Cicatricisial alopecia

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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. ANOTHER CLASSIFICATION PROPOSED TO FACILITATE DETERMINATION OF MOST SUITABLE SURGICAL CORRECTIVE THERAPIES FOR CA :stable Traumatic Aplasia cutis CCCAunstable Lymphocytic Neutrophilic Mixed Infections Congenital Neoplastic 4. PATHOGENESIS HFSCs destruction theories Impairment of self maintenance of HFSCs Alteration of lipid metabolism Neurogenic inflammation theory Environment factors Genetic factors 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. NORMALIN CICATRICIAL ALOPECIA 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 inammatory skin disease and radiation treatment or burns Drug intake loss of follicular ostia erythema, scaling, pustules, scalp bogginess and compound follicles (polytrichia)Biopsy 8. Non-scarring hair IncidenceErythema/scaling/pustulesMore commonScarring hair loss Less common-/++AtrophyabsentpresentLoss of follicular openingsabsentpresentTufted hairabsentpresentCourseregrowth is quite commonPrognosisgenerally favourableno regrowth generally unfavourable 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. 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 patchesFrontal Fibrosing Alopecia Resembles AGA with frontal recession C/Fs:perifollicular erythema and hyperkeratoses at marginal hairline Slow progressive disease Typically occurs in Post-menopausal womenGRAHAM-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. 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 centrifugallyPseudopelade Idiopathic,chronic,slowly progressive Patchy cicatricial alopecia that occur without any evidence of inflammation footprints in the snow 12. Chronic Cutaneous Lupus ErythematosusKeratosis follicularis spinulosa decalvans30% 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 undersurfaceX-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. Diagnosis in which neutrophils predominates includes: FOLLICULITIS DECALVANS DISSECTING CELLULITIS OF SCALP 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. 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. Diagnoses in which a mix of cell types predominate are as follows: ACNE KELOIDALIS ACNE NECROTICA EROSIVE PUSTULAR DERMATOSES OF SCALP 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 pseudofolliculitisAcne 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. 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. SECONDARY CICATRICIAL ALOPECIA 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. SCLEROSING DISORDERS Circumscribed scleroderma and linear morphoea rare in the scalp en coup de sabre morphoea is more commonCicatricial pemphigoid Women > men disease predominantly affects ocular and/or genital mucous membrane skin is involved in 4050% scalp involved in 10% of cases 22. TRAUMATIC Traction alopeciaCommon in Afro-Caribbean hair stylesDue to sustained pull on hair rootsFolliculitis , hair casts reduction in hair density with vellus hairs and sometimes broken hairsHair loss begins in temporal regions and in front of and above the ears but may involve other parts of scalpMedical traumaCHILD: 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. 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. Syphilitic alopecia. The scalp has moth-eaten appearance, eyebrow hair is absent there is rash on the cheekTrichotillomania. Hairs are thin and of different lengthsTRACTION ALOPECIA. TRACTION FOLLICULITIS IS COMMONLY ASSOCIATED 25. TRACTION ALOPECIADERMATOPHYTE:INFECTION 26. DIAGNOSIS Dermoscopy/Trichoscopy - first-line, noninvasive methodAbsence 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. 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. 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 STAGESfollicular plugging, superficial,deep perivascular and periappendageal lymphocytic infiltrateThere is linear staining of deposits of com