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Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
REVIEWHistopathology of alopecia: aclinicopathological approach                         Article firstto diagnosis         ...
The hair follicle may be divided anatomically into  four parts:1.The bulb consisting of the dermal papilla and matrix.2.Th...
:2.3. Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
The lower portion of the hair follicle      consists of five major portions:1. The dermal papilla2. The matrix3. The hair ...
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
The Hair Growth CycleHair follicles grow in repeated cycles. One cycle can be broken down into three phases.1.Anagen - Gro...
•Alopecia means loss of hair from the head or body.Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
1.   Alopecia Areata                               9..Scarring Alopecia2.   Alopecia Totalis                              ...
1.  Alopecia mucinosa                              12.Lupus erythematosus2.  Androgenic alopecia                          ...
I.Scarring AlopeciaII.Non-scarring Alopecia1.Primary Alopecia2.Secondary Alopecia Tuesday, September 11, 2012   Dr Mohamma...
Common forms of               Scarring alopeciasI.Lymphocytic1.Disdcoid lupus erythematosus2. Lichen planopilaris3.Central...
1.Androgenic alopecia2. Telogen effluvium3. Alopecia areata4. Trichotillomania5.Traction alopecia. Tuesday, September 11, ...
The histopathological interpretation of scalp biopsy  specimens of patients with alopecia may  represent a challenging tas...
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
Vertical (longitudinal) sectionsA 4-mm vertically-sectioned punch biopsy specimen  is adequate for assessing alopecias ass...
Vertical (longitudinal) sectionsThus, vertical sectioning will show only 10% of the  follicles present in the specimen, wi...
Transverse sectioning will allow detection of follicular pathology,   even if it is focal. Moreover, it will yield quantit...
Frishberg et al proposed a variant of the transver sesectioning   technique whereby the 4-mm biopsy specimen is sliced int...
Tansverse sectioning permits a quantitative approach to   diagnosis, it is imperfect, as it does not allow for a   qualita...
Transverse Section                        Vertical Section1. Quantitative approach                  1. Qualitative approac...
The St John’s                   multiteam clinicopathological                                        approachThe protocol ...
The St John’s multiteam                 clinicopathological approachA newly proposed model to avoid sample bias and toachi...
The St John’s                       clinicopathologicalThus, the key factors that will enhance the histopathological   dia...
"mode of operation”)Two 4-mm punch biopsy specimens, both taken at theperipheral edge of the alopecia deemed to be the ‘ac...
Figure 1. A, A 4-mm punch biopsyspecimen is sectioned horizontally.Figure 1. B, The two cut surfaces are red-inked before ...
The transversely sectioned skin biopsy specimen is  placed in a                     when grossing, and                    ...
Specimen 1                      Scarring Alopecia                                                                Specimen ...
Among the non-scarring alopecias, the most difficulttask for the histopathologist is to distinguish                       ...
Non-scarring Alopecia                                  1                       2Transverse section Figure 3. Schematic dia...
In both scarring and non-scarring alopecia protocols,the scalp biopsy specimens are                        until the histo...
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
(cicatricial) alopeciaScarring (cicatricial) alopecia represents a complex group of  hair disorders all characterized by h...
(cicatricial) alopeciaHistopathologically, primary scarring (cicatricial)  alopecia is characterized by the presence of fi...
Table 1.   Secondary scarring alopecia•          Aplasia cutis congenita• Cicatricial bullous pemphigoid• Infectious (keri...
Column1                  Column2       Column3                Column4   Column5Table 2. Classification of primary cicatric...
Keratosis follicularis spinulosa decalvansii.NeutrophilicFolliculitis decalvansDissecting cellulitis ⁄ folliculitis (perif...
i.Lymphocytic scarring alopeciasClinically, chronic cutaneous lupus erythematosus ischaracterized by                      ...
i.Lymphocytic scarring alopecias        Chronic Discoid Lupus ErythematosusHistopathological features include             ...
A                                                    B                                          C             D           ...
Clinically, typical lesions of lichen planopilaris (LPP)   present with atrophic, ill-defined patches of scarring   alopec...
Subsets of                        of LPP have been  described in patients presenting with progressive  fibrosing alopecia ...
Histopathologically, the features of LPP and its variants are similar,   irrespective of clinical presentation.In early le...
Mucinous perifollicular fibroplasia with absence of the  interfollicular dermal mucinin the upper dermis has been  describ...
A   B                                         CFigure 5. Lichen planopilaris (                                            ...
A                      BFigure 6.                    ,                  (vertical section).A, Superficial dermal wedge-sha...
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
CCCA                   CCCA                              or  Most commonly seen in young to middle-aged   women of African...
CCCA   The possibility of genetic and autoimmune involvement    in the               has been postulated.              , C...
perifollicular concentric                            ,  mild perifollicular and perivascular                              ...
