Cicatricisial alopecia

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LICHEN PLANOPILARIS
CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS
CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA
PSEUDOPELADE OF BROCQ
ALOPECIA MUCINOSIS
KERATOSIS PILARIS SPINULOSA DECALVANS

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  • PCA:based on the predominant inflammatorycell present in the biopsy specimenSCA, the hair follicles are secondarily damaged as a result of more generalized destructive process within the skin, which ultimately destroys the hair follicle stem cell (HFSC)-based capacity for regeneration
  • In normal pilosebaceous units, PPAR binds to PPAR response elements (PPREs) on target genes and maintains lipid homeostasis by regulating peroxisome biogenesis and lipid metabolism. PPAR also modulates the inflammatory response by regulating the expression of cytokine genes.
  • The tufts consist of a central anagen hair surrounded by telogen hairs, each arising from independent follicles, converging towards a common dilated follicular infundibulum.
  • no specific causative organism has been isolated
  • Multiple areas of hair loss with scarring
  • (A) loss of orifices in linear scleroderma (sclerodermie en coup de sabre), (B) broken hairs, (C) black dots, (D) tapering hairs (exclamation mark hairs), (E) yellow dots and (F) short vellus hairs in alopecia areata, (G) micropustules and (H) hair tufting with six or more hairs in folliculitisdecalvans/tufted folliculitis, (I) comma hairs in tineacapitis, and (J) short vellus hairs in androgenetic alopecia
  • Cicatricisial alopecia

    1. 1. TUTORIAL PRESENTATION CICATRICIAL ALOPECIA
    2. 2. Definition:Permanent area of hair loss associated with destruction of hair follicles  Pilosebaceous structures are replaced by fibrous tracts  Classified as: 1. Primary 2. Secondary  Trauma  Sclerosing disorders  Granulomatous disorders  Infections  Neoplastic 3. Developmental/Hereditary  PCA more common than SCA (4:1) 
    3. 3. ANOTHER CLASSIFICATION PROPOSED TO FACILITATE DETERMINATION OF MOST SUITABLE SURGICAL CORRECTIVE THERAPIES FOR  CA : stable    Traumatic Aplasia cutis CCCA unstable –       Lymphocytic Neutrophilic Mixed Infections Congenital Neoplastic
    4. 4. PATHOGENESIS       HFSCs destruction theories Impairment of self maintenance of HFSCs Alteration of lipid metabolism Neurogenic inflammation theory Environment factors Genetic factors
    5. 5. PPAR DEFICIENCY CAUSES LOSS OF PEROXISOME BIOGENESIS, DEREGULATES LIPID METABOLISM, AND PRODUCES PROINFLAMMATORY LIPIDS THAT TRIGGER INFLAMMATORY RESPONSE THAT IN TURN CAUSES TISSUE DAMAGE AND PERMANENT HAIR LOSS
    6. 6.  NORMAL IN CICATRICIAL ALOPECIA
    7. 7. APPROACH TO THE PATIENT  History      Clinical Findings    Onset Presence of- pruritus, irritation, pain, erythema and/or drainage from the scalp Evaluate for autoimmune disease, systemic illness, infections, neoplasms, associated inflammatory skin disease and radiation treatment or burns Drug intake loss of follicular ostia erythema, scaling, pustules, scalp bogginess and compound follicles (polytrichia) Biopsy
    8. 8. Non-scarring hair Incidence Erythema/scaling/pustules More common Scarring hair loss Less common -/+ + Atrophy absent present Loss of follicular openings absent present Tufted hair absent present Course regrowth is quite common Prognosis generally favourable no regrowth generally unfavourable
