Hair and Nails Dan Flashcards

1
Q

What are the causes of Pityriasis amiantacea?

what is treatment?

A

Psoriasis
seb derm
eczema

use strong keratolytics e.g. leave in coal tar and sal acid prep then topical steroids; diagnosis may become clear later

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2
Q

What are Terry’s nails?

What are the associations?

A
White nails with a narrow distal pink/red/brown band
May or may not see the lunulae
Assoc;
Liver failure
Renal failure
CCF
Diabetes
Hyperthyroidism
Malnutrition 
POEMS
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3
Q

T/F

FFA has been triggered by starting TNFα blockers

A

T

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4
Q

How long is anagen phase for scalp hairs?

A

2-6 years

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5
Q

How many scalp hairs are in anagen or telogen in the scalp?

A

90% in anagen
10% in telogen
small number in catagen - less than 1%

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6
Q

How quickly does scalp hair grow?

A

1cm per month

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7
Q

T/F

catagen phase is always about 2 weeks in all human hairs

A

T

No matter follicle type or site

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8
Q

How long does telogen phase last on scalp?

A

3 months

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9
Q

what is exogen?

A

end stage of telogen when new hair starts growing and telogen hair is shed from follice

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10
Q

which keratins are found in hair?

A

mainly keratin 1 and 2 almost 50/50 ratio

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11
Q

how many hairs are normally in a scalp follicular unit?

A

3-5 follicles connected to a single erector pili muscle

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12
Q

how much scalp hair is lost before thinning become evident in most cases?

A

over 50%

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13
Q

T/F

miniaturization is the process of terminal scalp hairs turning into vellus hairs mainly in pattern hair loss

A

T

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14
Q

what is kenogen?

A

when a telogen follicle has lost its club fibre (telogen hair) but not yet transitioned to anagen

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15
Q

T/F

type 1 5alpha reductase is found in the scalp, beard and chest hair follicles

A

F
type 2 5alpha reductase
converts testosterone to DHT which drives male AGA

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16
Q

what are the major DDs for diffuse hairloss?

A
PHL/AGA
acute TE
chronic TE
diffuse AA (rare)
diffuse anagen effluvium - nearly always drug or disease
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17
Q

T/F

AGA in prepubertal children who do not have precocious puberty/hyperandrogenism is rare and usually male pattern type

A

F

rare but usually female pattern type

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18
Q

what is grading system for MPHL?

A

Hamilton or Norwood systems

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19
Q

what is grading system for FPHL?

A

Sinclair or Ludwig systems

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20
Q

what is trichodynia?

A

scalp paraesthesia

often occurs in PHL esp FPHL in 20-30% of women

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21
Q

what is the Sinclair grading system of FPHL?

A

1 - normal
2 - widened central part line
3 - widened part + tanslucent border of central part line
4 - bald area along anterior of part line
5 - advanced hair loss

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22
Q

what are key trichoscopic and histo features of AGA?

A

Trichoscopy;
variable hair shaft thickness due to miniturization
Peripilar sign - brown halos around hair ostia
yellow dots (yellow ostia)

Histo;
reduced ratio of terminal:vellus hairs from over 8:1 (nomal) to
less than 4:1
+ some increase in telogen follicle count and fibrous streamers

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23
Q

what investigations should be done in PHL?

A

None routinely
If young women w/ not FHx or any other indication then do hormone screen
Consider TFTs, ferritin and zinc in all cases
sometimes B12, folate, VitD, ANA
If diagnosis unclear consider biopsy

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24
Q

What is treatment ladder for PHL

A

reassure and do nothing - eg if normal temporal loss; does not indicate increased risk of further PHL in men or women
treat dandruff/scalp disease and any reversible causes of hairloss or nutritional deficiency
advise sun protection if scalp visible
assess psychosocial impact and manage as necessary
camouflage - hair styles, wig, toppik thickener, scalp dye
topical - minoxidil 5%
oral - minoxidil, finasteride, dutastride (men only)
women - cOCP, spironolactone, CPA
surgical - excision, flap, scalp expander, follicular unit transplants