CCCA                                                              ,          , and                              .In CCCA p...
B                    A                   C                              DFigure 7.                                  . A, P...
An idiopathic and slowly progressive form of cicatricial  alopecia,               presenting with multiple small  alopecic...
Folliculitis decalvans is a primary neutrophilic cicatricial  alopecia affecting middle-aged adults.                      ...
Perifollicular fibrosis with fibrous tracts replacing the hair  follicles, follicular tufting and interstitial dermal fibr...
A                                             B                                            D                 CFigure 8. Fo...
2.                   (perifolliculitis capitis abscedens et suffodiens)This entity commonly occurs in young men of African...
2.                    , there is a dense deep dermal and  subcutaneous, predominately  with                    and        ...
iii.Mixed scarring alopeciasThis entity is also known as acne keloidalis nuchae, as it usually   occurs on the occipital s...
II.Non-scarring alopeciasAndrogenic alopecia is the most common type of hair   loss. It is a disorder of dominant inherita...
Histopathologically, the use of transverse sections is the  most valuable method to reach a diagnosis, as all the  hair fo...
Other findings include an increased number of telogen  hairs, decreased numbers of terminal hair follicles in  the subcuta...
B                        A                                                              CFigure 9. Female pattern hair los...
Telogen effluvium is a diffuse form of alopecia, in which the    hair shedding may be acute or chronic.Clinically, acute t...
Histopathology of acute telogen effluvium shows a normal number of   hair follicles with no miniaturization,and resembles ...
Alopecia areata (AA) is thought to be an organ-   specific autoimmune disorder.It commonly occurs in association with othe...
A                           B                                                    D                                CFigure ...
Clinically, it is characterized by sudden onset of   patches of nonscarring hair loss, with   ‘exclamation-point’ hairs.It...
Histopathologically, the morphological features are  dependent upon the duration of the episode, and  may be divided into ...
Initially, the terminal hairs are attacked, but   subsequently also the vellus hairs become involved.Eosinophils and plasm...
In longstanding (chronic) stages, with repeated  episodes, the peribulbar lymphoid cell infiltrate  also involves miniatur...
Many empty infundibula may be seen, corresponding to the   total scalp hair loss.In a small percentage of cases (10%) with...
Trichotillomania is characterized by the compulsive   intentional pulling or twisting of the hair.This hair loss disorder ...
Histopathologically, this is a non-inflammatory non-   scarring alopecia in which the morphological changes   are those of...
A                                                          B                     C                                        ...
Traction alopecia, like trichotillomania, is a   noninflammatory, non-scarring alopecia secondary to                      ...
Histopathologically, the features observed in early  traction alopecia are similar to those seen in  trichotillomania, whe...
Tuesday, September 11, 2012   Dr Mohammad Manzoor Mashwani
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  • Transcript of "Histopathology of alopecia"

    1. 1. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    2. 2. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    3. 3. REVIEWHistopathology of alopecia: aclinicopathological approach Article firstto diagnosis published online:Catherine M Stefanato 23 DEC 2009. ...Department of Dermatopathology, StJohn’s Institute of Dermatology, StThomas’ Hospital, London, UKStefanato C M(2010) Histopathology 56, 24–38 Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    4. 4. The hair follicle may be divided anatomically into four parts:1.The bulb consisting of the dermal papilla and matrix.2.The suprabulbar area from the matrix to the insertion of the arrector pili muscle.3.The isthmus that extends from the insertion of the arrector pili muscle to the sebaceous gland.4.The infundibulum that extends from the sebaceous gland to the follicular orifice. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    5. 5. :2.3. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    6. 6. The lower portion of the hair follicle consists of five major portions:1. The dermal papilla2. The matrix3. The hair shaft, consisting from inward to outward the medulla, cortex, and cuticle4. The inner root sheath (IRS) consisting of the inner root sheath cuticle, Huxley’s layer, and Henle’s layer5. The outer root sheath (ORS). Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    7. 7. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    8. 8. The Hair Growth CycleHair follicles grow in repeated cycles. One cycle can be broken down into three phases.1.Anagen - Growth Phase2.Catagen - Transitional phase3.Telogen - Resting PhaseTuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    9. 9. •Alopecia means loss of hair from the head or body.Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    10. 10. 