Diseases of the Skin Appendages Adam Wray, D.O. September 13, 2005

Diseases of the Skin Diseases of the Skin AppendagesAppendages

Adam Wray, D.O.Adam Wray, D.O.

September 13, 2005September 13, 2005

Non-scarring alopeciasNon-scarring alopecias

Alopecia areataAlopecia areata Characterized by rapid and complete loss of Characterized by rapid and complete loss of

hair in one or more often several round or oval hair in one or more often several round or oval patchespatches

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

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

1-5 cm 1-5 cm May progress to total loss of scalp hair – May progress to total loss of scalp hair –

alopecia totalis, or hair loss involving the alopecia totalis, or hair loss involving the entire body – alopecia universalisentire body – alopecia universalis

Alopecia AreataAlopecia Areata

Lifetime risk 1.7%Lifetime risk 1.7% Associated with thyroid disease, Associated with thyroid disease,

vitiligo, inflammatory bowel diseasevitiligo, inflammatory bowel disease AA may be protective against the AA may be protective against the

development of diabetesdevelopment of diabetes• Frequency of type I diabetes is Frequency of type I diabetes is

increased in relatives of patients with increased in relatives of patients with alopecia areataalopecia areata

Alopecia areataAlopecia areata

AA HistoAA Histo

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

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

Ophiasis/sisaiphoOphiasis/sisaipho

Alopecia totalisAlopecia totalis

Telogen effluviumTelogen effluvium

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

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

Associated with Fe deficiency and Associated with Fe deficiency and thyroid diseasethyroid disease

Follicle is not diseased and Follicle is not diseased and inflammation is absentinflammation is absent



““lots of hairs coming out by the roots”lots of hairs coming out by the roots” Loss is diffuse and only infrequently Loss is diffuse and only infrequently

causes clinically perceptible thinning of causes clinically perceptible thinning of hairhair

Normal telogen count is below 10%Normal telogen count is below 10% Can be estimated by the pull testCan be estimated by the pull test Grasping 40 hairs firmly between thumb Grasping 40 hairs firmly between thumb

and forefinger, followed by a slow pulland forefinger, followed by a slow pull Greater than 4-6 club hairs is abnormalGreater than 4-6 club hairs is abnormal

A Telogen fibers A Telogen fibers showing a club shapeshowing a club shape

B Anagen fibers with B Anagen fibers with attached inner root attached inner root sheath, demonstrating sheath, demonstrating pigmented, distorted pigmented, distorted bulbs appearing like bulbs appearing like the end of a the end of a broomstickbroomstick


100 – 200 hairs lost daily100 – 200 hairs lost daily 200 – 400 lost in telogen effluvium200 – 400 lost in telogen effluvium

TXTX

No specific therapyNo specific therapy In the majority of cases it will stop In the majority of cases it will stop

spontaneously within a few months spontaneously within a few months and the hair will regrowand the hair will regrow

Chronic form may occurChronic form may occur 5% minoxidil solution5% minoxidil solution educationeducation

Anagen effluviumAnagen effluvium Seen frequently following the Seen frequently following the

administration of cancer administration of cancer chemotherapeutic agents, such as chemotherapeutic agents, such as antimetabolites, alkylating agents, and antimetabolites, alkylating agents, and mitotic inhibitorsmitotic inhibitors

Only anagen hairs are involvedOnly anagen hairs are involved With cessation of the drug the follicle With cessation of the drug the follicle

resumes normal activity within a few resumes normal activity within a few weeksweeks

Process entirely reversibleProcess entirely reversible Topical minoxidil may decrease the Topical minoxidil may decrease the

period of baldness by an average of 50 period of baldness by an average of 50 daysdays

Anagen effluviumAnagen effluvium

Androgenetic alopeciaAndrogenetic alopeciamale-pattern baldnessmale-pattern baldness

Most prevalent type of hair loss in Most prevalent type of hair loss in humanshumans

Can be inherited from either or both Can be inherited from either or both parentsparents

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

Several patternsSeveral patterns Rate variesRate varies

Pathogenesis of AGAPathogenesis of AGA Related to dihydrotestosterone (DHT)Related to dihydrotestosterone (DHT) Testosterone converted to DHT by 5alpha Testosterone converted to DHT by 5alpha