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25
what are the associations of alopecia areata?
``` Nail changes Atopy inc eczema Hypothyroid (hashimotos) LS IBD Vitiligo Autoimmune polyendocrinopathy syndrome 1 ```
26
what nail changes are seen in alopecia areata?
``` brittle nails onycholysis Beau's lines Onychomadesis Koilonychia Trachyonychia Pitting - fine, organized (stippled), in grid pattern ```
27
what are poor prognostic factors in alopecia areata?
``` POLE V Yellow Paediatric onset Ophiasis Long standing Extensive trichoscopy - vellus hairs, yellow dots ```
28
how long do nails take to grow?
fingernails 3mm/month | toenails 1mm/month
29
T/F | treating associated hypothyroidism will improve alopecia areata
F | usually not but worth checking for and treating anyway
30
what is treatment ladder for AA?
Must assess psychosocial impact - if very concerned may treat more aggressively General; - Reassurance - 50% resolve in 1 year so if seen early can reassure only camouflage - hairstyle, wig, toppik, scalp dye artificial eye lashes, eyeliner pencil, wear glasses to disguise loss Topical - potent TCS - tacrolimus - alone or as topical steroid sparer - minoxidil (esp less extensive cases) - dithranol 1% + 2% sal acid in YSP; apply 30mins initially inc to 2 hrs - DPCP immunotherapy (3rd line) systemic - oral minoxidil - steroid; esp if widespread inv. pred 25-40mg daily until regrown and reduce over 6-8 wks can use IV methyl pred or IM triamcinolone - Cyclosporin - simvastatin-ezetimibe (40/10mg); small JAAD study physicial; - ILCS - 2nd lne after topicals for small areas of AA; 2.5-5mg/ml; must wait at least 4 weeks before repeating, usually 6 - PUVA has been reported Surgery - usually not indicated
31
how is DPCP performed?
explain process to pt; 50% response rate; slow to see effects - pts should not touch scalp for 6 hrs after application - must avoid sunlight as degrades DPCP - CIs - pregnancy, blood dyscrazia, malignancy - AEs - severe erutpion, dyspigmentation, caregiver sensitization sensitize on scalp or upper inner arm w/ 2% DPCP in acetone on filter paper in Finn chamber for 48 hrs wait 1 week before starting immunotherapy some advocate treating half the scalp initially and add in other half when response confirmed start w/ 0.001% in acetone Titrate up conc weekly as tolerated to reach point of; erythema +scale and itch lasting 2-3 days after application 0.01, 0.025, 0.05, 0.1, 0.25, 0.5, 1.0, 2.0 start to see response by 12 weeks stop if no response at 24 wks when hair fully regrown taper off by reducing frequency of application
32
how many hairs are normally lost from the scalp each day?
50-100
33
T/F | 75% of cases of telogen effluvium are idiopathic
F | 30%
34
what are the causes of telogen effluvium?
Physiological shed - women post partum, newborns physical illness - shed some time after trigger high fever - eg dengue, malaria severe infection major surgery or GA chronic illness - HIV, SLE, SCLE, dermatomyositis endo - thyroid disease, chronic hyperparathyroidism nutritional deficiency eg iron, zinc, or massive weight loss, anorexia nervosa, bulimia, crash dieting Drugs - ABCD ROME chronic (idiopathic) TE
35
what are drug causes of telogen effluvium?
``` ABCD ROME ACEi Beta blockers anti-coags anti-depressants Retinoids OCP or stopping long term OCP Minoxidil (initial shed), anti-Micorbials (isoniazid, indanivir) Epileptics - valproate, phenytoin, carbamazepine ```
36
what are features of chronic TE?
usually women age 30-60 TE lasting over 6 months up to 30% get trichodynia Can be caused by any of the causes of TE esp drugs and chronic disease as trigger is ongoing Idiopathic if no cause found after investigation - diagnosis of exclusion
37
In what condition do you typically see short hairs regrowing at frontal hairline and parting?
resolving telogen effluvium
38
T/F | hair pull test is negative in TE
False usually highly positive 5-6 telogen hairs from each part of scalp as TE affects whole scalp
39
how many scalp hairs are in telogen in TE?
Over 20% is diagnostic usually less than 50% (normal is up to 10%)
40
what are trichoscopy features of TE?
Similar to PHL but affect whole scalp not just frontal and vertex some miniaturization peripilar sign increased proportion of follicular units producing only 1-2 hairs
41
what is management of TE?
decide if acute or chronic (>6 months) look for trigger in Hx +/- tests assess for other cause of hair loss e.g. unmasked PHL If isolated and acute w/ trigger reassure - resolves completely in 3-6 months If chronic must investigate for triggers can use topical or oral minoxidil treat any scalp disease or other types of hairloss camouflage usually not necessary as mainly an issue of shedding not thinning
42
T/F | hairs lost in alopecia areata are catagen hairs
F | dystrophic tapered anagen hairs
43
T/F | anagen effluvium usually causes more widespread hair loss than telogen effluvium
T thinning and often body affected not just scalp cf - TE usually noticed by shedding and not much thinning
44
what are causes of anagen effluvium?
``` Infection - tinea capitis, kerion - boil or abscess AI disease - pemphigus vulgaris Drugs; 3Cs and T-BAG; - Chemo - MTX, 5FU, Doxorubicin, Bleomycin - Colchicine - Cyclosporin - Thallium - Bismuth - Arsenic - Gold Radiation therapy Loose anagen syndrome ```
45
what is trichoscopy of chemo-induced hairloss/ anagen effluvium?
Black dots Exclamation mark hairs Monilethrx-like hairs
46
how is anagen effluvium managed?
Treat cause camouflage minoxidil For chemo - sometimes scalp cooling during chemo Rx can reduce haiross; hair grows back 3-6 months after end of chemo in most cases. Sometimes straight hair grows back curly
47
What is age and sex distribution for trichotillomania?
more common in kids; peak in young kids, 5-12, rarely in preschool age 2nd peak in adolesence and young adults - more likely to persist F:M 4:1 in older kids and adults, but in preschool boys more common than girls
48
what are the associations of trichotillomania in children, adolescents and adults?
Children - habits eg. nail biting, thumb sucking, nose picking, learning difficulty, anxiety, iron deficiency Adolescents - bullying, abuse, body image issues, anxiety or depression Adults - anxiety disorder, depression, OCD, eating disorder, substance abuse
49
what are DSM IV diagnostic criteria for trichotilomania?
1. recurrent pulling causing hairloss 2. tension when resisting and increase immediately before hair pulling 3. relief or please immediately after pulling hairs out 4. not part of another mental health disorder 5. interferes with normal social life or work etc
50
what is a hair growth window?
diagnostic technique used in trichotillomania | shave small patch of hair in site and rw in one week to see normal uniform regrowth
51
T/F | axillary hair is often involved in tricotillomania
F Uncommon site eyebrows and lashes common in children body hair common in adults
52
what are clinical findings in trichotillomania?
