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Scarring Alopecia Dr Amir Hossein Siadat M.D.. Alopecia Scaring (Irreversible) None Scaring (Reversible)

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Presentation on theme: "Scarring Alopecia Dr Amir Hossein Siadat M.D.. Alopecia Scaring (Irreversible) None Scaring (Reversible)"— Presentation transcript:

1 Scarring Alopecia Dr Amir Hossein Siadat M.D.

2 Alopecia Scaring (Irreversible) None Scaring (Reversible)

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4 The term cicatricial or scarring alopecia : The term cicatricial or scarring alopecia : potential of permanent destruction of the hair follicle. potential of permanent destruction of the hair follicle. Clinically: there is effacement of follicular orifices Clinically: there is effacement of follicular orifices

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7 Dissecting Cellulites: Synonyms: Synonyms: dissecting folliculitis dissecting folliculitis dissecting perifolliculitis. dissecting perifolliculitis. most commonly begin on most commonly begin on the vertex the vertex or occiput or occiput

8 Presents as relatively painless, deep-seated, firm to fluctuant nodules and abscesses. as relatively painless, deep-seated, firm to fluctuant nodules and abscesses.

9 The nodules and deep abscesses commonly extend to form: The nodules and deep abscesses commonly extend to form: a complex serpiginous or reticulate pattern of interconnecting sinuses and abscesses filled with purulent, seropurulent, or hemopurulent material. a complex serpiginous or reticulate pattern of interconnecting sinuses and abscesses filled with purulent, seropurulent, or hemopurulent material.

10 Pressure applied to one nodule or abscess: can cause purulent exudate to emerge several centimeters away. Pressure applied to one nodule or abscess: can cause purulent exudate to emerge several centimeters away. The epidermal surface overlying the sinuses and nodules may or may not be erythematous and scaly. The epidermal surface overlying the sinuses and nodules may or may not be erythematous and scaly. Scattered superficial follicular pustules are frequently present on the surface of the nodules. Scattered superficial follicular pustules are frequently present on the surface of the nodules. Hypertrophic and keloidal scarring may occur in affected areas. Hypertrophic and keloidal scarring may occur in affected areas.

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14 Dissecting cellulitis of the scalp: Dissecting cellulitis of the scalp: more common in young to middle-aged black men. more common in young to middle-aged black men. It may occur simultaneously with acne conglobata and hidradenitis suppurativa. This complex is known as the follicular occlusion triad. It may occur simultaneously with acne conglobata and hidradenitis suppurativa. This complex is known as the follicular occlusion triad.

15 Pathology of Dissecting Cellulities Biopsy specimens from well-developed fluctuant nodules and sinuses reveal large, perifollicular, mid to deep reticular dermal abscesses often rich in plasma cells. Biopsy specimens from well-developed fluctuant nodules and sinuses reveal large, perifollicular, mid to deep reticular dermal abscesses often rich in plasma cells. Abscesses are located immediately adjacent to follicles, between follicles in the mid and deep reticular dermis, and subjacent to follicles within the superficial subcutis. Abscesses are located immediately adjacent to follicles, between follicles in the mid and deep reticular dermis, and subjacent to follicles within the superficial subcutis.

16 culture of abscess fluid and overlying pustules: culture of abscess fluid and overlying pustules: frequently sterile frequently sterile may yield Staphylococcus epidermidis or S. aureus. may yield Staphylococcus epidermidis or S. aureus. The pathogenic role of these organisms is uncertain because chronic antibiotic therapy is frequently not curative. The pathogenic role of these organisms is uncertain because chronic antibiotic therapy is frequently not curative.

17 Treatment of Dissecting Cellulities Initial therapy : oral antibiotics tetracycline (1 -2 g per day), doxycycline (200 mg per day), and minocycline (50-200 mg per day). Initial therapy : oral antibiotics tetracycline (1 -2 g per day), doxycycline (200 mg per day), and minocycline (50-200 mg per day). Combination antibiotic therapy of cephalexin (1 g per day) and rifampin (600 mg per day) has been helpful. Combination antibiotic therapy of cephalexin (1 g per day) and rifampin (600 mg per day) has been helpful.