– reductase– reductase• Two isoenzymes, type I and type IITwo isoenzymes, type I and type II• Type I 5alpha – reductase predominantly found Type I 5alpha – reductase predominantly found

in sebaceous glands and the liver as well as in sebaceous glands and the liver as well as scalp hair folliclesscalp hair follicles

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

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

AGA TXAGA TX MinoxidilMinoxidil

• 2% and 5%2% and 5%• Indefinite treatment to maintain a responseIndefinite treatment to maintain a response• 1/3 cases grow cosmetically useful hair1/3 cases grow cosmetically useful hair

FinasterideFinasteride• Effective in preventing further hair loss Effective in preventing further hair loss

(90% of men) and in increasing the hair (90% of men) and in increasing the hair counts (65% of men)counts (65% of men)

Hair transplantationHair transplantation

Androgenetic alopecia in Androgenetic alopecia in womenwomen

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

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

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

TXTX

Topical minoxidil 2%Topical minoxidil 2% Spironolactone or finasteride if Spironolactone or finasteride if

associated with hyperandrogenemiaassociated with hyperandrogenemia

Androgenetic alopecia in Androgenetic alopecia in womenwomen

Other forms of alopeciaOther forms of alopecia

TrichotillomaniaTrichotillomania

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

Usually localized but may be widespreadUsually localized but may be widespread Areas of alopecia characteristically contain Areas of alopecia characteristically contain

hairs of various lengthshairs of various lengths Seen mostly in girls under 10 and Seen mostly in girls under 10 and womenwomen, ,

may also be seen in boys and adultsmay also be seen in boys and adults


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

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

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

TrichotillomaniaTrichotillomania May be a May be a

manifestation of manifestation of obsessive-obsessive-compulsive disordercompulsive disorder

May be assoc with May be assoc with depression or depression or anxietyanxiety

TX – psychotherapy, TX – psychotherapy, behavioral therapy behavioral therapy or an appropriate or an appropriate psychopharmacologipsychopharmacologic medicationc medication

fluoxetinefluoxetine


Traction alopeciaTraction alopecia

Occurs from prolonged tension on Occurs from prolonged tension on the hairthe hair

Either from the hair tightly braided or Either from the hair tightly braided or in a ponytailin a ponytail

Tractional alopeciaTractional alopecia

Hot comb alopeciaHot comb alopecia

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

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

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

Histo same as for trichotillomania and Histo same as for trichotillomania and traction alopecia (follicular degeneration traction alopecia (follicular degeneration syndromesyndrome

Follicular degeneration syndromeFollicular degeneration syndrome

Pigmented casts in Pigmented casts in folliclefollicle

Empty folliclesEmpty follicles Perifollicular Perifollicular

lymphocytes, plasma lymphocytes, plasma cells, or neutrophils cells, or neutrophils usually sparse or usually sparse or absentabsent

Perifollicular Perifollicular hemorrhage hemorrhage sometimessometimes

Perifollicular fibrosis if Perifollicular fibrosis if follicle is destroyedfollicle is destroyed

Alopecia syphiliticaAlopecia syphilitica

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

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

May be the first sign of a syphilis May be the first sign of a syphilis infectioninfection



Tumor alopeciaTumor alopecia

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

Syringomas, nerve sheath Syringomas, nerve sheath myxomas, and steatocytoma myxomas, and steatocytoma multiplexmultiplex

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

Scarring alopeciasScarring alopecias

Pseudopelade of BrocqPseudopelade of Brocq Non-inflammatory, intermittently progressive Non-inflammatory, intermittently progressive

scarring alopeciascarring alopecia Unknown origin Unknown origin Lesions typically start on the crown and spread in Lesions typically start on the crown and spread in

a “pseudopod-like” fashion at irregular intervalsa “pseudopod-like” fashion at irregular intervals Clinically, pale depressed areas called “footprints Clinically, pale depressed areas called “footprints

in the snow”in the snow” Histo: development of elastic fibers around the Histo: development of elastic fibers around the

lower part of the follicle and less perifollicular lower part of the follicle and less perifollicular inflammationinflammation