ask about tension before pulling and gratification afterwards ask about symptoms of associations dependent on age group - screen for anxiety, depression and suicidality in adolescents and adults and for bullying and sexual abuse in adolescents may be vertex, temporal, frontal or occipital or non-scalp sites often on side of dominant hand can extend in centrifugal or linear pattern may be secondary folliculitis always ask about GI symptoms and hallitosis as some have trichophagia and can get bezoar (ball of hair in stomach) Must assess impact on life Trichoscopy - broken hairs, hair powder, coiled hairs, split ends, flame hairs, V-sign, no major scalp/perifollicular changes
53
T/F | biopsy plays minimal role in investigation of trichotillomania
F Most useful investigation Features - normal size and number of follicles empty follicles with no inflammation pigment casts - boken off hairs leave small dark bodies some increase in telogen and end-catagen hairs may be follicular plugging or trichomalacia (deep distortion and curling of hair bulb)
54
How is trichotillomania managed?
Dont lay blame consider need for barium swallow etc if bezoar suspected Consider need for psych rw if suicidal, major anxiety, depression etc and for paeds admission/ child protection if abuse suspected need good Hx for causes to address esp in kids worth checking diet and testing for iron studies some kids just need to address causes of stress or discourage behaviour may need to refer to psych; TCAs can work SSRI may help but best if combined with behavioural therapy relaxation techniques hypnosis
55
T/F | pressure induced alopecia always resolves w/ time
F not always can be permanent or scarring loss
56
T/F | temporal triangular alopecia affects young adult men
F May be present at birth or onset in childhood, in 1st decade temporal hair lost and replaced by vellus hair follicle numbers the same persists lifelong
57
what is lipoedematous alopecia?
mainly affects women w/ dark skin thick boggy area of hairloss at vertex oedema and thickening of fat layer on path but no inflammation follicles replaced by fibrous tracts can be boggy scalp and path changes without hair loss
58
T/F | thyroid disease causes hair loss known as 'glades in the wood'
F | this is secondary syphylis - describes patchy hairloss
59
which systemic disease cause hairloss?
``` all typically telogen effluvium type except pempigus vulgaris which causes anagen effluvium; SLE - also causes 'lupus hair' SCLE Dermatomyositis thyroid disease esp hypo secondary syphylis iron deficiency zinc deficiency malnutrition or crash dieting ```
60
what is lupus hair?
dry coarse hair w/ non-scarring alopecia in SLE
61
what scalp skin diseases can cause hairloss?
Tinea capitis esp kerion - can be scarring psoriasis - can be scarring thick seb derm
62
what are trichoscopy findings to tinea capitis?
comma hairs corkscrew hairs - more tightly coiled comma hairs black dots - esp endothrix rare - morse code hairs can combine w/ Woods lamp for UV-enhanced trichosocpy
63
what are biphasic types of alopecia?
Non-scarring types that can become scarring if continue for long time E.g. AGA, AA, traction alopecia, pityriasis amiantacea also alopecia due to psoriasis or tinea capitis
64
what are secondary causes of scarring alopecia?
Not primarily a scarring alopecia disease but causes follicle detruction causes are trauma, sclerosing diseases, infection and malignancy; E.g. skin cancer, deep burn, XRT, sarcoidoisis, radiation dermatitis, scleroderma, linear morphoea (eg en coupe de sabre), cicatricial pemphigoid (scalp affected in 10%), NLD, infections e.g. TB, tinea capitis
65
which scarring alopecias are lymphocytic and which are neutrophilic?
``` Lymphocytic: DLE LPP pseudopelade of Brocq CCCA (neuts too if infected pustular type) alopecia mucinosa ``` Neutrophilic: FDC Dissecting cellulitis ``` Mixed: acne keloidalis nuchae erosive pustular dermatosis inflammatory tinea capitis acne necrotica ```
66
what genetic or congenital disorders can cause scarring alopecias?
``` aplasia cutis epidermal naevus hair follicle hamartoma incontinentia pigmenti Goltz syndrome porokeratosis of Mibeli Icthyosis EB Conradi-Hunerman bullous porphyrias ```
67
what are major categories of causes of scarring alopecia?
Primary scarring alopecias Biphasic alopecias secondary causes of scarring alopecias; Trauma, sclerosing, infections, neoplasms Genetic and congenital causes of scarring alopecia
68
what triggers erosive pustular dermatosis?
Surgery, cryotherapy, XRT, skin grafts on scalp most often in pts w/ existing advanced AGA
69
what are histo features of erosive pustular dermatosis
epidermal erosion chronic lymphocytic infiltrate; often plasma cells destruction of follicles often w/ surrounding fb granulomatous reaction
70
T/F | erosive pustular dermatosis is treated with potent steroids
``` T swab to exclude infection +/- biopsy to exclude IEC sun protection potent TCS w/ slow taper over months then mod TCS for control ```
71
T/F | central centrifugal cicatricial alopecia is triggered by hair relaxers or hot combs
T can be but continues even after pt has stopped using these
72
what are clinical and histo features of CCCA?
chronic progressive scarring alopecia esp in black women starts at crown or vertex and expands centrifugally and symmetrically - active inflammation at edge can be some polytrichia tufts in central scarred area can be infection and pustules at active edge mild pruritus or tenderness Histo lymphocytic infiltrate atrophy of follicle epithelium concentric lamellar fibrosis of follicles (onion peel) can be hair fragments and fb granulomatous reaction
73
How is CCCA treated?
stop using hot combs, relaxers etc Potent TCS + Doxy 1st line if pustules present start w/ 10 week course of rifampicin + clindamycin then Doxy and TCS Can need long term Rx
74
what are the types of LPP?
Classic FFA Graham-little syndrome
75
T/F | LPP is the most common scarring alopecia
T
76
How many LPP pts have LP elsewhere?
50% | can be skin, nails, genital/mucosal
77
T/F | FFA has been triggered by starting ustekinumab
F | has been triggered by starting TNFalpha blockers
78
what are the associations of LPP?
autoimmune disease esp hypothyroidism also reported; lupus, sjogrens, hep C do FBC, ELFT, TFTs and ANA
79
what is the course of progression of LPP?
usually affects several foci, can be linear course; violaceous perifollicular erythema follicular keratotic spines scarring loss of ostia and perifollicular scale lonely hairs - few single hairs isolated in scarred areas
80
T/F | LPP usually shows lichenoid change of follicles and overlying DEJ
F | interfollicular dermis spared and overlying DEJ very rarely involved
81
what are the features of Graham-Little syndrome?
KP LP - skin or mucosa LPP scarring alopecia Non scarring hair loss of axillary and pubic hair
82
what are the trichoscopy features of LP?
Intense perifollicular scale is most characteristic can be tubular scale structures migrating along hair shaft fibrotic white dots represent scarred ostia milky red fibrosis concentric linear bood vessels lonely hairs
83
what is histo of LPP
lichenoid infiltrate of follicle epithelium in region of isthmus and infundibulum - does NOT extend down to bulb usually no interfollicular and little or no DEJ inflamation perifollicular fibrosis can be cleft between folllicle and stroma
84
what is treatment ladder for LPP?