18 Intralesional corticosteroid injections (triamcinolone acetonide, 40 mg/mL) can be helpful in noninfectious nodules and sinuses. Intralesional corticosteroid injections (triamcinolone acetonide, 40 mg/mL) can be helpful in noninfectious nodules and sinuses. Oral corticosteroids tapered to low-dose alternate-day usage may also be helpful. Oral corticosteroids tapered to low-dose alternate-day usage may also be helpful. Isotretinoin (1 mg/kg per day) has been effective in some reported cases. Isotretinoin (1 mg/kg per day) has been effective in some reported cases.

19 Oral zinc has also been reported to be effective. Oral zinc has also been reported to be effective. Surgical therapy may be considered in advanced cases but is usually quite deforming. Surgical therapy may be considered in advanced cases but is usually quite deforming. Complete scalp excision, carbon dioxide laser excision, or more conservative local incision and drainage of individual lesions maybe considered. Complete scalp excision, carbon dioxide laser excision, or more conservative local incision and drainage of individual lesions maybe considered.

20 Acne Keloidalis Nuchae

21 destructive pustulofollicular process destructive pustulofollicular process occurs on the occipital scalp and posterior neck occurs on the occipital scalp and posterior neck primarily affects young black men. primarily affects young black men.

22 begin as discrete follicular pustules and papules begin as discrete follicular pustules and papules progress to form less discrete, large, exophytic keloidal nodules and plaques largely devoid of hair. progress to form less discrete, large, exophytic keloidal nodules and plaques largely devoid of hair.

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27 The severity : The severity : few small follicular pustules and papules with minimal alopecia  extensive keloidal plaque formation with prominent patterned alopecia few small follicular pustules and papules with minimal alopecia  extensive keloidal plaque formation with prominent patterned alopecia Similar lesions may occur in the beard area of patients with pseudofolliculitis barbae. Similar lesions may occur in the beard area of patients with pseudofolliculitis barbae.

28 Pathogenesis and Etiology The etiology : unknown The etiology : unknown acne keloidalis nuchae : a variant of acne vulgaris acne keloidalis nuchae : a variant of acne vulgaris Bacteria may be isolated. Bacteria may be isolated.

29 Repetitive low-grade trauma Repetitive low-grade trauma friction by football helmets, other headware, and collars friction by football helmets, other headware, and collars Closed shaving of posterior scalp and neck hair Closed shaving of posterior scalp and neck hair

30 Treatment of Acne Keloidalis Nuchae Tetracycline, 1 g per day Tetracycline, 1 g per day Doxycycline 200mg/day Doxycycline 200mg/day Minocycline 50-100mg/day Minocycline 50-100mg/day Erythromycin 1g per day Erythromycin 1g per day 2. Topical antibiotics. 2. Topical antibiotics. a.Erythromycin solution 2% b. Clindamycin solution 1% 3. Tretinoin cream or gel 0.025%-0.1% 3. Tretinoin cream or gel 0.025%-0.1% 4. Benzoyl peroxide gel or lotion 5%-10% 4. Benzoyl peroxide gel or lotion 5%-10% 5. Intralesional corticosteroid injections (triamcinolone acetonide, 3- 40 mg/mL) 5. Intralesional corticosteroid injections (triamcinolone acetonide, 3- 40 mg/mL) 6. Surgical excision for large exophytic keloidal 6. Surgical excision for large exophytic keloidal

31 Discoid Lupus Erythematosus Discoid lupus erythematosus is a common lesion Discoid lupus erythematosus is a common lesion typically found in young to middle-aged adults typically found in young to middle-aged adults 2:1 female/male predominance. 2:1 female/male predominance. Age at onset : between 20-60 years Age at onset : between 20-60 years

32 most commonly located on the most commonly located on the face, scalp, and inner ear face, scalp, and inner ear 50% of patients with DLE have scalp lesions 50% of patients with DLE have scalp lesions 10% of patients: only scalp involvement 10% of patients: only scalp involvement

33 Patients with discoid lupus: Patients with discoid lupus: uncommonly progress to involvement with systemic lupus erythematosus. uncommonly progress to involvement with systemic lupus erythematosus. 15% of patients with SLE may exhibit DLE 15% of patients with SLE may exhibit DLE

34 Early lesions: Early lesions: small erythematous papules or irregular small, scaly plaques  round to irregular shaped atrophic, sclerotic plaques. small erythematous papules or irregular small, scaly plaques  round to irregular shaped atrophic, sclerotic plaques.