Tx: difficult; surgery best optionTx: difficult; surgery best option

pseudopeladepseudopelade

Follicular mucinosisFollicular mucinosis(alopecia mucinosa)(alopecia mucinosa)

Most commonly occurs on the scalp or Most commonly occurs on the scalp or beard areabeard area

Cicatricial alopecia occurs when enough Cicatricial alopecia occurs when enough mucin is deposited in the follicular outer mucin is deposited in the follicular outer root sheath and sebaceous gland to root sheath and sebaceous gland to cause extensive follicular disruptioncause extensive follicular disruption• Otherwise alopecia may be reversibleOtherwise alopecia may be reversible

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

Follicular mucinosisFollicular mucinosis(alopecia mucinosa)(alopecia mucinosa)

More generalized lesion have a More generalized lesion have a longer courselonger course

Young people are primarily affectedYoung people are primarily affected A secondary type – associated with A secondary type – associated with

cutaneous T-cell lymphoma, usually cutaneous T-cell lymphoma, usually more widespread and in older more widespread and in older personspersons

Follicular mucinosisFollicular mucinosis

Inflammatory alopeciaInflammatory alopecia

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

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

HAIR STRUCTURE DEFECTSHAIR STRUCTURE DEFECTS

Increased fragilityIncreased fragility

• TrichothiodystrophyTrichothiodystrophy• Pili tortiPili torti• MonilethrixMonilethrix• Trichorrhexis Trichorrhexis

nodosanodosa• Trichorrhexis Trichorrhexis

invaginatainvaginata

No increased No increased fragilityfragility

Loose anagen hair Loose anagen hair syndromesyndrome

Uncombable hairUncombable hair Woolly hairWoolly hair Pili multigeminiPili multigemini Pili annulatiPili annulati Pili bifurcatiPili bifurcati

Hair structure defectsHair structure defects

Increased fragilityIncreased fragility

TrichothiodystrophyTrichothiodystrophy

Autosomal recessiveAutosomal recessive Brittle hair with Brittle hair with

markedly reduced markedly reduced sulfur contentsulfur content

Sulfur reduced 50% of Sulfur reduced 50% of normal valuenormal value

Distinctive features Distinctive features under polarized light under polarized light and scanning electron and scanning electron microscopymicroscopy


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

With polarized With polarized microscopy the microscopy the hair shows hair shows alternating bright alternating bright and dark regionsand dark regions



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

½ are photosensitive½ are photosensitive Clinical phenotype: PIBIDS Clinical phenotype: PIBIDS

(photosensitivity, ichthyosis, brittle (photosensitivity, ichthyosis, brittle hair, impaired intelligence, hair, impaired intelligence, decreased fertility, short stature)decreased fertility, short stature)

Pili tortiPili torti(twisted hairs)(twisted hairs)

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

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

Scalp, eyebrows, and eyelashesScalp, eyebrows, and eyelashes Hairs are brittle and easily brokenHairs are brittle and easily broken Typically occurs in childhood and Typically occurs in childhood and

improves by pubertyimproves by puberty


May be associated with patchy May be associated with patchy alopeciaalopecia

Follows a dominant inheritance Follows a dominant inheritance patternpattern

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

Assoc with etretinate and isotretinoin Assoc with etretinate and isotretinoin

Pili tortiPili torti

Pili tortiPili torti


Menkes’ Kinky Hair SyndromeMenkes’ Kinky Hair Syndrome(steely hair disease)(steely hair disease)

X-linked recessive deficiency of X-linked recessive deficiency of serum copper and copper-dependent serum copper and copper-dependent enzymesenzymes

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

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

Menkes’ syndromeMenkes’ syndrome

Pudgy face with “cupid’s bow” upper Pudgy face with “cupid’s bow” upper liplip

Doughy skinDoughy skin Psychomotor retardationPsychomotor retardation SeizuresSeizures Majority die in early childhoodMajority die in early childhood Mutation in Mutation in ATP7A, ATP7A, a gene that a gene that

encodes a copper transporting encodes a copper transporting ATPaseATPase

Menkes’ Kinky Hair SyndromeMenkes’ Kinky Hair Syndrome(steely hair disease)(steely hair disease)