``` topical steroid - mainly help symptoms ILCS HCQ - main Rx after steroids Oral pred 30-40mg next best Rx Acitretin or isotretinoin Doxycycline MMF or CsA Pioglitazine Tacromlimus, Thalidomide, Pioglitazone rarely - MTX, TNFalpha blockers, Excimer laser ``` ``` Rx of FFA; TCS ILCS Short course oral pred HCQ Doxy Pioglitaone New - oral minoxidil + finasteride or dutasteride ```
85
T/F | sometimes DLE can mimic AA
T | less inflammatory types can mimic AA or morphoea
86
what are trichoscopy features of DLE?
Large yellow-brown dots are characteristic - dilated plugged ostia can be follicular red dots - good prognostic sign of regrowth thick arborizing vessels at peripheral edge of lesions end stage lesions show white fibrosis
87
How does histo of DLE differ from LPP?
DLE is mainly vacuolar type lichenoid w/ less florid lymphocytic infiltrate not confined to isthmus/infundibular region often chronic inflammation interfollicular dermis; may be plasma cells DEJ may be involved IMF shows granular IgG and C3 at junction of follicle epithelium and dermis and/or DEJ
88
T/F | pts wit DLE of scalp are more likely to develop SLE than if they have DLE elsewhere
F | very rare to get SLE if scalp DLE only
89
what is treatment ladder for DLE?
very similar to LPP dont forget sun protection steroids - topical or intralesional oral steroids sometimes used to induce remission HCQ - mainstay of Rx; usually remission in 3mnths If resistant; acitretin, dapsone, thalidomide (last line; CsA, MTX, MMF)
90
T/F | acne keloidalis 5x more common in black people
F | 10x more common
91
whats aetiology of acne keloidalis nuchae?
complex, poorly understood primary scarring alopecia not only due to ingrowing hairs may be a role for ingrowing hairs, staph and folliculitis w/ fb reaction similar to dissecting cellulits may co-exist w/ CCCA - can meet in middle
92
what are clinical features of acne keloidalis nuchae?
smooth follicular papules on neck area of hair resolve leaving small areas of alopecia coalesce into large firm keloidal plaques can be abscesses and sinus tracts
93
what is histo of acne keloidalis nuchae?
perifollicular chronic inflammation - lymphs, plasma cells, can be neuts, can be fb granulomatous rcn loss of sebaceous glands and follicles w/ lamellar fibroplasia can be extruded hair fragments may see abscesses and sinus tracts
94
what is Mx of acne keloidalis nuchae?
``` dont cut hair too short avoid rubbing collars swab for secondary infection Doxy is mainstay TCS can help settle inflamation ILCS esp if firm nodules sometimes oral pred to settle for large firm keloids - ILCS, Cryo, PDL, excision iostretinoin rarely - XRT or surgery followed by XRT ```
95
who gets dissecting cellulitis? | what is the cause?
young black men most often can be part of follicular occlusion tetrad cause known - thought to relate to obstruction of hair follicles and chronic infection genetic tendency
96
what are clinical features, trichoscopy and histology of dissecting cellulitis?
starts on occiput/vertex firm inflammatory nodules, superficial and deep abscesses connect into thick, boggy fluctuant mass w/ oval or linear ridges hair lost from summits of nodules/ridges but retained in valleys develop scarring can be a spondyloarthropathy - joint activity follows scalp trichoscopy shows 3D yellow dots superimposed over dystrophic hair shafts + yellow structureless areas - end stage shows white fribrosis Histo; Dense perifolliculitis of lymphocytes, histiocytes, neuts abscesses and sinus tracts destruction of follicles hair fragments with fb granulomatous rcn w/ plasma cells end stage is fibrosis and loss of appendages
97
what is management ladder for dissecting cellulitis?
swabs for bacteria and fungus and Rx as appropriate Rx similar to acne fulminans or pyoderma faciale; high dose pred 1mg/kg + EES then introduce isotretinoin and increase to 1mg/kg; then stop EES after 4 weeks iso and slowly wean pred and replace w/ TCS - can use ILCS if necessary. Continue iso for at least 6 mths and usually until 4 mths after skin settled then wean off Other options; Doxy zinc sulphate +/- oral and topical fucidic acid TNFalpha inhibitor CO2 laser surgery - incision and drainage - excision and grafting
98
what is folliculitis de calvans?
Very inflammatory scarring alopecia occurs in adults esp women in 30s May be some kind of staph hypersensitivity neutrophilic inflam infiltrate single area of enlarging scarring alopecia w/ inflamation and folliculr pustules + crusting at edge; Polytrichia is often seen - tufts of 5-20 shafts or more rare patterns; - multifocal - spread around margin of scalp - spread around margin of pre-existing baldness from AGA
99
what are histo features of FDC?
follicular abscesses with dense perifollicular infiltrate of neuts + some eos and plasma cells (does not have the thickness and sinus tracts of dissecting cellulitis) also fb granulomas, follicle destruction and fibrosis must do PAS to exclude inflammatory tinea capitis
100
what is management of FDC?
swabs +/- pluck hairs to r/o bacterial and fungal infection clindatech and TCS usually first line - add orals if not responding long course of antibiotics e.g. Doxy 1st line fluclox sometimes Rifampicin + clindamycin or sometimes rifampicin + fucidic acid/cipro/doxy isotretinoin - can induce LT remission in some pts Metvix PDT reported topical keratolytic can help tufting
101
what is tufted folliculitis?
The presence of a cicatricial alopecia with marked polytrichia some consider a variant of FDC w/ prominent tufting some consider as just the presence of prominent tufting that can be part of any scarring alopecia and can be seen in inflammatory tinea or pemphigus vulgaris Is not a complete diagnosis on its own
102
what is pseudopelade of Brocq?
Poorly defined entity - some say is a specific diagnosis but other don't believe that and see it as a pattern of scarring alopecia caused by another aetiology pelade means alopecia areata and pseudopelade means scarring alopecia Pseudopelade of Brocq is scarring alopecia in a patchy pattern usually called 'footprints in the snow' - later it can colaesce into larger areas this pattern affects mainly women in 40s areas are smooth, soft and may be slightly depressed on palpation - there is no discolouration or scale the hairs on the edge of the patches may be easily pulle dout if disease is active histo classically shows follicles replaced by fibrous streamers but no inflammation often it is slowly progressive essentially a diagnosis of exclusion No effective treatment if burnt out can consider surgery on scarred areas otherwise camouflage is mainstay
103
what are the inflammatory types of tinea capitis?
small spored ectothrix e.g. M. canis, M. equinum, M. audouiii agminate folliculitis e.g. M. canis, T. mentag mentag (zophilics) Kerion - esp T. mentag mentag, T verrucosum favus - T. schonleineii
104
what is agminate folliculitis?
type of inflammatory tinea w/ well-defined dull red paques studded w/ follicular papules caused by zoophilic dermatophytes
105
T/F | biopsy is rarely necessary for scarring alpecias
F | biopsy is essential - cannot distinguish without
106
Which stuctural hair abnormalities are associated with increased fragility?
``` Bubble hair Monilethrix Pili torti Trichorrhexis nodosa Trichorrhexis invaginata Trichothiodystrophy ```
107
which structural hair abnormality cannot be diagnosed by trichoscopy alone?
Trichothiodystrophy | Need polarised light microscopy