35 Thick,adherent scale frequently develops that when removed reveals keratinous plugs on its undersurface. Thick,adherent scale frequently develops that when removed reveals keratinous plugs on its undersurface.

36 When DLE is active: When DLE is active: surrounding borders erythematous and violaceous. surrounding borders erythematous and violaceous. symptomatic including pruritus, burning and pain. symptomatic including pruritus, burning and pain. Both hyperpigmentation and hypopigmentation is frequently seen. Both hyperpigmentation and hypopigmentation is frequently seen.

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55 Central atrophy and telangiectasia eventually become prominent. Central atrophy and telangiectasia eventually become prominent. Hypertrophic DLE is very destructive form of DLE that sometime occur on the scalp. Hypertrophic DLE is very destructive form of DLE that sometime occur on the scalp. This form is greatly elevated, verrucous, red-violet plaues with thick adherent scale. This form is greatly elevated, verrucous, red-violet plaues with thick adherent scale.

56 Vacuolar interface alteration of the epidermis and follicular epithelium is characteristic of discoid lupus. Vacuolar interface alteration of the epidermis and follicular epithelium is characteristic of discoid lupus. Dyskeratotic keratinocytes are often seen at the dermoepidermal junction and within the outer follicular epithelium, but the dyskeratosis is usually not as conspicuous as in lichen planopilaris. Dyskeratotic keratinocytes are often seen at the dermoepidermal junction and within the outer follicular epithelium, but the dyskeratosis is usually not as conspicuous as in lichen planopilaris.

57 The epidermis ranges from atrophic to acanthotic (hypertrophic discoid lupus). The epidermis ranges from atrophic to acanthotic (hypertrophic discoid lupus). Overlying orthokeratotic hyperkeratotic stratum corneum with focal parakeratosis is usually present. Overlying orthokeratotic hyperkeratotic stratum corneum with focal parakeratosis is usually present.

58 DIF is positive for granular IgG but also occasionally for IgM and IgA. DIF is positive for granular IgG but also occasionally for IgM and IgA. Complement at DEJ is observed in 75%- 90% of cases. Complement at DEJ is observed in 75%- 90% of cases.

59 The clinical diagnosis of DLE is usually not difficult. The clinical diagnosis of DLE is usually not difficult. The previously described clinical features and distribution of lupus lesions are usually quite characteristic. The previously described clinical features and distribution of lupus lesions are usually quite characteristic. The presence of classic lesions at other sites (ears, face, neck, chest, and arms) can aid in the diagnosis. The presence of classic lesions at other sites (ears, face, neck, chest, and arms) can aid in the diagnosis.

60 A routine skin biopsy to confirm the clinical diagnosis. A routine skin biopsy to confirm the clinical diagnosis. DIF in most cases is usually not necessary. DIF in most cases is usually not necessary. R/O SLE R/O SLE

61 relatively limited involvement: relatively limited involvement: Topical and intralesional corticosteroids Topical and intralesional corticosteroids ILof triamcinolone acetonide, 3-10mg/mL: ILof triamcinolone acetonide, 3-10mg/mL: into active erythematous and violaceous lesions into active erythematous and violaceous lesions

62 extensive scalp or cutaneous involvement is present and is not responding to local therapy: extensive scalp or cutaneous involvement is present and is not responding to local therapy: consideration may be given to systemic therapy. consideration may be given to systemic therapy. Effective systemic medications : Effective systemic medications : oral prednisone and chloroquines. oral prednisone and chloroquines.

63 Lichen planopilaris:

64 Lichen planopilaris: Lichen planopilaris: more common in women between 30 and 70 years of age. more common in women between 30 and 70 years of age. Early lesions : acuminate, spinous, and hyperkeratotic follicular papules with perifollicular erythema. Early lesions : acuminate, spinous, and hyperkeratotic follicular papules with perifollicular erythema.