MonilethrixMonilethrix(beaded hairs)(beaded hairs)

Greek and latin derivative: necklace hairGreek and latin derivative: necklace hair Autosomal dominantAutosomal dominant Short, fragile, broken hair that appears Short, fragile, broken hair that appears

normal at birthnormal at birth Ultrastructure: alternating elliptical Ultrastructure: alternating elliptical

nodes and constrictionsnodes and constrictions Scalp usually the only region involvedScalp usually the only region involved Involvement of the hair may occur Involvement of the hair may occur

during pregnancy and resolve after during pregnancy and resolve after deliverydelivery


Improvement may occur with ageImprovement may occur with age Seasonal improvement during the Seasonal improvement during the

summersummer Has been described in assoc with Has been described in assoc with

Menkes’Menkes’ Disease of the hair cortexDisease of the hair cortex No effective treatmentNo effective treatment



Trichorrhexis nodosaTrichorrhexis nodosa

Most common of all the structural hair Most common of all the structural hair abnormalitiesabnormalities

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

The nodes are the sites of fracture of the hair The nodes are the sites of fracture of the hair cortexcortex

Hairs soon break at the nodesHairs soon break at the nodes Found mostly on the scalpFound mostly on the scalp Often in a small area or areasOften in a small area or areas Pubic area, axillae and chest may be involvedPubic area, axillae and chest may be involved Microscopy: individual cortical cells and their Microscopy: individual cortical cells and their

fragments play out at fracture pointfragments play out at fracture point

Trichorrhexis nodosaTrichorrhexis nodosa

Trichorrhexis invaginataTrichorrhexis invaginata(bamboo hair)(bamboo hair)

Caused by intussusception of the hair Caused by intussusception of the hair shaft at the zone where keratinization shaft at the zone where keratinization beginsbegins

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

patient has nodose ball and socket patient has nodose ball and socket deformitiesdeformities

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

Trichorrhexis invaginataTrichorrhexis invaginata

Trichorrhexis invaginataTrichorrhexis invaginata(bamboo hair)(bamboo hair)

Netherton’s syndromeNetherton’s syndrome

Netherton’sNetherton’ssyndromesyndrome

Hair shaft abnormalitiesHair shaft abnormalities

No increased fragilityNo increased fragility

Loose anagen syndromeLoose anagen syndrome

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

Occurs mostly in blonde girls Occurs mostly in blonde girls No increased hair fragilityNo increased hair fragility Anagen hair can be easily and painlessly Anagen hair can be easily and painlessly

pulled from the scalppulled from the scalp Improves with age Improves with age Microscopic exam reveals a ruffled Microscopic exam reveals a ruffled

proximal cuticle and absence of the root proximal cuticle and absence of the root sheathsheath

Loose anagen syndromeLoose anagen syndrome

UncombableUncombablehair syndromehair syndrome

Uncombable hair syndromeUncombable hair syndrome(pili triangulati et canaliculi)(pili triangulati et canaliculi)

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

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

Longitudinal grooves make hair rigidLongitudinal grooves make hair rigid Autosomal dominant inheritanceAutosomal dominant inheritance Sporadic cases reportedSporadic cases reported Some cases improve spontaneously in Some cases improve spontaneously in

childhoodchildhood

UncombableUncombablehair syndromehair syndrome

Pili annulatiPili annulati(Ringed Hair)(Ringed Hair)

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

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

Hair growth is normalHair growth is normal No other assoc abnormalitiesNo other assoc abnormalities Autosomal dominantAutosomal dominant Begins in infancy and requires no Begins in infancy and requires no

treatmenttreatment

Pili annulatiPili annulati(Ringed Hair)(Ringed Hair)

Pili pseudoannulatiPili pseudoannulati

Mimics pili annulatiMimics pili annulati Bright segments are caused by Bright segments are caused by

reflection and refraction of light by reflection and refraction of light by flattened, twisted surfaces of hairflattened, twisted surfaces of hair

