Ch 88: Cicatricial Alopecia Flashcards
All scarring alopecias are characterized by clinically by _______, and pathologically by _______.
Clinical: (+) loss of follicular ostia
Pathological: (+) replacement of hair follicles with fibrous tissue
Etiology and pathogenesis of Primary Cicatricial Alopecias
Not completely understood
Inflammatory infiltrate affecting infundibulum and isthmus –> damage to bulge and sebaceous glands –> incomplete hair cycle –> chronic follicular inflammation and foreign-body reaction
Cells responsible for renewal of the upper part of the hair follicle and sebaceous glands, as well as restoration of the lower cyclical component of the follicles at the onset of a new anagen period
Pluripotent hair follicle stem cells (found in the bulge region of the isthmus)
Sites affected first in Primary Cicatricial Alopecia
Central and parietal scalp
Clinical presentation of Primary Cicatricial Alopecia
(+) isolated alopecic patches with atrophy and lack of follicular ostia
(+) diffuse/perifollicular erythema
(+) follicular hyperkeratosis, pigment changes, tufting, pustules
*clinically visible inflammation may be absent, seen on HP in the deep dermis and SQ
Diagnostic Tools
Dermatoscope
Punch biopsy (4mm), with SQ
- 1 horizontal: H&E, elastin (acid alcohololic orcein), mucin, PAS
- 1 vertical: transverse cut, IHC
Findings on dermoscopy (10x)
(+) absence of follicular ostia
(+) perifollicular erythema
(+) follicular hyperkeratosis
Prognosis once hair follicle is destroyed and replaced by fibrous tissue:
No hope for hair regrowth. :(
Requirements for hair restoration surgery (eg. hair transplantation, scalp reduction)
No disease activity should occur on the scalp for at least 1 year after therapy
Classifications of Primary Cicatricial Alopecias
Lymphocytic
Neutrophilic
Mixed
Lymphocytic Primary Cicatricial Alopecias
Chronic cutaneous LE (DLE) Lichen planopilaris Classic lichen planopilaris Frontal fibrosing alopecia Graham-Little syndrome Classic pseudopelade of Brocq Central centrifugal cicatricial alopecia Alopecia mucinosa Keratosis follicularis spinulosa decalvans
Neutrophilic Primary Cicatricial Alopecias (higher risk of limited graft survival and disease recurrence)
Folliculitis decalvans
Dissecting cellulites/folliculitis (perifolliculitis abscedens et suffodiens)
Mixed Cicatricial Alopecias
Folliculitis (acne) keloidalis
Folliculitis (acne) necrotica
Erosive pustular dermatitis
Most common cause of inflammatory Cicatricial Alopecia
DLE, Lichen Planopilaris
Percentage of patients with DLE that will progress to SLE
Children: 26 to 31%
Adults: 5 to 10%
Conditions associated with DLE
Verruciform xanthoma
Papulonodular dermal mucinosis
Histopathologic findings of DLE
(+) lymphocyte-mediated interface dermatitis with vacuolar degeneration of the basal cell layer and necrotic keratinocytes
(+) thickening of basement membrabce
(+) destruction of sebaceous glands and elastic fibers
DIF findings of DLE
(+) linear, granular deposition of IgG and C3 at the DEJ
Treatment for DLE
Baseline: ophthalmologic examination, CBC
Hydroxychloroquine (adults: 200 to 400mg OD, children: 4 to 6mg/kg)
Bridge tx: oral Prednisone (1 mg/kg) over the first 8 weeks of treatment
Others: TAC ILSI 10mg/mL q4-6wks, oral acitretin and isotretinoin, MMF, MTX, azathioprine
Classic LPP most commonly seen in
Women, fifth decade
Clinical manifestations of Graham-Little Syndrome
LPP of the scalp
Noncicatricial alopecia of the eyebrows, axilla, groin
Keratosis pilaris
Frontal Fibrosing Alopecia (FFA) most commonly seen in
postmenopausal women
Triggers for lichenoid drug eruptions
Gold, antimalarials, captopril, quinine, thiazide diuretics
Sites affected first in Classic LPP
Crown and vertex of scalp
Classic LPP vs DLE
Classic LPP: alopecic areas are smaller, irregularly shaped, and interconnected (may appear reticulated), (+) itching, burning sensation, sensitivity of scalp
Clinical presentation of FFA
(+) frontal, band-like or circumferential scarring alopecia, with (+) follicular hyperkeratosis and perifollicular/diffuse erythema
(+) sparing of few hairs on original frontal hairline
(+) alopecia of the eyebrows
Histopathologic findings of LPP/FFA
