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

Transcription

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

2 DISCLOSURE OF RELATIONSHIPS WITH INDUSTRY Antonella Tosti, MD S049 Alopecia DISCLOSURES Fotofinder :Consultant, Springer & Verlag, CRC Press :Author-Royalties, Karger : Editor in chief

3 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

4 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

5 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

6 1 Lack of clinico/pathological correlations Most common reasons Site of biopsy This site might not be affected and pathology shows no scarring

7 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

8 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

9 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!

10 1 Lack of clinico/pathological correlations Most common reasons Specimen processing Transverse Vertical

11 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 Jan;31(1):43-56.

12 1 Lack of clinico/pathological correlations Best 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.

13 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 Oct;27(10):

14 2 Utilize dermoscopy to select optimal biopsy site Instruments -DermLite (3Gen LLC.) -Handyscope (FotoFinder Systems) -DermScope (Canfield Imaging Systems)

15 2 Utilize dermoscopy to select optimal biopsy site Area 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

16 2 Utilize dermoscopy to select optimal biopsy site Dermoscopic features associated with disease activity in scarring alopecias. Peripilar casts Hair tufting Keratotic plugs White gray halos

17 2 Utilize dermoscopy to select optimal biopsy site Peripilar casts White concentric scales surrounding the hair shaft at its emergency

18 2 Utilize dermoscopy to select optimal biopsy site Hair tufting Tuft of 2 or more hairs surrounded by casts

19 2 Utilize dermoscopy to select optimal biopsy site Keratotic plugs Keratotic masses filling the follicular openings

20 2 Utilize dermoscopy to select optimal biopsy site White gray halos White gray dots surrounding a tuft of 2 hais

21 2 Utilize dermoscopy to select optimal biopsy site Site of Biopsy in Scarring Alopecias 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

22 2 Utilize dermoscopy to select optimal biopsy site 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

23 3 Distinguish early scarring alopecias from FPHL/MPHL Important mimics of MPHL/FPHL Frontal fibrosing alopecia Fibrosing alopecia with a pattern distribution

24 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Frequency is increasing world wide Not limited to postmenopausal women Commonly associated with androgenetic alopecia Early cases can be difficult to detects

25 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Clinical features Frontal hairline recession Loss of eyebrows Prominent temporal/frontal veins Hair loss in the limbs Facial lesions

26 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Frontal hairline recession Mean glabellar frontal distance : 8,5 cm ( controls 5.9 cm) The alopecic area shows less signs of photodamage as compared with the forehead Lencastre A, Tosti A. Images in clinical medicine. A receding hairline. N Engl J Med Jul 11;369(2):e2.

27 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia 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 Tosti A, Miteva M, Torres F. Lonely hair: a clue to the diagnosis of frontalfibrosing alopecia. Arch Dermatol Oct;147(10):1240

28 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Frontal hairline recession Perifollicular erythema and scaling

29 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Clinical features Loss of eyebrows (75% of patients

30 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Clinical features Prominent temporal/frontal veins

31 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Frequence is increasing world wide Very common in Europe, Americas, Africa, few cases reported from China, rare in South Arabia Role of sunscreens

32 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Not limited to postmenopausal women Also seen in young women and men

33 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Increasingly reported in men 1: Tolkachjov SN, Chaudhry HM, Camilleri MJ, Torgerson RR. Frontal fibrosing alopecia among men: A clinicopathologic study of 7 cases. J Am Acad Dermatol 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 Dec;107(10): : White F, Callahan S, Kim RH, et al Frontal fibrosing alopecia in a 46-year-old man. Dermatol Online J 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 Nov-Dec;80(6): : Khan S, Fenton DA, Stefanato CM. Frontal fibrosing alopecia and lupus overlap in a man: guilt by association? Int J Trichology Oct;5(4): : Chen W, Kigitsidou E, Prucha H, Ring J, Andres C. Male frontal fibrosing alopecia with generalised hair loss. Australas J Dermatol May;55(2):e : Debroy Kidambi A, Dobson K, Holmes S et al. Frontal fibrosing alopecia in men: an association with facial moisturizers and sunscreens. Br J Dermatol Jul;177(1):

34 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Increasingly reported in men Can start from sideburns Beard and body hair commonly involved

35 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Commonly associated with androgenetic alopecia

36 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Look at the hairline of all women consulting for hair loss! Parietal hairline often first site of involvement

37 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia 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

38 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Tips to recognize early FFA Suspect 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 May 13

39 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Tips to recognize early FFA Be aware of facial lesions! Facial papules Keratosis pilaris like lesions Facial erythema Facial macules Facial hyperpigmentation

40 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Facial papules More common in women with dark phototypes Forehead Temples Checks Chin

41 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Facial papules Lichenoid inflammation involving vellus hair follicles and perifollicular fibrosis

42 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Facial papules Pinkus acid orcein staining showing reduction and fragmentation of elastic fibers.

43 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia 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 Feb;78(2):e45.

44 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia 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 Oct;77(4):

45 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Other facial lesions In dark phototypes easily confused with melasma In fair phototypes easily confused with rosacea

46 3 Distinguish early scarring alopecias from FPHL/MPHL Frontal fibrosing alopecia Tips to recognize early FFA Look at hairline for presence/absence of vellus hair You need a dermatoscope!

47 3 Distinguish early scarring alopecias from FPHL/MPHL Take home message FFA increasingly common Not limited to postmenopausal women You might get eyebrow biopsies You might get biopsies of facial lesions

48 3 Distinguish early scarring alopecias from FPHL/MPHL 26 year old man with patterned alopecia and scalp itching

49 3 Distinguish early scarring alopecias from FPHL/MPHL Dry dermoscopy Hair shaft variability Peripilar casts Hair tufting V sign

50 3 Distinguish early scarring alopecias from FPHL/MPHL Fibrosing alopecia with a pattern distribution First described by Zinkernagel &Trueb in 2011 Pathology :miniaturization (as in androgenetic alopecia) and lichenoid perifollicular inflammation Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch Dermatol Feb;136(2):

51 3 Distinguish early scarring alopecias from FPHL/MPHL Fibrosing alopecia with a pattern distribution Diagnosis : Need to take dermoscopy guided biopsy!

52 3 Distinguish early scarring alopecias from FPHL/MPHL Fibrosing alopecia with a pattern distribution Diagnosis pathology : Need horizontal sections!

53 3 Distinguish early scarring alopecias from FPHL/MPHL Fibrosing alopecia with a pattern distribution vs lichen planopilaris FAPD :miniaturization is a specific feature LPP : vellus hairs are lost

54 3 Distinguish early scarring alopecias from FPHL/MPHL Fibrosing alopecia with a pattern distribution 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 Mar;166(3):

55 4 Pitfalls 14 year ol african american girl 3 months history of erythema, boggy induration, serosanguinous drainage and hair loss

56 4 Pitfalls A scalp biopsy was read as consistent with dissecting cellulitis, PAS stain negative

57 4 Pitfalls Treatment 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

58 4 Pitfalls Scalp dermoscopy Scales,broken hairs, comma and corkscrew hairs

59 4 Pitfalls Diagnosis : 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

60 4 Pitfalls Why pathology showed dissecting cellulitis? Why fungal stains were negative?

61 4 Pitfalls Tinea Capitis Mimicking Dissecting Cellulitis Nodulocystic form of tinea capitis with overlying black alopecia, dots closely resembling dissecting cellulitis of the scalp. 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 Jan;41(1):2-4.

62 4 Pitfalls Tinea 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 Dec 10. doi: /jdv

63 Thank you!