65 The hair follicles are subsequently destroyed, yielding atrophic,irregular, especially angular or polygonal shaped patches of alopecia with similar follicular papules at the periphery of the patch of alopecia. The hair follicles are subsequently destroyed, yielding atrophic,irregular, especially angular or polygonal shaped patches of alopecia with similar follicular papules at the periphery of the patch of alopecia. In addition, similar spinous follicular papules as well as more typical lichen planus lesions are frequently present elsewhere on the body, including mucous membranes and nails. In addition, similar spinous follicular papules as well as more typical lichen planus lesions are frequently present elsewhere on the body, including mucous membranes and nails.

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78 50% to 70% of patients with lichen planopilaris : have lichen planus elsewhere on the body. 50% to 70% of patients with lichen planopilaris : have lichen planus elsewhere on the body. The main symptom: pruritus. The main symptom: pruritus.

79 initial perifollicular erythema  the follicular acuminate papules initial perifollicular erythema  the follicular acuminate papules smooth patches of hair loss, and in the scalp, scarring alopecia is a common sequela. smooth patches of hair loss, and in the scalp, scarring alopecia is a common sequela. End stage : a permanent loss of pilosebaceous units and may clinically resemble pseudopelade. End stage : a permanent loss of pilosebaceous units and may clinically resemble pseudopelade.

80 Pathology Lichenoid interface alteration of the epidermis and follicular epithelium is characteristic of lichen planopilaris. Lichenoid interface alteration of the epidermis and follicular epithelium is characteristic of lichen planopilaris.

81 Diagnosis of Lichen planopilaris: The combination of acuminate follicular papules in an area of alopecia and signs of lichen planus elsewhere on the body is virtually diagnostic of lichen planopilaris. The combination of acuminate follicular papules in an area of alopecia and signs of lichen planus elsewhere on the body is virtually diagnostic of lichen planopilaris.

82 Chronic cutaneous lupus erythematosus is generally not confused with lichen planopilaris. Chronic cutaneous lupus erythematosus is generally not confused with lichen planopilaris. Lesions of discoid lupus erythematosus frequently exhibit follicular plugging and follicular accentuation, but the interfollicular skin is more inflamed and sclerotic than in lichen planopilaris. Lesions of discoid lupus erythematosus frequently exhibit follicular plugging and follicular accentuation, but the interfollicular skin is more inflamed and sclerotic than in lichen planopilaris.

83 Course: can last several months to several years. Course: can last several months to several years. Therapy : controlling perifollicular inflammation and pruritus. Therapy : controlling perifollicular inflammation and pruritus. the most effective therapies in LPP.:High- potency topical corticosteroids and oral corticosteroids the most effective therapies in LPP.:High- potency topical corticosteroids and oral corticosteroids

84 Oral corticosteroids (30 to 40 mg per day) with subsequent taper have been reported to be the most efficacious therapy for controlling pruritus and hair loss. Oral corticosteroids (30 to 40 mg per day) with subsequent taper have been reported to be the most efficacious therapy for controlling pruritus and hair loss.

85 Erosive Pustular Dermatosis of the Scalp: uncommon erosive and pustular process affecting the scalp uncommon erosive and pustular process affecting the scalp most commonly found in elderly white females most commonly found in elderly white females usually in the 6th to 9th decades. usually in the 6th to 9th decades.

86 begins on the: begins on the: crown, vertex, or lateral scalp crown, vertex, or lateral scalp pustules and erosions with gradual increase in size over a period of months to years. pustules and erosions with gradual increase in size over a period of months to years. Thick purulent crusts frequently overlie erosions Thick purulent crusts frequently overlie erosions

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90 The process is limited to the scalp. The process is limited to the scalp. frequently occur in areas of recent or remote trauma to the scalp. frequently occur in areas of recent or remote trauma to the scalp.

91 Pathology The microscopic features of erosive pustular dermatosis of the scalp are not specific. The microscopic features of erosive pustular dermatosis of the scalp are not specific. Epidermal and superficial dermal ulceration with subjacent mixed inflammatory infiltrates and abscesses have been reported. Epidermal and superficial dermal ulceration with subjacent mixed inflammatory infiltrates and abscesses have been reported. Direct immunofluorescence microscopy of lesional and perilesional skin is negative. Direct immunofluorescence microscopy of lesional and perilesional skin is negative. No circulating autoantibodies are seen by indirect immunofluorescence microscopy. No circulating autoantibodies are seen by indirect immunofluorescence microscopy.