1. Alopecia Areata 9..Scarring Alopecia2. Alopecia Totalis 10.Chemo Hair Loss3. Alopecia Universalis 11.Childrens Alopecia4. Alopecia Barbae 12.Female Pattern Baldness5. Alopecia Musinosa 13.Diffuse Hair Loss6. Anagen Effluvium 14.Alopecia Trichotillomania7. Traction Alopecia 15.Alopecia Cicatricial8. Telogen Effluvium 16.Alopecia Ophiasis Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    11. 11. 1. Alopecia mucinosa 12.Lupus erythematosus2. Androgenic alopecia 13.Medications (side effects from3. Diabetes drugs, including chemotherapy,4. Dissecting cellulitis anabolic steroids, and birth5. Fungal infections (such as tinea control pills) capitis) 14.Pseudopelade of Brocq6. Hair treatments (chemicals in 15.Radiation therapy relaxers, hair straighteners) 16.Scalp infection7. Hereditary disorders8. Hormonal changes 17.Secondary syphilis9. Hyperthyroidism and 18.Telogen effluvium hypothyroidism 19.Traction alopecia10. Hypervitaminosis A 20.Trichotillomania11. Iron deficiency or malnutrition 21.Tufted folliculitis in general Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    12. 12. I.Scarring AlopeciaII.Non-scarring Alopecia1.Primary Alopecia2.Secondary Alopecia Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    13. 13. Common forms of Scarring alopeciasI.Lymphocytic1.Disdcoid lupus erythematosus2. Lichen planopilaris3.Central centrifugal cicatricial alopecia4.Cseuopelade of BrocqII. Neutrophilic1.Folliculitis decalvans2. Dissecting folliculitisIII. MixedAcne keloidalis. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    14. 14. 1.Androgenic alopecia2. Telogen effluvium3. Alopecia areata4. Trichotillomania5.Traction alopecia. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    15. 15. The histopathological interpretation of scalp biopsy specimens of patients with alopecia may represent a challenging task, especially in the absence of a good, definitive clinical history, adequate tissue sampling, and an appropriate grossing technique.The histopathology of the most commonly encountered varieties of primary scarring (cicatricial) and nonscarring alopecias forms the basis of this review. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    16. 16. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    17. 17. Vertical (longitudinal) sectionsA 4-mm vertically-sectioned punch biopsy specimen is adequate for assessing alopecias associated with interface changes, lichenoid infiltrates, and subcutaneous pathology.However, only portions of a small number of follicular units (2–3) are seen in a given tissue section, because the hair follicles, which grow at an angle, are cut tangentially and, as such, cannot be visualized in their entirety in conventional vertical sections. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    18. 18. Vertical (longitudinal) sectionsThus, vertical sectioning will show only 10% of the follicles present in the specimen, with the consequent risk of sampling error, as the pathological changes may be present only focally in a few hair follicles.Moreover, obtaining hair follicles’ quantitative data in non-scarring hair loss disorders such as androgenic alopecia, telogen effluvium and alopecia areata, is precluded.On the other hand, a transversely sectioned specimen will include all the hair follicles present in the biopsy, and in the same section. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    19. 19. Transverse sectioning will allow detection of follicular pathology, even if it is focal. Moreover, it will yield quantitative data of follicular cycling, as well as morphometric evaluation of the hair follicles throughout their entire length, from the bulb to the acroinfundibulum.To achieve this, 1. a 4-mm punch biopsy specimen including subcutaneous tissue is required, as well as 2. special training of the pathology laboratory personnel for appropriate grossing and embedding of the specimen. Since the original description by Headington, who demonstrated that the best area to bisect the biopsy transversely is 1 mm above the dermal– subcutaneous junction,other authors have indicated slightly different areas to section, such as 1–1.5 mm below the epidermal–dermal junction, or 1–2 mm below the epidermal surface. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    20. 20. Frishberg et al proposed a variant of the transver sesectioning technique whereby the 4-mm biopsy specimen is sliced into three or four sections, and all the disks embedded with the cut surface down in the same cassette.Thus, the sampling of the hair follicles at different levels is directly visualized on the same glass slide, and, as a practical consequence, fewer histological sections and slides are required.Irrespective of whichever level of transverse sectioningof the punch biopsy specimen is chosen, the ultimate goal is to reach the . This is the site where the follicular units reside and affords the greatest amount of diagnostic findings, including the opportunity to perform accurate follicular counts and follicular ratios, which are critical, for example, in the assessment of non-scarring alopecia. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    21. 21. Tansverse sectioning permits a quantitative approach to diagnosis, it is imperfect, as it does not allow for a qualitative approach based on the repetitive criteria that can be obtained in vertically oriented sections. Clearly, there are limitations with both methods if they are used singly.However, transverse sections are undoubtedly superior to vertical sections in the study of the diameter of the hair follicles and the hair cycle, and allow visualization of virtually all the hair follicles present in the skin biopsy specimen, a feature particularly helpful in the evaluation of . Vertical sections find their importance in assessing the full thickness of the skin in every section, a feature that proves useful in the evaluation of . Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    22. 22. Transverse Section Vertical Section1. Quantitative approach 1. Qualitative approach2. Include all the hair follicles 2. Include 10% of hair follicle present in the biopsy3. Detectection of even focal 3. Sampling error pathology. 4. Assessing alopecias4. Quantitative data of follicular associated with interface cycling, as well as morphometric changes, lichenoid infiltrates, evaluation of the hair follicles throughout their entire length. and subcutaneous5. Evaluation of pathology. . 5. Evaluation of . Tuesday, September 11, 2012 Created by Dr Mohammad Manzoor Mashwani
    23. 23. The St John’s multiteam clinicopathological approachThe protocol adopted in our dermatopathology laboratory for processing scalp biopsies is based on the evaluation of combined transverse and vertical sections of two 4-mm punch biopsy specimens. This method is derived from that previously described by Elston et al.The choice of which combination of biopsies and sectioning to use may vary, based on the clinical data provided (scarring versus non-scarring alopecia). This approach acknowledges the advantage of both methods with the goal of obtaining the ‘best of two worlds’. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    24. 24. The St John’s multiteam clinicopathological approachA newly proposed model to avoid sample bias and toachieve optimal diagnostic yield is the St John’smultiteam clinicopathological approach.As biopsy site selection is crucial, this model is based upon between the 1. performing the biopsies in the clinic,2. the dermatopathology laboratory processing the specimens, and 3. the dermatopathologist trained in hair histopathology.These three elements are individually and collectivelyimportant factors required to reach histopathologicallythe final diagnostic interpretation. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    25. 25. The St John’s clinicopathologicalThus, the key factors that will enhance the histopathological diagnosis of alopecia include1. knowledge of the patient’s clinical history and2. the pattern of the alopecia,3. adequate choice of sampling ‘active’ areas, and4. two 4-mm punch biopsy specimens that include . In addition,5. numerous haematoxylin and eosin (H&E) serial sections and ancillary studies (directimmunofluorescence, periodic acid–Schiff (PAS), elastictissue and mucin stains) are carried out. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    26. 26. "mode of operation”)Two 4-mm punch biopsy specimens, both taken at theperipheral edge of the alopecia deemed to be the ‘active’area, are more likely to show diagnostic features.A biopsy of the central portion of the scar cannot be ofdiagnostic help, except for confirming a scarring process.Of the two specimens obtained, one is sectioned transverselywith the embedding cut surfaces indicated by red ink , while the second one is sectioned vertically, with 1 ⁄ 2 of it placed in Michel’s medium and sent for direct immunofluorescence studies, in accordance with the scarring alopecia PROTOCOL. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    27. 27. Figure 1. A, A 4-mm punch biopsyspecimen is sectioned horizontally.Figure 1. B, The two cut surfaces are red-inked before laboratory processing. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    28. 28. The transversely sectioned skin biopsy specimen is placed in a when grossing, and from the vertically sectioned one.Finally, in order to obtain a perfect horizontal section, special attention is also given to the final sectioning of the paraffin block at the microtome:the blade should be adjusted horizontally according to the specimen requirements. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    29. 29. Specimen 1 Scarring Alopecia Specimen 2 Two 4 mm Verticle Section Punch Biopsy 1/2 Specim 1 ens. / 2Transverse Scarring Alopecia H&Esection H&E IMF Figure 2. Schematic diagram of the St John’s scarring alopecia protocol. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    30. 30. Among the non-scarring alopecias, the most difficulttask for the histopathologist is to distinguish (androgenic alopecia) fromIn accord with the nonscarring alopecia protocol, the clinician will have taken one biopsy from the site of clinical involvement, usually the , and the second from an uninvolved area of the scalp, commonly theHair growth in the OCCIPUT is non-androgen dependent, and serves as the positive control of the patient’s normal hair characteristics. In this instance,both specimens are sectioned In cases of trichotillomania and AA, a specimen transversely sectioned will suffice. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    31. 31. Non-scarring Alopecia 1 2Transverse section Figure 3. Schematic diagram of the St John’s non-scarring alopecia protocol. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    32. 32. In both scarring and non-scarring alopecia protocols,the scalp biopsy specimens are until the histological ‘clues’required to make the diagnosis are identified.Ancillary stains, including PAS, elastic tissue and mucin stains, are also performed. If only one specimen is provided for evaluation, the ultimate choice of sectioning (transversely versus vertically) will depend on the clinical query provided. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    33. 33. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    34. 34. (cicatricial) alopeciaScarring (cicatricial) alopecia represents a complex group of hair disorders all characterized by having as a common final pathway .This process may be secondary, and due to numerous aetiologies or primary, where the cause and pathogenesis are largely unknown, but the target is the hair follicle itself.Biphasic scarring alopecias comprise another subset of permanent alopecias; in these cases, early non-scarring hair loss is followed by permanent follicle drop-out. This situation occurs in longstanding androgenic alopecia, AA and traction alopecia. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    35. 35. (cicatricial) alopeciaHistopathologically, primary scarring (cicatricial) alopecia is characterized by the presence of fibrous tissue replacing the hair follicles.This corresponds clinically to loss of hair follicle Ostia.The current working alopecia is that proposed by the North American Hair Research Society, in which the cicatricial alopecias are divided into several categories, including Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    36. 36. Table 1. Secondary scarring alopecia• Aplasia cutis congenita• Cicatricial bullous pemphigoid• Infectious (kerion, staphylococcal)• Neoplastic (primary, metastasis)• Connective tissue disease (morphea)• Trauma (burn)• Metabolic (amyloid, mucin)• Granulomatous (sarcoid) Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    37. 37. Column1 Column2 Column3 Column4 Column5Table 2. Classification of primary cicatricial alopecia*i.LymphocyticChronic cutaneous lupus erythematosusLichen planopilaris (LPP)Classic LPPFrontal fibrosing alopeciaGraham–Little syndromeClassic pseudopelade (Brocq)Central centrifugal cicatricial alopeciaAlopecia mucinosa Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    38. 38. Keratosis follicularis spinulosa decalvansii.NeutrophilicFolliculitis decalvansDissecting cellulitis ⁄ folliculitis (perifolliculitis capitisabscedens et suffodiens)iii.MixedFolliculitis (acne) keloidalisFolliculitis (acne) necroticaErosive pustular dermato Dr Mohammad Manzoor Mashwani Tuesday, September 11, 2012
    39. 39. i.Lymphocytic scarring alopeciasClinically, chronic cutaneous lupus erythematosus ischaracterized by , with , , , and . of patients progress to and a few patients may haveconcomitant lesions of Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    40. 40. i.Lymphocytic scarring alopecias Chronic Discoid Lupus ErythematosusHistopathological features include and a at the level of the infundibulum . may at times also be involved,in association with a perivascular and periappendageal superficial and deep . are characterized by and by that is highlighted by PAS. , and may also be seen.On vertical sections shows throughout the dermis. will with along the epidermal and follicular basement Mohammad Manzoor Mashwani Tuesday, September 11, 2012 Dr membrane zone.
    41. 41. A B C D EFigure 4. Chronic cutaneous (discoid) lupus erythematosus. A, Patch ofalopecia involving the anterior vertex. B, Transverse section showing only a fewhair follicles with follicular hyperkeratosis. C, Vacuolar-interface change of thefollicular epithelium with mild lymphoid cell infiltrate. D, Periodic acid–Schiffhighlights the perifollicular basement membrane zone thickening. E, Granular Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwanideposits of IgG along the basementmembrane zone.
    42. 42. Clinically, typical lesions of lichen planopilaris (LPP) present with atrophic, ill-defined patches of scarring alopecia with decreased follicular orifices.The margins of the alopecia, where the process is still active, will show perifollicular erythema with follicular scale.Cutaneous lesions of lichen planus may be present in up to 28% of cases, and with on the trunk and extremities, such as in the Graham– Little syndrome (also known as Piccardi–Lassuer– Graham Little syndrome) in which the hair loss not only affects the scalp, but also involves eyebrows, axillae and ⁄ or pubic hair. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    43. 43. Subsets of of LPP have been described in patients presenting with progressive fibrosing alopecia of the central scalp distributed in an androgenic pattern, or with progressive frontal recession, as initially reported by Kossard in postmenopausal women, and subsequently observed also in premenopausal women.In this entity, now designated as the changes of LPP appear not only to be localized to the scalp, but Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    44. 44. Histopathologically, the features of LPP and its variants are similar, irrespective of clinical presentation.In early lesions there is with a at the level of the infundibulum and isthmus.Occasionally, the interfollicular epidermis may have an associated lichenoid infiltrate. Inadvanced lesions, concentric lamellar perifollicular fibrosis occurs, and the lichenoid infiltrate ‘backs away’ from the follicle. Clefting between the follicular epithelium and the stroma may be seen in longstanding lesions.. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    45. 45. Mucinous perifollicular fibroplasia with absence of the interfollicular dermal mucinin the upper dermis has been described in vertical sections.End-stage LPP will show in a superficial dermal wedge-shaped scar which is better demarcated with the Verhoeff–vanGieson elastic stain.Direct immunofluorescence highlights the presence of staining with IgM .There is a ‘shaggy’ or linear deposition along the basement membrane zone of affected follicles, while the interfollicular epidermis is negative for immunoreactants Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    46. 46. A B CFigure 5. Lichen planopilaris ( ). A,Active margin of the alopecia with perifollicular erythema and follicular scale. B,Isthmus: a hair follicle showing interface alteration, perifollicular fibrosis and a mildperifollicular lymphoid cell infiltrate. C, Another hair follicle with a more advancedperifollicular concentric fibrosis, and with a lymphoid cell infiltrate that ‘backsaway’ from the 11, 2012follicle. Tuesday, September hair Dr Mohammad Manzoor Mashwani
