s stubbly hairs nevus


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Generalized stubbly hairs Congenital Hypertrichosis(congenital hypertrichosis lanuginosa)

  • Rare type of excessive and generalized hairiness

  • Fully penetrant X-linked dominant trait

  • Entire body covered with fine vellus hairs 2-10 cm long, spares palms and soles

  • Scalp hair is normal

  • May be assoc with dental anomalies and gingival fibromatosis


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“dog-faced boy” “human werewolf” stubbly hairs “human Skye terrier”


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Generalized Congenital Hypertrichosis stubbly hairs (congenital hypertrichosis lanuginosa)

  • Other cases may be secondary to drug ingestion by the mother

  • Fetal hydantoin syndrome

  • Fetal alcohol syndrome

  • Minoxidil – case of generalized hypertrichosis and multiple congenital defects in a baby born to a mother who used minoxidil throughout pregnancy


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Generalized or patterned acquired hypertrichosis stubbly hairs

  • These cases include those caused by acquired hypertrichosis lanuginosa, those associated with various syndromes, and those secondary to drug intake

  • Also an ominous sign of internal malignancy


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Hirsutism stubbly hairs

  • An excess of terminal hair growth in women in a pattern more typical of men

  • Androgen-dependent growth areas affected include the upper lip, cheeks, chin, central chest, breasts, lower abdomen and groin

  • May or may not be assoc with other signs of virilization

  • Acne may be seen

  • When virilization accompanies hirsutism, especially when progression is rapid, a neoplastic cause is likely


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Hirsutism stubbly hairs pathogenesis

  • Racial variation

  • May result either from excessive androgens from either the ovary or the adrenal gland, or from excessive stimulation by pituitary tumors

  • All cases of progressive or severe hirsutism should be investigated for an endocrinopathy


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Hirsutism stubbly hairs pathogenesis

  • Ovarian causes include polycystic ovarian disease and a variety of ovarian tumors, both benign and malignant

  • Stein-Leventhal syndrome characterized by hirsutism 50%

  • Serum free testosterone is generally elevated as is luteinizing hormone


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Hirsutism stubbly hairs pathogenesis

  • Adrenal causes include congenital adrenal hyperplasia and adrenal tumors such as adrenal adenomas and carcinomas

  • Adrenogenital syndrome is an autosomal dominant disorder resulting from deficiencies in the following enzymes:

    • 21-hydroxylase

    • 11B-hydroxylase

    • 3B-hydroxy steroid dehydrogenase


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Hirsutism stubbly hairs pathogenesis

  • Pituitary causes include Cushing’s disease, acromegaly, and prolactin-secreting adenomas

  • Other conditions in which prolactin levels may be elevated and that may lead to hirsutism include hypothyroidism, phenothiazine intake, and hepatorenal failure


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Hirsutism stubbly hairs pathogenesis

  • Other causes include the exogenous intake of androgens and certain high-progesterone bcp (uncommonly)

  • Minoxidil, diazoxide, corticosteroids and phenytoin, which have been reported to cause hirsutism, generally cause hypertrichosis


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diagnosis stubbly hairs

  • Refer to endocrinologist

  • Refer to gynecologist

  • Major elevation in the DHEA sulfate level (above 7000 ng/ml) suggests an adrenal neoplasm CT is recommended


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treatment stubbly hairs

  • Once appropriate testing has led to diagnosis and referral of patients requiring special methods of specific treatment, such as surgical intervention, therapeutic alternatives including cosmetic treatments, nonspecific suppressive therapy, and specific antiandrogens can be used

  • Shaving, wax depilatories, chemical depilatories, bleaching of the hair, laser hair removal, and electrolysis


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treatment stubbly hairs

  • Oral contraceptives and glucocorticoids

  • bcp helpful in 75% of hirsute women

  • Antiandrogens include, cimetidine, cyproterone acetate, spironolactone, flutamide, and ketoconazole

  • Finasteride

  • Gonadotropin-releasing hormone agonist such as leuprolide and nafarelin