92 pathogenesis of erosive pustular dermatosis: is unknown. pathogenesis of erosive pustular dermatosis: is unknown. Staphylococcus species, Pseudomonas aeruginosa, Proteus mirabilis,Candida species, and Aspergillus ? Staphylococcus species, Pseudomonas aeruginosa, Proteus mirabilis,Candida species, and Aspergillus ? may represent secondary colonization. may represent secondary colonization.

93 Treatment Treatment is difficult. Treatment is difficult. If bacterial cultures are +, If bacterial cultures are +, appropriate oral antibiotics are suggested. appropriate oral antibiotics are suggested. the most efficacious therapy : the most efficacious therapy : Oral Oral intralesional intralesional potent topical corticosteroids potent topical corticosteroids

94 Alopecia Mucinosa (Follicular Mucinosis) Alopecia Mucinosa (Follicular Mucinosis)

95 Mucin deposition and subsequent degeneration of hair follicles : patches and plaques of alopecia. Mucin deposition and subsequent degeneration of hair follicles : patches and plaques of alopecia. most commonly involves: most commonly involves: the face, scalp, and neck, but involvement of extremities may also occur. the face, scalp, and neck, but involvement of extremities may also occur. Clinically detectable alopecia is most often present in those lesions involving the scalp and face. Clinically detectable alopecia is most often present in those lesions involving the scalp and face.

96 have variable morphology ranging have variable morphology ranging from indurated, scaling, erythematous plaques and nodules to noninflammatory circular patches resembling alopecia areata. from indurated, scaling, erythematous plaques and nodules to noninflammatory circular patches resembling alopecia areata.

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104 Age at onset ranges from 2 to 80 years. Age at onset ranges from 2 to 80 years. Two clinically distinct groups of alopecia mucinosa (follicular mucinosis) exist: lymphoma associated and non-lymphoma associated. Two clinically distinct groups of alopecia mucinosa (follicular mucinosis) exist: lymphoma associated and non-lymphoma associated.

105 Mycosis fungoides is the most common lymphoproliferative disorder associated with alopecia mucinosa and follicular mucinosis and usually occurs in patients 30 years of age and older. Mycosis fungoides is the most common lymphoproliferative disorder associated with alopecia mucinosa and follicular mucinosis and usually occurs in patients 30 years of age and older.

106 The reported incidence of lymphoma in alopecia mucinosa ranges from 9.4% to 60%. The reported incidence of lymphoma in alopecia mucinosa ranges from 9.4% to 60%. Close follow up of patients with repeated biopsy is therefore required. Close follow up of patients with repeated biopsy is therefore required.

107 Non-lymphoma associated alopecia mucinosa: Non-lymphoma associated alopecia mucinosa: frequently occurs in children and young adults and is usually self-limiting without resultant permanent alopecia. frequently occurs in children and young adults and is usually self-limiting without resultant permanent alopecia. In addition, Hodgkin's lymphoma may rarely occur in association with childhood alopecia mucinosa. In addition, Hodgkin's lymphoma may rarely occur in association with childhood alopecia mucinosa.

108 Patholgy The earliest findings are mucin deposits within the outer root sheath. The earliest findings are mucin deposits within the outer root sheath. The keranocytes of the outer root sheath are splayed apart by the mucin and may appear stellate. The keranocytes of the outer root sheath are splayed apart by the mucin and may appear stellate. Although no single hislologic criterion has been shown Although no single hislologic criterion has been shown to predict the future development of lymphoma, the presence of a band-like, atypical lymphocytic infiltrate near the dermoepidermal junction with significant epidermotropism is more commonly seen in lymphoma- associated follicular mucinosis. to predict the future development of lymphoma, the presence of a band-like, atypical lymphocytic infiltrate near the dermoepidermal junction with significant epidermotropism is more commonly seen in lymphoma- associated follicular mucinosis.

109 Treatment of alopecia mucinosa: Non-lymphoma associated : Non-lymphoma associated : Topical Coticosteroids Topical Coticosteroids Intralesional Coticosteroids Intralesional Coticosteroids lymphoma associated : lymphoma associated : Treatment of lymphoma Treatment of lymphoma

110 Have a good time Have a good time

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