    47. 47. A BFigure 6. , (vertical section).A, Superficial dermal wedge-shaped scar with loss of hair folliclesand residual arrector pili muscle. B, Verhoeff–van Gieson elasticstain demarcates the wedge-shaped morphology of the scar with lossof elastic fibres. 2012 Tuesday, September 11, Dr Mohammad Manzoor Mashwani
    48. 48. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    49. 49. CCCA CCCA or Most commonly seen in young to middle-aged women of African descent, but has been reported also in Black men. The aetioloy is unknown, but patients often report a history of involving a combination of hair straighteners and perms, oils, heat, chemicals and traction. No history of trauma associated with hairstyling is present in men.Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    50. 50. CCCA The possibility of genetic and autoimmune involvement in the has been postulated. , CCCA typically starts at the vertex or crown of the scalp, and spreads centrifugally ; it progresses slowly and in time burns itself out. In the early stages, it may show associated features of , with pustules, crusting and erythema with bacterial superinfection.Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    51. 51. perifollicular concentric , mild perifollicular and perivascular , , and, in terminal phases, .However, is found below the isthmus, root sheath and eccentric thinning of the follicular epithelium.Whereas desquamation of the inner root sheath is a feature normally observed at the isthmus, its . can be found in other inflammatory conditions of the scalp, including LPP. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    52. 52. CCCA , , and .In CCCA premature desquamation of the inner root sheath may be observed also in otherwiseIn vertical sections, thickened dermal elastic fibres in a hyalinized dermis have been reported.Numerous end-stage fibrous tracts replaced by amorphous connective tissue, consistent with follicular scars, are seen in the subcutaneous tissue. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    53. 53. B A C DFigure 7. . A, Patch of scarring alopecia involving the vertexand the crown. B, Isthmus: near-total loss of follicular units and sebaceous glands, withreplacement by follicular scars. C, Sub-isthmic region: high power of two residual hairfollicles both showing perifollicular fibrosis and eccentric thinning of the follicularepithelium. Premature desquamation of the inner root sheath is present in the follicle at 12o’clock. D, Subcutaneous tissue: numerous end-stage fibrous tracts (left); the same two hair Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwanifollicles of C are herepresent, showing the previously described features (right).
    54. 54. An idiopathic and slowly progressive form of cicatricial alopecia, presenting with multiple small alopecic patches on the vertex and parietal areas, in a pattern that has been defined as : end-stage scarring alopecia, with concentric perifollicular lamellar fibrosis, loss of sebaceous glands, loss of follicular units with follicular scars and a minimal residual inflammatory cell infiltrate.Thus, it is still debated whether this is an entity per se, or instead represents the end stage of other scarring alopecias, such as chronic cutaneous lupus erythematosus or LPP. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    55. 55. Folliculitis decalvans is a primary neutrophilic cicatricial alopecia affecting middle-aged adults. and the have been linked to its pathogenesis. , the alopecia is localized to the vertex and occipital area, with follicular pustules, perifollicular erythema and tufting. early lesions show features characteristic of an acute dense dermal perifollicular and later on, as the follicle ruptures, an intrafollicular and perifollicular is seen. Gram stain will commonly show Gram-positive cocci of S. aureus . Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    56. 56. Perifollicular fibrosis with fibrous tracts replacing the hair follicles, follicular tufting and interstitial dermal fibrosis are all features observed in .This entity has been linked to CCCA, as overlapping features are seen.In contrast to dissecting cellulitis, are present. similar to that observed in LPP, has been described in vertical sections. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    57. 57. A B D CFigure 8. Folliculitis decalvans. A, Numerous follicular papules andpustules with erythema and scarring at the vertex. B, Neutrophilic microabscesswithin a partially destroyed follicular infundibulum. C, Gram stain highlightsnumerous Gram-positive bacterial colonies of Staphylococcus aureus. D, Latestage: dense dermal fibrosis and mixed inflammatory cell infiltrate with follicular Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwanitufting.