108
what is Bubble hair?
localised area of bubbles in hair shaft - hairs are brittle | hairs are short, uneven and kinky
109
What is monilethrix?
means necklace hairs - beaded appearance like a pearl necklace AD w/ variable expression mutation in keratins 81 or 86 normal hair at birth then after shed is replaced by short, brittle, kinky fair hair often have KP; can have koilonychia or other nail changes trichoscopy/mount shows nodes of fusiform thicker areas in between internode constrictions
110
What is Pili torti? | what are main associations?
Twisted hairs shaft is flattened and twists in groups of 3-10 can be genetic syndrome alone or part of larger syndrome e.g; Bjornstad's syndrome - AR w/ sensorineural deafness Menke's syndrome - X-linked copper transport Dx + get trichorrexis nodosa + in 40% of female Menke's carriers Can be acquired e.g. Anorexia nervosa, at edge of DLE or LPP lesions or in 'retinoid hair' due to aciretin or isotretinoin
111
What is trichorrhexis nodosa?
'No don't paint me!' Brush breaks in hair shafts like ends of paint brushes pushed together patches of short kinky broken hairs - not usually generalized Can be part of Nethertons Also congenital and acquired forms - congenital form accompanied by metabolic defects and mental retardation; can be seen in trichothiodystropy with icthyosis Acquired - due to straightening trauma etc, can occur in beard
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What is trichorrhexis invaginata?
Bamboo hair defect characteristic of Netherton's When hair breaks at defect golf-tee shaped socket end remains - scalp, eyebrows and lashes affected affected in Nethertons - hair doesnt grow long in infants; scalp hair improves in adults but remains affected elsewhere; most easy to identify in eyebrow hairs
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What hair defects are seen in trichothiodystrophy?
Literally means sulphur-deficient hair Pts usually have intellectual impairment and may have photosensitivity Hair is patchy and brittle most characteristic feature is tiger tail hair - alternate light and dark bands only seen on polarised light microscopy Can also get - trichoschisis (clean transverse fractures), distal trichorrhexis nodosa, pili torti
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What is loose anagen syndrome?
Familial disease w/ faulty cornification of inner root sheath - hair is poorly anchored into follicle children esp girls have short, blonde hair +ve hair pull test - loose anagen hairs are characteristic; roughened proximal cuticle - looks like ruffle sock on a hockey stick
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What is pili annulati?
shiny hair due to bright and dark regions in hairs under reflected light - lighter areas are due to abnormal air-filled cavities in the hair AD or sporadic trait On microscopy the light areas are dark regions
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What is Pili trianguli et canaliculi?
Uncombable hair or Spun glass hair hair is triangular in cross section - has longitudinal grooves visible on EM looks like spun glass when it reflects light is stiff and hard to comb and usually silvery colour, presents in childhood improves in adulthood but defect still visible on microscopy
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what is woolly hair?
tightly curled hair sometimes w/ various microscopic features e.g. elliptical cross section, axial twisting, trichorrhexis nodosa can be all over scalp as AD or AR trait can be localised patch = woolly hair naevus can be part of 3 AR syndromes w/ cardiomyopathy; - Naxos disease - Carvajal syndrome - EBS-desmoplakin; skin fragility woolly hair syndrome Also seen in Noonan's or cardio-facio-cutaneous syndrome Can be retinal defects in pts w/ Woolly hair naevus - refer to ophthal and to gen paeds in kids as risk of precocious puberty
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what is trichoschisis?
clean transverse fracture in hair shaft | can occur in trichothiodystrophy
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what is trichoclasis?
Greenstick fracture of hair | can occur in trauma
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what is trichoptilosis?
split ends | occur due to trauma
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What are circle hairs?
circle or spiral hairs trapped under stratum corneum layer
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What is trichomalacia?
has different clinical and histo definition; clinical - softened hair residue and keratin in an open follicular orifice in the scalp histo - deep distortion and curling of hair bulb
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What is a Pohl-pinkus constriction?
a zone of constriction in a hair shaft due to a phase of poor growth similar to beaus's line - occurs due to illness etc
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T/F | new anagen hairs have blunt ends
F have pointed ends blunt ends seen in broken hairs
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What is trichonodosis?
knots in the hair | happen in very curly hair e.g. afro-caribbeans
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What is bayonet hair?
slight kink in the hair within 1-2mm of the tip | normal variant
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What are hair casts?
AKA pseudoknits keratin cylinders at base of hair shaft - arise from upper part of inner root sheath seen in scaly scalp conditons or styles with tension on hair
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``` T/F the following cause nails to grow faster; Young age male sex Summer time nails on right hand pregnancy hyperhtyroidism azathioprene ```
``` F all true except AZA AZA and MTX and L-Dopa slow nail growth Itraconazole speeds up nail growth Acitretin can speed up nail growth in Pso pts but otherwise slows it down ```
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What are Beau's lines? | what are causes?
transverse depressions in nail due to period of reduced growth causes of Beau's lines and onychomadesis are the same e.g. severe illness, erythroderma, dialysis, immunodeficiency derm Dx affecting nails eg. BP, PV, linear IgA, paronychia, SJS/TEN, nail pso, pustular pso, LP, Raynauds drugs esp chemo also retinoids, penicillin, azithromycin, lithium, lead antiepileptics
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What is onychomadesis? | what are causes?
``` detachment of nail plate from prox nail fold - more severe form of Beau's line - nail still attached to bed but falls off after some time due to complete arrest of nail matrix activity for a time all same causes as Beau's lines also some specific associations; alopecia areata Kawasaki disease MF infections eg. hand, foot + mouth, varicella, candida, dermatophyte, fusarium, meningitis Other types; familial idiopathic sporadic onychomadesis seasonal onychomadesis ```
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What is retronychia? | what are causes?
proximal ingrowing of nail into ventral surface of nailfold similar to beau's and onychomadesis but directon of nail growth altered by insult caused by trauma, OA or thrombophlebitis can diagnose on USS in pt w/ chronic proximal paronychia or abnormal onychomadesis avulse nail - usually grows back normally
