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63
Alopecia Antonella Tosti Fredric Brandt Endowed Professor of Dermatology&Cutaneous Surgery Miller School of Medicine, University of Miami
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Alopecia

Antonella TostiFredric Brandt Endowed Professor of

Dermatology&Cutaneous Surgery Miller School of Medicine, University of Miami

Antonella Tosti, MDS049 Alopecia

DISCLOSURES

Fotofinder :Consultant, Springer & Verlag , CRC Press :Author-Royalties , Karger : Editor in chief

DISCLOSURE OF RELATIONSHIPS WITH INDUSTRY

Most important challenges

1 Clinico/pathological correlations

2 Utilize dermoscopy to select optimal biopsy site

3 Distinguish early scarring alopecias from MPHL/FPHL

4 Pitfalls

Most important challenges

1 Lack of clinico/pathological correlations

The clinician is convinced that the patient has scarring alopecia

Pathologist signs as non scarring alopecia

1 Lack of clinico/pathological correlations

Most common reasons

Site of biopsy

Clinicians often decide to take the biopsy at the periphery of the patch as this is where the disease is active and it is more likely to obtain a pathological diagnosis

1 Lack of clinico/pathological correlations

Most common reasons

Site of biopsy

This site might not be affected and pathology shows no scarring

1 Lack of clinico/pathological correlations

How to deal?

Look at the problem together!

1)Take a new biopsy in the scarring area, as patient otherwise gets confused

2) Use dermoscopy to see where the disease is active at periphery and in this case take a dermoscopy guided biopsy

1 Lack of clinico/pathological correlations

How to deal?

Look at the problem together!

2) Use dermoscopy to see where the disease is active at periphery and in this case take a dermoscopy guided biopsy

1 Lack of clinico/pathological correlations

Different situation

In this case the clinician is unsure if this is scarring or non scarring: it is very important to take the biopsy at the center of the patch!

1 Lack of clinico/pathological correlations

Transverse

Vertical

Most common reasons

Specimen processing

1 Lack of clinico/pathological correlations

Best approach

If the clinician provides two biopsies process one for horizontal and one for vertical sections

If the clinician provides one biopsy process for horizontal sections

Childs JM, Sperling LC. Histopathology of scarring and nonscarring hair loss.Dermatol Clin. 2013 Jan;31(1):43-56.

1 Lack of clinico/pathological correlationsBest approach

Nguyen JV, Hudacek K, Whitten JA, Rubin AI, Seykora JT. The HoVert technique:a novel method for the sectioning of alopecia biopsies. J Cutan Pathol. 2011May;38(5):401-6.

2 Utilize dermoscopy to select optimal biopsy site

Use the dermatoscope to select the biopsy site!

Area to select depends on disease

Miteva M, Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia. J Eur Acad Dermatol Venereol. 2013 Oct;27(10):1299-303.

-DermLite® (3Gen LLC.)

-Handyscope® (FotoFinderSystems)

-DermScope ® (Canfield Imaging Systems)

2 Utilize dermoscopy to select optimal biopsy site

Instruments

2 Utilize dermoscopy to select optimal biopsy siteArea to select depends on clinical diagnosis and dermoscopic features

Select the area with dermoscopy

Mark and circle the area

Confirm selection with a dermoscopic picture

2 Utilize dermoscopy to select optimal biopsy siteDermoscopic features associated with disease activity in scarring alopecias.

Peripilar castsHair tuftingKeratotic plugsWhite gray halos

2 Utilize dermoscopy to select optimal biopsy site

Peripilar casts

White concentric scales surrounding the hair shaft at its emergency

2 Utilize dermoscopy to select optimal biopsy siteHair tufting

Tuft of 2 or more hairs surrounded by casts

2 Utilize dermoscopy to select optimal biopsy siteKeratotic plugs

Keratotic masses filling the follicular openings

2 Utilize dermoscopy to select optimal biopsy siteWhite gray halos

White gray dots surrounding a tuft of 2 hais

2 Utilize dermoscopy to select optimal biopsy site

Lichen planopilaris: tufted hairs with peripilar casts

Frontal fibrosing alopecia: terminal hairs with peripilar casts

Discoid lupus erythematosus: keratotic plugs, red dots

Folliculitis decalvans: tufts of six or more hairs emerging together

Central centrifugal cicatricial alopecia: white-gray halos

Site of Biopsy in Scarring Alopecias

Dermoscopy guided biopsy

•Increases pathological accuracy(diagnosis in 95% of biopsies)