    58. 58. 2. (perifolliculitis capitis abscedens et suffodiens)This entity commonly occurs in young men of African descent. It is characterized clinically by large fluctuant nodules that begin on the occiput or vertex, but may extend throughout the entire scalp, and is often associated with sinus tracts and purulent discharge.Its pathogenesis is poorly understood, but it has been associated with disorders of the follicular occlusion triad (hidradentitis , suppurativa and acne conglobata). As the depth of dissection may spare a few hair follicles overlying it, the scarring alopecia ordinarily involves only half of the hair follicles. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    59. 59. 2. , there is a dense deep dermal and subcutaneous, predominately with and .Characteristic is the formation of sinus tracts lined by squamous epithelium with surrounding dense fibrosis.CommentAs a footnote to the foregoing description of the scarring alopecias, it has been reported that the ratio of lymphocytic to neutrophilic alopecias is 4:1, with the former favouring middle-aged women, and the latter middle-aged men. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    60. 60. iii.Mixed scarring alopeciasThis entity is also known as acne keloidalis nuchae, as it usually occurs on the occipital scalp in young men of African descent. Clinically, it is characterized by follicular papules and pustules that progress to fibrosis, with keloid formation.Histopathologically, early in the disease there is follicular dilatation with neutrophils, and follicular rupture with perifollicular abscesses.Late lesions show perifollicular granulomas around naked hair shafts mixed with a lymphoplasmacellular cell infiltrate, andhypertrophic scar with broad eosinophilic hyalinized keloidal collagen bundles. Sebaceous glands are absent in all stages of the folliculitis. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    61. 61. II.Non-scarring alopeciasAndrogenic alopecia is the most common type of hair loss. It is a disorder of dominant inheritance with variable penetrance, affecting approximately half of the population by the age of 50 years, of both sexes.The disease represents an end-organ androgen sensitivity of hair follicles in which terminal hair follicles are genetically programmed, under the influence of androgens, to undergo miniaturization.Clinically, it is a patterned alopecia, in that it is characterized by bitemporal recession and vertex balding in men, and in women (female pattern hair loss) by diffuse hair thinning of the crown with an intact frontal hairline. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    62. 62. Histopathologically, the use of transverse sections is the most valuable method to reach a diagnosis, as all the hair follicles can be visualized. While the total number of hair follicles is unchanged, there is , with a variation in size of the hair follicles and .The terminal (T) to vellus (V) ratio is T:V = <4:1 (normal scalp T:V = 7:1). A ratio of T:V = 3:1 or less is considered to be diagnostic.This ratio does not take into account, however, the intermediate hairs that have a hair shaft diameter in between the terminal and vellus hair follicles, and are currently classified as terminal in follicular counts. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    63. 63. Other findings include an increased number of telogen hairs, decreased numbers of terminal hair follicles in the subcutaneous fat, variation of shaft diameter, and increased numbers of fibrous tracts.A mild peri-infundibular lymphocytic cell infiltrate and perifollicular collagen deposition are present in 40% of cases.A relationship between increased mast cells and perifollicular collagen deposition has been reported. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    64. 64. B A CFigure 9. Female pattern hair loss. A, Hair ‘thinning’at the vertex. B, Isthmus: hair follicle miniaturizationwith variation in hair follicle size.C, Mildperifollicular lymphoid cell infiltrate. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    65. 65. Telogen effluvium is a diffuse form of alopecia, in which the hair shedding may be acute or chronic.Clinically, acute telogen effluvium can occur in both sexes and be triggered by numerous precipitating factors (major surgery, injury, severe illness, childbirth, crash diet and numerous medications); there is no obvious trigger factor in chronic telogen effluvium. Chronic telogen effluvium is characterized by diffuse scalp hair thinning in middle-aged women, and has a prolonged and fluctuating course. It may be confused with female pattern hair loss, but is distinguished from it by the lack of hair follicle miniaturization. However, overlap cases have been reported. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    66. 66. Histopathology of acute telogen effluvium shows a normal number of hair follicles with no miniaturization,and resembles normal scalp.In chronic telogen effluvium there is also a normal number of hair follicles, but with an increased number (20–30%) of (normal scalp 10% telogen hairs), and some evidence of miniaturization if it is superimposed on an evolving androgenic alopecia. The standard cut-off points for the differential diagnosis between chronic telogen effluvium and female pattern hair loss are T:V = >8:1 for chronic telogen effluvium, andT:V = <4:1 for female pattern hair loss, but this difference does not include the presence of intermediate hair follicles, which would suggest early evolving female pattern hair loss.Moreover, concentric layers of perifollicular collagen have also been observed in 10% of cases of androgenic alopecia. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    67. 67. Alopecia areata (AA) is thought to be an organ- specific autoimmune disorder.It commonly occurs in association with other autoimmune diseases such as vitiligo and thyroiditis, and the lifetime risk of acquiring AA is approximately 1.7%. It equally affects males and females at all ages, and 60% of patients before the age of 20 years. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    68. 