    9. 9. PRIMARY CICATRICAL ALOPECIA Diagnosis in which lymphocytes predominates includes: LICHEN PLANOPILARIS CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA PSEUDOPELADE OF BROCQ ALOPECIA MUCINOSIS KERATOSIS PILARIS SPINULOSA DECALVANS
    10. 10. Classic lichen planus 40% of pt have skin manifestations C/Fs: violaceous papules,erythema scaling Papules replaced by follicular plugs Plugs shed and finally atrophic,smooth,scarred area remains Pt commonly presents with pseudopelade like patches Frontal Fibrosing Alopecia Resembles AGA with frontal recession C/Fs:perifollicular erythema and hyperkeratoses at marginal hairline Slow progressive disease Typically occurs in Post-menopausal women GRAHAM-LITTLE PICCARDI LASSUEUR SYNDROME AFFECTS WOMEN BETWEEN 30-70 YRS SYNDROME CHARACTERIZED BY:PROGRESSIVE CICATRICIAL ALOPECIA OF SCALP,NON SCARRING ALOPECIA OF AXILLA AND PUBIC AREA AND KERATOSIS PILARIS
    11. 11. Central centrifugal cicatricial alopecia hot comb alopecia, follicular degeneration syndrome, pseudopelade in African Americans central elliptical pseudopelade Premature disintegration of inner root sheath epithelium occurs Begins as single focus over vertex of scalp and then spread centrifugally Pseudopelade Idiopathic,chronic,slowly progressive Patchy cicatricial alopecia that occur without any evidence of inflammation footprints in the snow
    12. 12. Chronic Cutaneous Lupus Erythematosus Keratosis follicularis spinulosa decalvans 30% have skin manifestation Erythema, scaling and pigmentary changes are more pronounced Follicular plugging and adherent scale may be present. The “carpet tack” sign may be elicited with retraction of scale revealing keratotic spikes that correspond to follicular openings on undersurface X-Linked recessive SSAT gene defect Erythema, plugging of eyebrow follicles follicular hyperkeratosis & prominent cuticles Ocular signs include blepharitis, ectropion, corneal dystrophy and photophobia Focal PPK may be present
    13. 13.  Diagnosis in which neutrophils predominates includes: FOLLICULITIS DECALVANS DISSECTING CELLULITIS OF SCALP
    14. 14. Folliculitis decalvans -Recurrent crops of follicular pustules that result in permanent epilation -Staph aureus may be grown from pustules -Pustular folliculitis followed by rounded patches of alopecia develop surrounded by crusting and few follicular pustules. -Successive crops of pustules appear and are followed by progressive destruction of affected follicles -Tufted folliculitis variant of folliculitis decalvans where circumscribed areas of scalp inflammation heal with scarring characterized by tufts of up to 15 hairs emerging from single orifice
    15. 15. Dissecting cellulitis of the scalp Perifolliculitis capitis abscedens et suffodiens       Perifolliculitis of scalp, deep and superficial abscesses in dermis, sinus tract formation and extensive scarring Aetiology:staphylococci, streptococci and Pseudomonas may be cultured from various lesions C/Fs:Painful, firm, skin-coloured nodules develop near vertex Confluent nodules form tubular ridges with an irregular cerebriform pattern Progressive scarring and permanent alopecia occur Chronic condition with frequent acute exacerbations.