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what are causes of nail pitting?
``` focal deep large pits; Psoriasis, Reactive arthritis, trauma diffuse shallow pits; alopecia areata (geometric), SLE, Eczema can be seen w/ other changes in tinea unguim ```
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what is onychorrhexis? | what are causes?
thin brittle nails w/ superficial grooves + can be distal splitting e.g. Old age, LP, RA, Raynaud's, Darier's, tumour compressing matrix
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What causes longitudinal grooves in nails?
habit tic - pushing back cuticles or onychotillomania subungual tumour etc e.g. glomus, myxoid cyst, verruca, SCC, melanoma, pterygium Median canaliform dystrophy of Heller = wide deep central split with inverted fir tree like appearance - spares cuticle - several causes; familial, trauma, retinoids
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What is trachyonychia? | what are causes?
rough surface to nails like sandpaper - due to many tiny pits causes; Pso, alopecia areata, eczema, LP retinoids, trauma 20 nail dsytrophy - idiopathic widespread variant
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what is 20 nail dsytrophy?
d
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what is koilonychia? | what are causes?
spoon-shaped nails with concave curvature in transverse and longitudinal directions is normal in infancy esp thumb and big toe - self resolves familial AD trait is most common cause of koilonychia starting at young age and persisting into adulthood (nail disorder, non-syndromic congenital 2) other causes; iron deficiency, haemochromatosis, trauma can be seen in pts w/ trichothiodystrophy or monilethrix
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what is a pterygium? | what are types and causes?
Dorsal pterygium is most common = scarring between nail fold and matrix resulting in no nail growth from part of the matrix - instead skin grows out from prox nailfold onto nail bed in a triangular appearance e.g. LP also sarcoidosis, leprosy Ventral pterygium is when the nail bed epithelium extend forward disrupting the hyponychium e.g. scleroderma also Raynauds, LE, trauma, familial, infection
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what is onycholysis? | what are causes?
detachment of distal nail plate from bed e.g. trauma/toxic - wet work, irritants, traumatic nail picking Pso, LP, dermaitis, bullous dermatoses, onychomycosis hyperthryroidism, PCT photo-onycholysis - psoralens, doxycycline, NSAIDs, amioderone etc tumour of nail bed AR hereditary distal onycholysis
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what is onychochuxis? | what are causes?
``` = subungual hyperkeratosis e.g. Pso PRP onychomycosis eczema ```
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what are causes of splinter haemorrhages?
trauma - distal and longitudinal Pso onychomycosis systemic (usually proximal) e.g. infective endocarditis, vasculitis, SLE, antiphospholipid syndrome
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What is apparent leukonychia? | what are causes?
Apparent leukonychia - Generalised e.g. anaemia, oedema, vascular impairment of nail bed - nail bed anomoly causing white appearance that disappears w/ pressure - Terry's nails; White proximally w/ distal 20% normal/red/brown e.g. cirrhosis, CCF, T2DM - Half and half nails; white proximally w/ distal 50% brown e.g. CRF, chemotherapy - Muehercke's nails (striate leukonychia); Transverse white lines parallel to lunula e.g. SLE, low albumin, nephrotic syndrome
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How is Leukonychia classified?
True leukonychia - disturbed keratinizaton causes parakeratosis in nail plate resulting in loss of translucency and white appearance that doesnt disappear with pressure - Punctate - Longitudinal - Transverse = Mee's lines - Complete = nail disorder, non-syndromic congenital 3 Apparent leukonychia - nail bed anomoly causing white appearance that disappears w/ pressure - Generalised - Terry's nails - Half and half nails - Muehercke's nails (striate leukonychia) Pseudo-leukonychia - white discolouration other than those above
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what is pseudo-leukonychia? | what causes it?
Pseudo-leukonychia - white discolouration not cased by nail bed parakeratosis or nail bed anomoly e. g. white superficial onychomycosis
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What is true leukonychia? | what are causes?
True leukonychia - disturbed keratinizaton causes parakeratosis in nail plate resulting in loss of translucency and white appearance that doesnt disappear with pressure - Punctate e.g. Pso, alopecia areata - Longitudinal e.g. Dariers, Hailey-Hailey - Transverse = Mee's lines; truly transverse across nail and do not curve e.g. trauma, chemo, renal failure, heavy metal poisoning - Complete = nail disorder, non-syndromic congenital 3; AD/AR - PLCD1 gene muations
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what causes erythronychia?
anything that causes thinning of the nail plate makes the nail look more red Can be any type of skin tumour or tumour arising from adjacent bone or cartilage disrupting the matrix also local skin Dx e.g. Dariers, AKV of Hopf, LP, Pso, DLE, Langerhans cell histiocytosis
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what are causes of longitudinal melanonychia?
activation of nail matrix melanocytes - usually several nails melanoctyic lesions - naevus, lentigo, melanoma triggers for nail matrix melanocyte activation - Racial melanonychia (often increases w/ age) Pregnancy Addison's PIH after local dermatosis e.g. LP, Pso, onychomycosis Inherited pigmentary disorders e.g. Laugier-Hunziker, Peutz-Jehger Trauma - esp lateral 2 toenails Drugs - tetracyclines, hydroxyurea, chemo (5FU, Doxorubicin), cyclophosphamide, psoralens, zidovudine
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What features are concerning when assessing longitudinal melanonychia?
Single affected nail Age - adults over 40 brown-black w. different colours/ variegated borders 3mm or wider in diameter Hutchinson's sign - pigment extends to proximal and/or lateral nail fold rapidly changing esp if band widening Hx - any high risk melanoma features in history e.g. one or more melanomas in past
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What are the causes of longitudinal melanonychia in children? when should you biopsy?
``` Most due to subungual lentigo some are subungual naevus or atypical naevus Dermoscopy NOT useful can usually manage conservatively Think to biopsy if (same as for adults really); multicoloured Hutchinson's sign pigment band 3mm or more ```
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What is green nail syndrome? what causes the green colour? how is it treated?
Due to infection with pseudomonas aeruginosa usually starts as onycholysis or chronic paronychia which allow pseudomonas to get in usually only 1 or 2 nails nail looks green due to accumulation of pyocyanin under the nail plate Rx 1:4 white vinegar soaks BD gentamycin identify and treat pre-existing nail disease