68. A B D CFigure 10. Alopecia areata. A, Multiple patches of hair loss with‘exclamation-point’ hairs. B, Sub-isthmic region: a hair bulb withperibulbar lymphoid cell infiltrate (‘swarm of bees’). C, ‘Shift out ofanagen’: all the hair follicles are in telogen phase. D, Anagen-like nanogenhair follicle with no central hair shaft, and perifollicular lymphocytes. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    69. 69. Clinically, it is characterized by sudden onset of patches of nonscarring hair loss, with ‘exclamation-point’ hairs.It may undergo spontaneous remissions or exacerbations and become extensive to involve the entire scalp ( ) and body hair ( ). (pitting, thickening and ridging) may be seen in 10–66% of cases. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    70. 70. Histopathologically, the morphological features are dependent upon the duration of the episode, and may be divided into early active (acute and subacute) and longstanding (chronic) stages.The early active stage is characterized by a peribulbar lymphoid cell infiltrate (‘swarm of bees’) affecting the terminal hair follicles.This infiltrate can be quite prominent and may invade the follicular epithelium and the matrix, as well as extend above the hair bulb and into fibrous tracts. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    71. 71. Initially, the terminal hairs are attacked, but subsequently also the vellus hairs become involved.Eosinophils and plasma cells may be present.There is a 70–90% ‘shift out of anagen’ of the hair follicles into catagen or telogen phase but the number of hair follicles is unchanged.An increased number of vellus hair follicles is also present.Additional features secondary to hair matrix damage include trichomalacia (dysmorphic hair shafts), and melanin pigment casts in fibrous tracts. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    72. 72. In longstanding (chronic) stages, with repeated episodes, the peribulbar lymphoid cell infiltrate also involves miniaturized hairs. The majority of the hair follicles will be in catagen telogen phase, with the resence of nanogen hair follicles.These are miniaturized, rapidly cycling hair follicles with mixed features of anagen, catagen and telogen, which may contain remnants of the inner root sheath, but lack hair shafts. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    73. 73. Many empty infundibula may be seen, corresponding to the total scalp hair loss.In a small percentage of cases (10%) with a long history of repeated attacks there is perifollicular fibrosis with follicular dropout.The main differential diagnosis is with the nonscarring variant of SLE, where peribulbar lymphoid cells are seen as in AA. Distinguishing features are the presence in the former of vacuolar-interfacechanges of the infundibular epithelium, a perieccrineand perivascular lymphoplasmacellular cell infiltrate,and increased interstitial mucin, particularly if in the deep dermis. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    74. 74. Trichotillomania is characterized by the compulsive intentional pulling or twisting of the hair.This hair loss disorder may reflect a background of emotional instability, and often occurs in children. A biopsy is an important tool to provide the clinician objective support for the diagnosis, as often both the child and parents deny hair-pulling as a cause of the hair loss. Clinically, the patients present with diffuse or bizarre- shaped patches of hair loss.The hair shafts have various lengths, due to different points of fracture of the hair shafts or to the hair being pulled at different times.. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    75. 75. Histopathologically, this is a non-inflammatory non- scarring alopecia in which the morphological changes are those of follicular damage ( ) secondary to the external insult, with distortion of the hair follicle anatomy and with perifollicular and intrafollicular haemorrhage.. Additional findings include melanin pigment casts, loss of hair shafts, and trichomalacia, where the hair shaft is dysmorphic, with incomplete cornification and irregular pigmentation .The number of hair follicles is normal, with an increased number of catagen ⁄ telogen hair follicles, and without significant inflammation. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    76. 76. A B C D E F G H Figure 11. Trichotillomania. A, Subtle diffuse alopecia of the scalp. B, Isthmus: absence of inflammation and hair follicles with signs of follicularFigure 11. Trichotillomania. A, Subtle diffuse alopecia of the scalp. B, Isthmus: absence of damage (trichomalacia). C, D, Irregularly shaped hair follicles. E, Intrafollicular haemorrhage. F, A melanin pigment cast within a hair shaft. G, A hair follicle with loss of the hair shaft. H, A severely distorted hair shaftinflammation and hair follicles with signs of follicular damage (trichomalacia). C, D, Irregularlyshaped hair follicles. E, Intrafollicular haemorrhage. F, A melanin pigment cast within a hair Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwanishaft.
    77. 77. Traction alopecia, like trichotillomania, is a noninflammatory, non-scarring alopecia secondary to , which in this case is related, and is seen in of descent.Clinically, the hair loss is often seen at the margins of the scalp, involving the frontal, temporal and parietal regions. In early traction alopecia the hair loss is temporary, provided that the damaging noxa is suspended, whereas in late ‘burnt out’ alopecia, where the excessive traction persists, the hair loss is permanent. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    78. 78. Histopathologically, the features observed in early traction alopecia are similar to those seen in trichotillomania, whereas in late traction alopecia there is marked loss of the terminal follicles with preservation of the vellus hairs and sebaceous glands.The follicular units at the isthmus are replaced by fibrous tissue, consistent with a scarring process, and thus permanent alopecia. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
    79. 79. Tuesday, September 11, 2012 Dr Mohammad Manzoor Mashwani
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