Variant of normal hairVariant of normal hair

Woolly hairWoolly hair Present at birth and usually most Present at birth and usually most

severe during childhoodsevere during childhood Variable amelioration in adulthoodVariable amelioration in adulthood Four subgroupsFour subgroups

• Hereditary woolly hair (AD)Hereditary woolly hair (AD)• Familial woolly hair (AR)Familial woolly hair (AR)• Woolly hair nevusWoolly hair nevus• Naxos diseaseNaxos disease

Tend to unite into Tend to unite into lockslocks

No assoc No assoc cutaneous or cutaneous or systemic diseasessystemic diseases

Woolly hairWoolly hair

Woolly hairWoolly hairNOTNOT

Pili multigeminiPili multigemini

Rare malformation of the pilary Rare malformation of the pilary apparatusapparatus

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

No treatmentNo treatment

Pili multigeminiPili multigemini

Pili bifurcatiPili bifurcati

Described in a 3 year old seen for Described in a 3 year old seen for hair losshair loss

Bifurcation was found in short Bifurcation was found in short segments along the shafts of several segments along the shafts of several hairshairs

Anomaly was transientAnomaly was transient

Pili bifurcatiPili bifurcati

Miscellaneous Miscellaneous hair/follicle abnormalitieshair/follicle abnormalities

Trichostasis spinulosaTrichostasis spinulosa A common disorder of the hair follicles A common disorder of the hair follicles

that clinically gives the impression of that clinically gives the impression of follicular keratosisfollicular keratosis

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

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

Occurs on the nose and forehead of Occurs on the nose and forehead of elderlyelderly

Shoulders and backShoulders and back

Trichostasis spinulosaTrichostasis spinulosa

Results from retention of telogen Results from retention of telogen hairshairs

Treatment with keratolytics after Treatment with keratolytics after using a wax depilatoryusing a wax depilatory

Tretinoin 0.05% solutionTretinoin 0.05% solution



Trichostasis spinulosaTrichostasis spinulosathe removed plugthe removed plug

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


Numerous vellus hairs Numerous vellus hairs within a folliclewithin a follicle

HypertrichosisHypertrichosis

An overgrowth of hair not localized to An overgrowth of hair not localized to the androgen-dependent areas of the the androgen-dependent areas of the skin.skin.• As opposed to hirsutism which is As opposed to hirsutism which is

excessive growth of terminal hairs in a excessive growth of terminal hairs in a “male pattern”“male pattern”

Several forms existSeveral forms exist

Localized acquired Localized acquired hypertrichosishypertrichosis

Systemic diseaseSystemic disease• PCTPCT

After repeated trauma, friction, After repeated trauma, friction, irritation or inflammation; hair may irritation or inflammation; hair may become longer and thickerbecome longer and thicker• On back in sack carriers, affected limb On back in sack carriers, affected limb

after plaster castafter plaster cast

PCTPCT

Congenital localized hypertrichosisCongenital localized hypertrichosis

Congenital melanocytic neviCongenital melanocytic nevi• Hair may be present at birth, but usually increases in Hair may be present at birth, but usually increases in

infancyinfancy Becker’s neviBecker’s nevi

• Hamartoma, not a true nevusHamartoma, not a true nevus• Associated SNUB syndrome (supernummary nipples, Associated SNUB syndrome (supernummary nipples,

uropathies, Becker’s nevus)uropathies, Becker’s nevus) Nevoid hypertrichosisNevoid hypertrichosis

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

Spinal dysraphismSpinal dysraphism• Alteration in the formation of a fold or elevation which is Alteration in the formation of a fold or elevation which is

constituted in the midline of the body as a consequence constituted in the midline of the body as a consequence of the union of two lateral portionsof the union of two lateral portions

• ““faun tail” mark as in spina bifidafaun tail” mark as in spina bifida

Becker’s nevusBecker’s nevus

Becker’sBecker’snevusnevus

GeneralizedGeneralized Congenital HypertrichosisCongenital Hypertrichosis(congenital hypertrichosis lanuginosa)(congenital hypertrichosis lanuginosa)