    16. 16.  Diagnoses in which a mix of cell types predominate are as follows: ACNE KELOIDALIS ACNE NECROTICA EROSIVE PUSTULAR DERMATOSES OF SCALP
    17. 17. Acne keloidalis It occurs in males after puberty between the ages of 14 -25 yrs C/Fs:Pts present with pustules, alopecia and hypertrophic scarring on posterior neck Friction from the collar is often incriminated Process begins with penetration of cut hair into the skin as in pseudofolliculitis Acne necrotica More frequent in men than in women 30- 50 yrs C/Fs:red itchy acneiform papules arise spontaneously on the front and sides of scalp papules are usually centered around pilosebaceous unit Often umbilicated and rapidly transformed by necrosis into an adherent haemorrhagic crust which separates after 3 or 4 weeks to leave a permanent varioliform scar
    18. 18. Erosive pustular dermatosis of the scalp       Particularly affects the elderly Precipitating factors:local trauma sundamage,surgery, cryosurgery skin grafting and radiation therapy Initially, a small area of scalp becomes red, crusted and irritable crusting and superficial pustulation overlie a moist, eroded surface As condition extends areas of activity coexist with areas of scarring. Squamous carcinoma has developed in scars
    19. 19. SECONDARY CICATRICIAL ALOPECIA
    20. 20. GRANULOMATOUS DISORDERS Necrobiosis lipoidica, granuloma annulare and sarcoidosis      C/F:The oval atrophic plaques on the shins but may be seen on other parts of body including scalp. The patches are glazed, yellowish often with conspicuous telangiectasia Scarring may be dense. Clinical features in scalp vary from large plaques of cicatricial alopecia to multiple small areas of scarring Cutaneous sarcoidosis may produce plaques or nodules on scalp
    21. 21. SCLEROSING DISORDERS Circumscribed scleroderma and linear morphoea rare in the scalp ‟en coup de sabre‟ morphoea – is more common Cicatricial pemphigoid Women > men disease predominantly affects ocular and/or genital mucous membrane skin is involved in 40–50% scalp involved in 10% of cases
    22. 22. TRAUMATIC Traction alopecia  Common in Afro-Caribbean hair styles  Due to sustained pull on hair roots  Folliculitis , hair casts reduction in hair density with vellus hairs and sometimes broken hairs  Hair loss begins in temporal regions and in front of and above the ears but may involve other parts of scalp Medical trauma  CHILD: Scalp electrodes or infusion or forceps delivery or uterine rings in neonate can result in trauma.  ADULT:Brain surgery, gynaecological surgery in Trendelenburg position
    23. 23. TRICHOTILLOMANIA          Behavioural disorder characterized by compulsive hair pulling Trichoteiromania : Compulsive hair rubbing Trichotemnomania : Compulsive hair cutting Hair is plucked most frequently from one frontoparietal region Patch of hair loss bizarre or angular pattern in which hairs are twisted and broken at various distances from clinically normal scalp H/P: Numerous empty canals , clefts in hair matrix, intraepithelial and perifollicular haemorrhages and intrafollicular pigment casts Some follicles are severely damaged Follicular epithelium is separated from connective tissue sheath Trichomalacia - Injured follicles may form only soft, twisted hair
    24. 24. Syphilitic alopecia. The scalp has moth-eaten appearance, eyebrow hair is absent there is rash on the cheek Trichotillomania. Hairs are thin and of different lengths TRACTION ALOPECIA. TRACTION FOLLICULITIS IS COMMONLY ASSOCIATED
    25. 25. TRACTION ALOPECIA DERMATOPHYTE:INFECTION
    26. 26. DIAGNOSIS  Dermoscopy/Trichoscopy - first-line, noninvasive method  Absence of follicular ostia in 100% cases even if it is not evident clinically FFA :loss of orifices, perifollicular scale and feeble perifollicular erythema Folliculitis decalvans :existence of micropustules and/or hair tufting with >=6 hairs DLE: follicular red dots LPP: hair tufting, violaceous-blue interfollicular area, corresponding to pigment incontinence Lipedematous alopecia: linear area of telangiectasia within scalp creases, possibly caused by compression of the superficial blood capillaries Scalp sarcoidosis: orange spots seen(round, well-formed granulomas in superficial dermis) Traction alopecia: Hair casts       