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what are the genetic disease causing complete or partial absence of the nails?
Complete absence of nails - Nail disorder non-syndromic congenital type 4 - AR - mutation in RPO4 (R-spondin 4) - Wnt signalling pathway Partial absence of nails - Nail disorder non-syndromic congenital type 6
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What is the ABCDEF list for suspected nail melanoma?
Age - peak in age 40-60s Brown-Black, Breadth 3mm or more, Borders variegated Changing colour or breadth / failed to change after Rx Digits most often affected - thumb and big toe Extension of pigment to nailfold Family or personal Hx of MM or dysplastic naevi
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what is onychoschizia? what are causes? what is management?
Distal free edge of nail plate splits horizontally into multiple plate-like layers causes; chronic trauma, wet work, picking etc Sometimes due to poor nutrition, iron deficiency etc - if only fingernails affected less likely systemic cause Mx reduce trauma and wet work wear cloths moisturize keep nails short avoid acrylics and other nail cosmetics avoid nail affecting drugs such as retinoids check iron, zinc and albumin levels - discuss diet biotin 5-10mg/day may help - trial for 2-3 months
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what causes chronic paronychia? | How is it treated?
Inflammation of prox and/or lateral nail fold - red, swollen, cuticle usually lost chronic course w/ periodic exacerbation often sec infection w/ candida or pseudomonas Causes; Irritation eg wet work and trauma contact dermatitis ICD or ACD food hypersensitivity (allergy) eg limes, garlic ACD Candida hypersensitivity True primary chronic candidal paronychia - rare; can be in HIV or chronic mucocutaeous candidiasis eg immunodeficient neoplastic - due to adjacent SCC, IEC etc Drug-induced - chemo, retinoids, antiretrovirals Mx hand care; avoid trauma and wet work, wear gloves for work w/ hands no nail cometics while settling then avoid excessive manicuring etc swabs fro baceria, fungus, yeast consider need or biopsy or imaging topical TCS or tacro BD topical nizoral cream BD topical antiseptic soak eg. vinegar, Condys severe cases may need ILCS (2.5mg/ml monthly) or short course oral prednisolone Usually oral antifungals not needed but may do if candida confirmed
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What is acute paronychia? | how is it managed?
In acute paronychia there is rapid onset erythema, swelling, heat and pain and often a pointing asbcess Causes; picking or biting nails, thumb sucking, manicures or other traumas, ingrown nails, drugs - retinoids, EGFR inhibitors, BRAF inhibitors Mx can be bacterial or viral - must swab (rarely fungal) Often need incision and drainage topical antibiotics or antiseptics sometimes need oral antibiotics or antivirals
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What is onychocryptosis? | what is management?
Ingrown nails usually toe nails esp big toe usually lateral nailfold can occur in pts w/ congenital malalignment of the great toenails can be triggered by improper nail cutting, or pointed shoes or other trauma to the nail retinoids can trigger higher risk if hyperhydrosis Mx removal of edge of nail uplifting lateral nail plate w/ cotton or dental floss after warm water soaks several times each day destruction of lateral nail matrix e.g. surgery, laser, phenol can be hypertrophy of lateral nail fold which may need surgery
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what is onychogryphosis? | what are causes and management?
Thick yellow nail like a rams horn ageing, psoriasis, trauma nail avulsion is treatment of choice
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What causes pincer nails? | what is management?
hereditary or acquired toenails usually affected, often painful excess transverse curvature causes compression of distal nail bed causes; Inherited - thumbs and big toes, onset in adulthood; other nails involved later but usually less so psoriasis Individual pincer nail - MRI to investigate for tumour of matrix
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T/F | periungual fibromas are rare in the general population
F common But if multiple consider TSC (Koenen's tumours)
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What is management of digital myxoid cyst?
Cryotherapy 1st line - empty of gel then apply pre-frozen probe for 4-5 secs if unsuccessful refer to hand surgeon for definitive management some suggest - repeated drainage, sclerotherapy, steroid injection but often recur
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What is subungual exostosis?
bony outgrowth of terminal phalanx that forms a firm lump and can lift the nail plate
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How do glomus tumours present? | how is it managed?
painful blue-red macule beneath nail Can present w/ longitudinal groove, longitudinal erythronychia, pincer nail etc Get MRI to assess extent and refer to hand surgeon
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What is Onychomatricoma?
fibroma of the proximal nail fold, closely associated with the matrix causes focal or complete thickening of nail plate mimicking subungual hyperkeratosis with multiple holes in thickened free margin representing tunnels of tumour
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T/F | periungual SCC is associated with HPV
T esp HPV16 also 18 and 34
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what is treatment for periungual SCC, KA or IEC?
``` SCC- X-ray to rule out bony involvement Moh’s micrographic surgery Local excision Digit amputation XRT - rarely need ampuation for digital IEC but complete excision recommended - non-surgical methids not advisable (PDT, efudix etc) - KAs can invade phalanges and dont regress as they do elsewhere; treat aggressively like SCC ```
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T/F | Psoriasis can cause almost every possible nail change
T
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What are the main nail changes of psoriasis?
Irregular large deep pits Salmon patches (‘oil drop’ sign) – due to focal parakeratosis of nail bed and lifting of nail Onycholysis w splinter hemorrhages, subungual hyperkeratosis, nail plate thickening and crumbling, paronychia
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What is treatment ladder for psoriatic nail disease?
JAMA Derm rw 2015: General; Avoid trauma/manicures, keep nails short, emollient, gloves for wet work Evaluate for PsA – refer to rheum if appropriate Consider extent of Pso – do they qualify for systemic/biologic based on skin severity Exclude onychomycosis Rx; Topicals first line; - Potent TCS (clobetasol in studies) - Daivobet gel ILCS – 1st line non systemic if failed topicals MTX – 1st line systemic if cant get biologic Acitretin – 2nd line non biologic systemic Biologics – adalimumab>etanercept (then ILCS)>ustekinumab (But nails alone don’t qualify on PBS) If skin and Nails bad; 1st line – adalimumab>etanercept>ustekinumab 2nd line – MTX>Acitretin> infliximab>apremilast If skin, nails and joint disease; Adalimumab>etanercept>ustekinumab>infliximab> MTX>apremilast>golimumab