Rare type of excessive and generalized Rare type of excessive and generalized hairinesshairiness

Fully penetrant X-linked dominant traitFully penetrant X-linked dominant trait Entire body covered with fine vellus hairs Entire body covered with fine vellus hairs

2-10 cm long, spares palms and soles2-10 cm long, spares palms and soles Scalp hair is normalScalp hair is normal May be assoc with dental anomalies and May be assoc with dental anomalies and

gingival fibromatosisgingival fibromatosis

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

Generalized Congenital HypertrichosisGeneralized Congenital Hypertrichosis(congenital hypertrichosis lanuginosa)(congenital hypertrichosis lanuginosa)

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

Fetal hydantoin syndromeFetal hydantoin syndrome Fetal alcohol syndromeFetal alcohol syndrome Minoxidil – case of generalized Minoxidil – case of generalized

hypertrichosis and multiple hypertrichosis and multiple congenital defects in a baby born congenital defects in a baby born to a mother who used minoxidil to a mother who used minoxidil throughout pregnancythroughout pregnancy

Generalized or patterned Generalized or patterned acquired hypertrichosisacquired hypertrichosis

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

Also an ominous sign of internal Also an ominous sign of internal malignancymalignancy

HirsutismHirsutism An excess of terminal hair growth in An excess of terminal hair growth in

women in a pattern more typical of menwomen in a pattern more typical of men Androgen-dependent growth areas Androgen-dependent growth areas

affected include the upper lip, cheeks, affected include the upper lip, cheeks, chin, central chest, breasts, lower chin, central chest, breasts, lower abdomen and groinabdomen and groin

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

Acne may be seenAcne may be seen When virilization accompanies hirsutism, When virilization accompanies hirsutism,

especially when progression is rapid, a especially when progression is rapid, a neoplastic cause is likelyneoplastic cause is likely

HirsutismHirsutismpathogenesispathogenesis

Racial variationRacial variation May result either from excessive May result either from excessive

androgens from either the ovary or androgens from either the ovary or the adrenal gland, or from excessive the adrenal gland, or from excessive stimulation by pituitary tumorsstimulation by pituitary tumors

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


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

Stein-Leventhal syndrome Stein-Leventhal syndrome characterized by hirsutism 50%characterized by hirsutism 50%

Serum free testosterone is generally Serum free testosterone is generally elevated as is luteinizing hormoneelevated as is luteinizing hormone


Adrenal causes include congenital Adrenal causes include congenital adrenal hyperplasia and adrenal tumors adrenal hyperplasia and adrenal tumors such as adrenal adenomas and such as adrenal adenomas and carcinomascarcinomas

Adrenogenital syndrome is an autosomal Adrenogenital syndrome is an autosomal dominant disorder resulting from dominant disorder resulting from deficiencies in the following enzymes:deficiencies in the following enzymes:• 21-hydroxylase21-hydroxylase• 11B-hydroxylase11B-hydroxylase• 3B-hydroxy steroid dehydrogenase3B-hydroxy steroid dehydrogenase


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

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


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

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

diagnosisdiagnosis

Refer to endocrinologistRefer to endocrinologist Refer to gynecologistRefer to gynecologist Major elevation in the DHEA sulfate Major elevation in the DHEA sulfate

level (above 7000 ng/ml) suggests an level (above 7000 ng/ml) suggests an adrenal neoplasm CT is adrenal neoplasm CT is recommendedrecommended

treatmenttreatment

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

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

treatmenttreatment

Oral contraceptives and Oral contraceptives and glucocorticoidsglucocorticoids

bcp helpful in 75% of hirsute womenbcp helpful in 75% of hirsute women Antiandrogens include, cimetidine, Antiandrogens include, cimetidine,

cyproterone acetate, spironolactone, cyproterone acetate, spironolactone, flutamide, and ketoconazoleflutamide, and ketoconazole

FinasterideFinasteride Gonadotropin-releasing hormone Gonadotropin-releasing hormone

agonist such as leuprolide and agonist such as leuprolide and nafarelinnafarelin

Documents

Diseases of the Skin Appendages Adam Wray, D.O. September 13, 2005