    27. 27. Reflectance confocal microscopy Microscopic imaging of superficial layers of skin down to superficial reticular dermis with resolution at cellular level close to conventional histopathology  May also help in choosing most appropriate biopsy site for more informative histology  Histopathology Direct immunofluorescence Microarray analysis
    28. 28. LUPUS ERYTHEMATOUS: H/P:VACUOLAR INTERFACE ALTERATION OF FOLLICULAR EPITHELIUM, SCATTERING OF DYSKERATOTIC KERATINOCYTES, VARIABLY DENSE PERIADNEXAL, PERIFOLLICULAR (UPPER PORTION), PERIVASCULAR AND INTERSTITIAL LYMPHOCYTIC INFILTRATE WITH DERMAL MUCIN, ATROPHY OF SEBACEOUS GLANDS AND FOLLICULAR PLUGGING. EPIDERMIS MAY BE ATROPHIED WITH VACUOLAR INTERFACE CHANGES. CONCENTRIC LAMELLAR FIBROSIS AROUND THE FOLLICLE IN END STAGES follicular plugging, superficial,deep perivascular and periappendageal lymphocytic infiltrate There is linear staining of deposits of complement (C3), IgM and IgG on the basement membrane in more than 80% of cases of LE
    29. 29. LICHEN PLANUS: ACTIVE : FOLLICULAR LYMPHOCYTIC INTERFACE DERMATITIS WITH DENSE BAND LIKE LYMPHOCYTES AROUND UPPER FOLLICLE & INFUNDIBULUM OBSCURING DEJ, INFUNDIBULAR HYPERKERATOSIS AND HYPERGRANULOSIS, CYTOID BODIES SCATTERED ALONG THE BMZ, ABSENT OR ATROPHIC SEBACEOUS GLANDS WITH OR WITHOUT PIGMENTARY INCONTINENCE. END –STAGE : LONGITUDINAL TRACTS OF FIBROSIS,LAMELLAR FIBROSIS,EPIDERMAL ATROPHY DIF:„SHAGGY‟ OR „PATCHY‟ DEPOSITION OF FIBRINOGEN AND CLUMPED IGM OR LESS COMMONLY IGA AND C3 DEPOSITS ARE SEEN ALONG FOLLICULAR BMZ
    30. 30. Pseudopelade of Brocq   Variably dense perifollicular lymphocytic infiltrate in early stage, followed by eccentric atrophy of follicle infundibular epithelium, concentric lamellar fibrosis around upper follicle and loss of sebaceous gland in later stage Elastin stains reveal dense elastic tissue cuffing a broad fibrotic follicular tract in advanced disease thinned out epidermis with total loss of hair follicles, replaced by collagen
    31. 31.  Central centrifugal cicatricial alopecia :Earliest feature is premature disintegration of IRS resulting in outward migration of hair shaft through ORS at level of isthmus. Lamellar fibroplasias and lymphocytic inflammation surround the follicle at this level, resulting in follicular destruction and fibrous tract formation.  Alopecia mucinosa :Mucinous degeneration of ORS and sebaceous glands. A perifollicular lymphocytic infiltrate often with eosinophils and histocytes  Keratosis follicularis spinulosa decalvans :Compact hyperkeratosis, hypergranulosis of upper follicular epithelium with superficial intrafollicular and peri-follicular edema in early stage whereas in advanced stage there is concentric perifollicular, horizontal adventitial lamellar fibrosis and scarred follicular tracts
    32. 32.     Folliculitis decalvans: Acneiform dilatation with perifollicular neutrophilic inflammation- later mixed inflammatory infiltrate of neutrophils, lymphocytes, plasma cells. Follicular rupture,foreignbody giant cell granuloma formation around exposed hair shaft fragments. In burnt outstage, follicular and adventitial fibrosis is seen Dissecting cellulitis of scalp: Infundibular acneiform distention with intrafollicular and perifollicular neutrophilic infiltration, abscess formation,sinus tracts Acne keloidalis : perifollicular and intrafollicular lymphoplasmacytic infiltrate at the level of sebaceous glands -complete follicular destruction occurs with loss of sebaceous glands and dermal fibrosis Acne necrotica:dense perivascular and perifollicular lymphocytic infiltrate with prominent sub-epidermal edema. Necrosis of individual keratinocyte is seen initially and is followed by confluent necrosis of the central follicle and interfollicular epidermis