•Very helpful in cases of early or focal disease

•Useful for dermoscopic-pathological correlations

2 Utilize dermoscopy to select optimal biopsy site

3 Distinguish early scarring alopecias from FPHL/MPHL

Frontal fibrosing alopecia

Fibrosing alopecia with a pattern distribution

Important mimics of MPHL/FPHL

Frontal fibrosing alopecia

Frequency is increasing world wideNot limited to postmenopausal womenCommonly associated with androgenetic alopeciaEarly cases can be difficult to detects

3 Distinguish early scarring alopecias from FPHL/MPHL

Frontal hairline recession Loss of eyebrows Prominent temporal/frontal veinsHair loss in the limbsFacial lesions

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Clinical features

Mean glabellar–frontal distance : 8,5 cm ( controls 5.9 cm)

The alopecic area shows less signs of photodamage as compared with the forehead

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Lencastre A, Tosti A. Images in clinical medicine. A receding hairline. N Engl J Med. 2013 Jul 11;369(2):e2.

Frontal hairline recession

Lonely hair : a clue to diagnose Frontal Fibrosing Alopecia

Presence of one or few isolated remaining terminal hair in the middle of the forehead, at site of the original hairline implantation is a clinical clue for diagnosis of FFA

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Frontal hairline recession

Tosti A, Miteva M, Torres F. Lonely hair: a clue to the diagnosis of frontalfibrosing alopecia. ArchDermatol. 2011 Oct;147(10):1240

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Frontal hairline recession

Perifollicular erythema and scaling

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Clinical features

Loss of eyebrows (75% of patients

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Clinical features

Prominent temporal/frontal veins

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Very common in Europe, Americas, Africa, few cases reported from China, rare in South Arabia

Role of sunscreens

Frequence is increasing world wide

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Also seen in young women and men

Not limited to postmenopausal women

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Increasingly reported in men1: Tolkachjov SN, Chaudhry HM, Camilleri MJ, Torgerson RR. Frontal fibrosing alopecia among men: A clinicopathologic study of 7 cases. J Am Acad Dermatol. 2017 Jul 14.

2: Ormaechea-Pérez N, López-Pestaña A, Zubizarreta-Salvador et al . Frontal Fibrosing Alopecia in Men: Presentations in 12 Cases and a Review of the Literature. Actas Dermosifiliogr. 2016 Dec;107(10):836-844

3: White F, Callahan S, Kim RH, et al Frontal fibrosing alopecia in a 46-year-old man. Dermatol Online J. 2016 Dec 15;22(12).

4: Salido-Vallejo R, Garnacho-Saucedo G, Moreno-Gimenez JC, Camacho-Martinez FM. Beard involvement in a man with frontal fibrosing alopecia. Indian J Dermatol Venereol Leprol. 2014 Nov-Dec;80(6):542-4.

5: Khan S, Fenton DA, Stefanato CM. Frontal fibrosing alopecia and lupus overlap in a man: guilt by association? Int J Trichology. 2013 Oct;5(4):217-9.

6: Chen W, Kigitsidou E, Prucha H, Ring J, Andres C. Male frontal fibrosing alopecia with generalised hair loss. Australas J Dermatol. 2014 May;55(2):e37-9.

7: Debroy Kidambi A, Dobson K, Holmes S et al . Frontal fibrosing alopecia in men: an association with facial moisturizers and sunscreens. Br JDermatol. 2017 Jul;177(1):260-261.

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Increasingly reported in men

Can start from sideburns

Beard and body hair commonly involved

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Commonly associated with androgenetic alopecia

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Look at the hairline of all women consulting for hair loss!

Parietal hairline often first site of involvement

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Tips to recognize early FFA

Suspect FFA in all patients showing sparse/tattooed eyebrows

Be aware of facial lesions!

Look for presence/absence of vellus hair at the hairline

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Tips to recognize early FFASuspect FFA in all patients showing sparse/tattooed eyebrows

Anzai A, Donati A, Valente NY, Romiti R, Tosti A. Isolated eyebrow loss in frontal fibrosing alopecia: relevance of early diagnosis and treatment. Br J Dermatol. 2016 May 13

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Tips to recognize early FFA

Facial papulesKeratosis pilaris like lesionsFacial erythemaFacial maculesFacial hyperpigmentation

Be aware of facial lesions!

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Facial papulesMore common in women with dark phototypes

Forehead Temples ChecksChin

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Facial papulesLichenoid inflammation involving vellus hair follicles and perifollicular fibrosis

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Facial papules

Pinkus acid orcein staining showing reduction and fragmentation of elastic fibers .

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Facial papules

We propose that an abnormal elastic framework could be responsible for the remodeling of the shape of sebaceous lobules and ducts in this anatomic microenvironment, leading to the popping out of sebaceous glands and the clinical formation of FP

Pirmez R, Barreto T, Duque-Estrada B, Quintella DC, Cuzzi T. Histopathology offacial papules in frontal fibrosing alopecia and therapeutic response to oralisotretinoin. J Am Acad Dermatol. 2018 Feb;78(2):e45.