(+) lymphocytic infiltrate and interface dermatitis in and around upper permanent part of hair follicle
(-) vascular plexus not affected by inflammation, no mucin deposits –> both seen in DLE
DIF findings of LPP/FFA
(+) globular cytoid depositions of IgM
First-line treatment for Classic LPP/FFA
TAC ILSI 10mg/mL q4-6wks +/- Class I or II topical corticosteroids
Near the face: TAC 2.5mg/mL injected 1cm behind the hairline
Others: minoxidil, platelet-rich plasma, oral cyclosporine, retunoids, antimalarials, griseofulvin, oral corticosteroids
Pseudopelade of Brocq (PPB) most commonly seen in
Women, 30-50yo
Sites affected first in PPB
Vertex and occiput of scalp
Clinical presentation of PPB
(+) small flesh-toned alopecic patches with irregular margins (“footprints in the snow”
OR (+) non-inflammatory centrifugally spreading patch of alopecia
(-) follicular hyperkeratosis
(-) perifollicular/diffuse erythema
Clinical presentation of DLE
(+) >=1 erythemtatous, atrophic, alopecic patches on scalp
(+) follicular hyperkeratosis, hyperpigmentation, hypopigmentation, telangiectasia
(+/-) sensitivity, pruritus, worsening with UV exposure
Histopathologic findings of PPB
(+) sparse to moderate lymphocytic infiltrate around follicular infundibulum
(+) complete destruction of sebaceous glands
(+) replacement of hair follicles with fibrous tracts (late stage)
(-) interface dermatitis; elastic fibers preserved and thickened
Treatment of PPB
1st Line: TAC ILSI 10mg/mL +/- corticosteroids
Hydroxychloroquine, oral prednisone, isotretinoin
Central Centrifugal Cicatricial Alopecia (CCCA) most commonly seen in
Women of African descent
Etiology of CCCA
Autosomal dominant inheritance
Chemical hair grooming
Traction-inducing hair styles
Clinical presentation of CCCA
(+) skin-colored patch of scarring alopecia on the crown, progressing centrifugally
(+) perifollicular hyperpigmentation, polytrichia
(+/-) pain, itching, tenderness, “pins-and-needles” sensation
Histopathologic Findings of CCCA
(+) dermal hyalinization, hair fiber granulomas, loss of follicular epithelium, follicular lymphocytic inflammation of the lower infundibulum to isthmus, premature desquamation of inner root sheath and fibrous connective tissue
Treatment of CCCA
Early diagnosis is crucial
More natural, less traumatizing hair practices
ILSI/topical steroids, tetracycline, HCQ, immunosuppressants (MMF, cyclosporine), antiandrogens
Clinical presentation of Alopecia Mucinosa
(+) well-demarcated, indurated, erythematous or skin-colored patches of scarring/nonscarring alopecia
(+) diffuse hair loss
(+) alopecia of the eyebrows
(+/-) grouped follicular papules, follicular cysts, follicular hyperkeratosis
Histopathologic findings of Alopecia Mucinosa
(+) mucin deposition in the outer root sheath
(+) replacement of entire pilosebaceous unit by pools of mucin
*strictly speaking, not a primary cicatricial alopecia because hair follicle is not replaced by a true scar
Conditions associated with Alopecia Mucinosa
Cutaneous T-cell lymphoma
Mycosis fungoides, Sezary syndrome
Treatment for Alopecia Mucinosa
Oral corticosteroids, minocycline, isotretinoin, topical/intralesional CS, dapsone, indomethacin, light therapy
Keratosis follicularis spinulosa decalvans (other names)
Keratosis atrophicans faciei
Ulerythema ophyrogenes
Keratosis pilaris rubra atrophicans faciei
Etiology of KFSD
X-linked inheritance
KFSD usually seen in
Adolescence
Clinical presentation of KFSD
(+) scarring alopecic patches, follicular hyperkeratosis, +/- pustules
(+/-) involvement of eyebrows/lashes
Histopathologic findings of KFSD
(+) mixed infiltrate of lymphocytes and neutrophils in the infundibular area (early)
(+) predominantly lymphocytic, follicle replaced by fibrous tissue (late)
Treatment of KFSD
May improve with age
Topica/intralesional CS, oral retinoids
Folliculitis Decalvans most commonly seen in ____, starts at ____
Young and middle-aged adults, male
Vertex of scalp
Etiology of Folliculitis Decalvans
S. aureas infection in combination with hypersensitivity reaction to “superantigens’ and defects in host cell-mediated immunity
Clinical presentation of Folliculitis Decalvans