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T/F | Acrodermatitis continua of Hallopeau arises in pts with plaque psoriasis
F | considered a variant of pustular Pso but doesnt usually arise in pts w/ Pso vulgaris
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what are features of Acrodermatitis continua of Hallopeau?
Relapsing episodes of acute painful inflammation with pustules around and under nail plate Others: onycholysis, onychomadesis, and scaling of the nail bed and periungual skin
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What is management of Acrodermatitis continua of Hallopeau?
``` May need biopsy to confirm diagnosis Potent TCS (clobetasol, dip OV) Daivonex/ Daivobet Tacrolimus Efudix reported Acitretin in severe cases MTX TNFs – but can trigger pustular pso PUVA - topical ```
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T/F | 10% of cases of LP involve the nails
T | esp seen in pts over 40
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T/F | LP of the nails is commonly associated with LP elsewhere
F | often isolated nail disease
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What are features of lichen planus of nails?
Nail thinning esp lateral edges Ridging Longitudinal Fissuring or distal splitting Dorsal pterygium Others; onycholysis, subungual hyperkeratosis, yellow discoloration, loss of nail
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How is nail LP managed?
Systemic therapies may be required to avoid pterygium formation – pred, retinoids Pre-Rx biopsy of matrix is ideal to confirm diagnosis Resistant to TCS Topical tacro may work If only 1 or few nails ILCS into prox nailfold – 2.5-5mg/ml Systemic treatment usually required – pred 1st line; 25mg OD for 4 wks then taper over 2-4 weeks – can induce long term remission but recurrence is common Alitretinoin reported (not in Aus) Etanercept reported
176
What is cause of 20 nail dystrophy?
unknown | some cases may be lichen planus - seen in children however LP of nails is otherwise rare in kids
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What is 20 nail dystrophy? | How is it managed?
Widespread trachyonychia involving majority of nails Mostly occurs in childhood Cause unknown but some cases AD (gene unknown) May have LP, Pso, eczema, alopecia areata can be thinning, thickening, pitting, ridging, koilonychias, opalescence or loss of lustre or some nails may be spared No good Rx; check for associations topical, IL and systemic steroids used Can try topical PUVA
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What nail changes may be seen in pts w/ eczema?
Irregular pitting and beau’s lines Subungual hyperkeratosis Chronic paronychia
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T/F | Dermatomyositis pts get Dilated nailfold capillaries and Drop-out of capillaries as well as ragged cuticles
T
180
What is Lovibonds profile sign?
Feature of digitial clubbing | Lovibond’s angle between proximal nail fold and nail plate (normally 180˚
181
What is Curths’ modified profile sign?
Feature of digitial clubbing Curth’s angle of distal phalanx relative to the more proximal phalanx across the DIPJ is normally about 180˚ and is reduced to
182
What is Schamroth’s sign?
Feature of digitial clubbing | Closure of Schamroth’s window
183
What is Shell nail syndrome?
rare variant of clubbing associated w/ bronchiectasis; nail looks similar but distal nail bed is atrophic and distal nail curves over an empty space rather than soft tissue
184
T/F | Clubbing occurs due to enlargement of the soft tissue pulp of distal digit
T Increased longitudinal and transverse nail curvature cause unknown but may be due to altered vagal tone or microvascular infarcts
185
What are the causes of digital clubbing?
Lung disease: Lung cancer, mainly non-small-cell (54% of all NSCLC) Interstitial lung disease most commonly fibrosing alveolitis Tuberculosis Suppurative lung disease: lung abscess, empyema, bronchiectasis, cystic fibrosis Mesothelioma of the pleura Arteriovenous fistula or malformation Heart disease: Any disease featuring chronic hypoxia Congenital cyanotic heart disease (most common cardiac cause) Subacute bacterial endocarditis Atrial myxoma (benign tumour) inc Carney complex Tetralogy of Fallot (Di George) Gastrointestinal and hepatobiliary: Malabsorption Crohn's disease and ulcerative colitis Cirrhosis, especially in primary biliary cirrhosis Hepatopulmonary syndrome, a complication of cirrhosis Others: Hereditary nail clubbing; mutations in HPGD gene SLE Complex regional pain syndrome Pachydermoperiostosis Seen in hemiplegic limbs HIV If single digit think of subungual tumour Hypertrophic (pulmonary) osteoarthropathy: Thyroid acropachy
186
What should you look for in pts with yellow nails?
If all yellow think of Yellow nail syndrome; Triad; Yellow nails, Lung pathology, Lymphoedema; Presents in 30s-50s All 20 nails thick and yellow Lower limb lymphoedema Lungs - chronic bronchitis, bronchiectasis, sinusitis, pleural effusions Can be RA, immunodeficiency or internal malignancies Do full work up including ABPI, chest imaging, RF, blds and malignancy screen Yellow colour is due to thickening but there may also be chronic infection (pseudomonas) - nails dont grow out but get thicker instead; cuticle is lost, can be onycholysis, onychomadesis Rx - difficult • Vitamin E 1200 IU/day or topical cream • Pulse itraconazole
187
What nail changes are seen in HIV?
``` Clubbing Transverse lines Onychoshcizia Leukonychia Longitudinal melanoychia Infection: dermatophytes, yeasts, HPV, HSV, crusted scabies ```
188
What is the likely cause of a single clubbed digit
subungual tumour
189
What is herpetic whitlow?
Primary inoculation of HSV into periungual fingertip region Important type of acute paronychia Clear at first the blisters soon become purulent, may rupture and crust Very painful and may take 3 weeks to resolve antivirals help to settle
190
Who is at risk of periungual warts?
People who bite their nails or suck thumb
191
What are the treatments for warts on fingertips
``` Exclude SCC Stop nail biting, thumb sucking etc Do nothing LN cryotherapy including 2mm margin for 5secs repeated 2-3 week intervals Topical cantharidin Topical immunotherapy with diphencyprone (DCP) Keratolytics (Salicylic acid, lactic acid) under occlusion Silver nitrate Duct tape occlusion 5-FU Imiquimod Ablative laser CO2 or Erbium:YAG Pulsed dye laser C+C Bleomycin PDT ```
192
Which hair loss pts need a biopsy?
scarring alopecias non-responding AA - to exclude lupus panniculitis and metastatic breast cancer diffuse AA moth eaten alopecia
193
T/F | The outermost active part of a scarring alopecia provides the most informative histo
F biopsy any area that has been present and active for at least 4 months and still has erythema - avoid the outer most inflammatory edge as less diagnostic and the inner as scar only
194
What causes someone to turn 'white overnight'?
Alopecia areata in greying hair - pigmented hairs are lost as it is a reaction to the pigment. White hairs spared
195
Why must you cut the hair and examine the scalp in acquired poliosis?
Can be melanoma with immune reaction to pigment - just like a halo naevus in an older person
196
What is DD for patchy hairloss?
Alopecia areata Tinea capitis Trichotillomania Syphylis