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Facial papules

Pedrosa AF, Duarte AF, Haneke E, Correia O. Yellow facial papules associatedwith frontal fibrosing alopecia: A distinct histologic pattern and response toisotretinoin. J Am Acad Dermatol. 2017 Oct;77(4):764-766.

Frontal fibrosing alopecia

3 Distinguish early scarring alopecias from FPHL/MPHL

Other facial lesions

In dark phototypes easily confused with melasma

In fair phototypes easily confused with rosacea

Frontal fibrosing alopecia

Look at hairline for presence/absence of vellus hair

3 Distinguish early scarring alopecias from FPHL/MPHL

Tips to recognize early FFA

You need a dermatoscope!

3 Distinguish early scarring alopecias from FPHL/MPHL

FFA increasingly commonNot limited to postmenopausal womenYou might get eyebrow biopsiesYou might get biopsies of facial lesions

Take home message

3 Distinguish early scarring alopecias from FPHL/MPHL

26 year old man with patterned alopecia and scalp itching

3 Distinguish early scarring alopecias from FPHL/MPHL

Dry dermoscopy

Hair shaft variability

Peripilar casts

Hair tufting

V sign

First described by Zinkernagel &Trueb in 2011

Pathology :miniaturization (as in androgenetic alopecia) and lichenoid perifollicular inflammation

Fibrosing alopecia with a pattern distribution

Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch Dermatol. 2000 Feb;136(2):205-11.

3 Distinguish early scarring alopecias from FPHL/MPHL

Fibrosing alopecia with a pattern distribution

Diagnosis :

Need to take dermoscopy guided biopsy !

3 Distinguish early scarring alopecias from FPHL/MPHL

Fibrosing alopecia with a pattern distribution

Diagnosis pathology :

Need horizontal sections!

3 Distinguish early scarring alopecias from FPHL/MPHL

Fibrosing alopecia with a pattern distribution vs lichen planopilaris

FAPD :miniaturization LPP : vellus hairs areis a specific feature lost

3 Distinguish early scarring alopecias from FPHL/MPHL

Fibrosing alopecia with a pattern distribution

3 Distinguish early scarring alopecias from FPHL/MPHL

May be no so uncommon

Might be the reason of LPP after hair transplantation

Pathologists are really important in detecting these patients

Chiang YZ, Tosti A, Chaudhry IH, Lyne L, Farjo B, Farjo N, Cadore de Farias D,Griffiths CE, Paus R, Harries MJ. Lichen planopilaris following hairtransplantation and face-lift surgery. Br J Dermatol. 2012 Mar;166(3):666-370

4 Pitfalls

14 year ol africanamerican girl

3 months history of erythema, boggy induration,serosanguinous drainage and hair loss

4 Pitfalls

A scalp biopsy was read as consistent with dissecting cellulitis, PAS stain negative

4 PitfallsTreatment with doxycicline 200 mg daily and clobetasol 0.01% foam produced no improvement.

Follow up after 3 months showed persistence of tender scalp nodules , scalp erythema ,severe alopecia, pus discharge and cervical adenopathy

4 PitfallsScalp dermoscopy

Scales,broken hairs, comma and corkscrew hairs

4 PitfallsDiagnosis : tinea capitis

Terbinafine 250 mg day for 6 weeks

At end of treatment inflammation had completely resolved but areas of alopecia were still present

Diagnosis confirmed by culture that grew Trichophyton sp

4 Pitfalls

Why pathology showed dissecting cellulitis?

Why fungal stains were negative?

black dots

Tinea Capitis Mimicking Dissecting Cellulitis

Nodulocystic form of tinea capitis with overlying alopecia, closely resembling dissecting cellulitis of thescalp.

Histopathology shows a dense mixed lympho -plasmacytic and neutrophilic infiltrate and fungal stains are usually negative

Miletta NR, Schwartz C, Sperling L. Tinea capitis mimicking dissectingcellulitis of the scalp: a histopathologic pitfall when evaluating alopecia inthe post-pubertal patient. J Cutan Pathol. 2014 Jan;41(1):2-4.

4 Pitfalls

4 PitfallsTinea Capitis Mimicking Dissecting Cellulitis

Inflammatory tinea capitis can mimic dissecting cellulitis clinically and histologically

Dermoscopy may indicate correct diagnosis

Always take a culture in inflammatory scalp diseases of children and adolescents!!!

Culture and fungal stains maybe negative

LaSenna CE, Miteva M, Tosti A. Pitfalls in the diagnosis of kerion. J Eur Acad Dermatol Venereol. 2014 Dec 10. doi: 10.1111/jdv.12912.

Thank you!

[email protected]


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