    33. 33. TREATMENT  Aim of treatment currently focuses reduction of symptoms and to reduce or stop progression of disease. LMPCA with immunosuppression  NMPCA with antimicrobials or dapsone 
    34. 34. DLE First line  Class I or II potent topical corticosteroids  I/L steroid inj (10 mg/ml max 2 ml, every 4-6 weeks)  Results are assessed till 8 weeks, if no response shift to next level Second line  Antimalarials (HCQ 200-400 mg/day, clinical effect in 4-8 weeks, continued till 3-6 mths)  Oral corticosteroids (1 mg/kg, for initial actively progressing disease, tapered over 8 weeks)  Retinoids (acitretin and Isotretinoin 1040 mg/day) Third line  Thalidomide, topical immunomodulators , oral vit E, gold, dapsone,MMF,methotrexate, azathioprine,clofazamine,systemic or intralesional INFα2, monoclonal antiCD4 antibodies, topical 5-FU, topical tazarotene imiquimod. LPP First line  Potent topical corticosteriods  I/L Triamcinolone acetonide Second line  Oral corticosteroids  Oral cyclosporine(4-5 mg/kg for 4-6 mths)  Topical cyclosporine (oily solution, applied twice daily for initial 3 mths and once daily for further 3 mths)  Oral tetracycline Third line  Retinoids ,Antimalarials,MMF(500 mg twice daily)  Others: Thalidomide,griseofulvin,low molecular weight heparin (s/c injections 3 mg once weekly), exicmer laser Newer therapies  PPARγ agonist like thiozolidinediones
    35. 35. FFA       Intralesional triamcinolone acetonide Finasteride (2.5 mg OD) Oral corticosteroids Antimalarials Topical corticosteroids with topical minoxidil Oral retinoids GLP     Topical and intralesional corticosteroids Oral ciclosporin Systemic corticosteroids Topical tacrolimus PPB  Potent topical corticosteroids(±) Alopecia mucinosa First line  Potent topical corticosteriods Second line  I/L triamcinolone  Minocycline  Dapsone Third line  Systemic steroids,isotretinoin, antimalarials,PUVA,interferon α2b+Interferon γ,superficial X-rays KFSD     Oral antibiotics Dapsone Oral retinoids Laser epilation
    36. 36. FD First line  Oral±topical antibiotics Second line  Oral rifampicin (300 mg BD)+oral clindamycin (300 mg BD)  Rifampicin+ (doxycycline/ciprofl oxacin/clarithromycin)  Oral rifampicin+topical antibiotics Third line  Oral fusidic acid  Oral zinc  Dapsone  Oral cyclosporine  Excision,laser,radiotherapy,i/m Human immunoglobulin Dissecting cellulitis of scalp First line  Oral isotretinoin  Oral isotretinoin+i/l triamcinolone acetonide Second line  Oral antibiotics+topical antibiotics/topical retinoids  Aspiration and i/l triamcinolone acetonide Third line  Low dose corticosteroids  Colchicine  Dapsone  Excision and skin grafting  Lasers and radiotherapy
    37. 37. Acne necrotica varioliformis Acne keloidalis nuchae First line  Potent topical steroid  Oral antibiotics+topical steroids/intralesional triamcinolone Second line  Surgical excision  CO2 laser  Diode laser hair epilation Third line  Radiotherapy  Isotretinoin Oral antibiotics  Oral isotretinoin  I/L triamcinolone  Erosive pustular dermatosis of scalp  Topical corticosteroids  Topical immunomodulators  Calcipotriol cream  Oral Isotretinoin
    38. 38.  Surgical treatment of scarring alopecia includes :  hair transplantation  scalp reduction or alopecia reduction surgeries  tissue expansion  flap surgeries
    39. 39. CONCLUSION  CA is „trichology emergency‟ situation, in which lack of prompt and early treatment will lead to the inevitable loss of hair follicles along with permanent scarring.  Newer pathogenesis has given the platform for development of emerging treatment modalities.  But still great deal of research is required in this field, may be developing viable stem cell therapies or bioengineered human hair follicles are the answers to it in future
    40. 40. THANK YOU

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