(+) erythematous, alopecic patches, follicular pustules, follicular hyperkeratosis
(+) tufted folliculitis - 5 to 15 hairs emerging from single dilated orifice
(+) pain, itching, burning sensation
Histopathologic findings of Folliculitis Decalvans
(+) keratin aggregation in infundibulum with numerous neutrophils
(+) intra/perifollicular neutrophilic infiltrate
(+) sebaceous glands destroyed early
(+) neuts, lymphocytes, plasma cells extend into dermis; hair-shaft granulomas with FB giant cells (advanced)
(+) follicular and interstitial dermal fibrosis, hypertrophic scarring (end-stage)
Treatment of Folliculitis Decalvans
Low-dose antibiotics for many years
Eradication of S. aureus: minocycline, erythromycin, cephalosporins, TMP-SMX, rifampicin + clindamycin, oral fucidic acid, mupirocin,
TAC ILSI 10mg/mL to reduce inflamm
Dissecting Folliculitis (other names)
Dissecting cellulites
Perifolliculitis capitis abscedens et suffodiens of Hoffman
Follicular Occlusion Triad
Dissecting folliculitis
Acne Conglobata
Hidradenitis suppurativa
Dissecting Folliculitis most commonly seen in
Young men, 18-40yo, African-American
Pathophysiology of Dissecting Folliculitis
Follicular occlusion, seborrhea, androgens, secondary bacterial population, abnormal host response
Clinical presentation of Dissecting Folliculitis
(+) fluctuating nodules, abscesses, sinuses with spontaneous discharge of pus
(+) erythematous, follicular papules and pustules
(+) multifocal lesions - form intercommunicating ridge and seropurulent exudates
(+) tenderness, pruritus
(+) keloids/HS (chronic/relapsing)
Dissecting Folliculitis usually begins on
Vertex and occipital scalp
Histopathologic findings of Dissecting Folliculitis
(+) intra/perifollicular neutrophilic infiltrate with follicular occlusion (early)
(+) interconnecting sinus tracts lined by squamous epithelium, follicular perforation, perifollicular and deep dermal abscesses
Treatment of Dissecting Folliculitis
Multimodal tx: oral abx (anti-inflamm; minocycline, tetracycline, erythromycin, cephalosporin, clindamycin +/- rifampin) + intralesional CS + oral prednisolone
Isotretinoin 0.5-1mg/kg/day - prolongs remission
Surgical methods (extreme cases): I&D, marsupialization with curettage of cyst wall, complete scalp extirpation with skin grafting
Acne Keloidalis Nuchae most commonly seen in
African-American men, 14-25yo
Etiology of Acne Keloidalis Nuchae
Trauma (shirt collars)
Infection (Demodex, bacteria)
Clinical presentation of Acne Keloidalis Nuchae
(+) skin-colored follicular papule,s pustules, plaques
(+) keloid-like scarred lesions in occipital scalp
Histopathologic findings of Acne Keloidalis Nuchae
(+) acute inflamm with neutrophilic/lymphocytic infiltration
(+) chronic granulomatous inflamm around isthmus and lower infundibulum
Treatment of Acne Keloidalis Nuchae
1st Line: TAC ILSI (10-40mg/mL) +/- topical clindamycin 2% or oral tetracyclines
Others: topical retinoids, cryotherapy, laser, surgical excision
Clinical presentation of Acne Necrotica (Varioliformis)
Frontal and parietal scalp, seborrheic areas of face; adults
(+) umbilicated, pruritic/painful papules with central necrosis
(+) varioliform/smallpox-like scars
Histopathologic findings of Acne Necrotica (Varioliformis)
(+) suppurative, necrotic, infundibular folliculitis with lymphocytic/mixed inflammatory infiltrate
Clinical presentation of Erosive Pustular Dermatosis
Elderly women
(+) suppurative, necrotic, erosive papule or plaque
Histopathologic findings of Acne Necrotica (Varioliformis)
(+) extensive, chronic, mixed inflamm infiltrate in the dermis, later with dermal fibrosis
Treatment of Acne Necrotica (Varioliformis)
Class I or II topical CS +/- topical/systemic antibiotics + oral isotretinoin
Possible causes of Secondary Cicatricial Alopecia
Congenital defects, trauma, inflammatory conditions, infections, neoplasms, drugs, chronic traction and surgical scars
Etiologic agents of Tinea Capitis
Endothrix: Trichophyton tonsurans subspecies sulfureum (90% overall)
- (+) black dots
- hair loss, inflamm, scaling is minimal
Ectothrix: Microsporum canis, Epidermophyton spp.
- (+) alopecic patches with signs of inflamm and scaling with brittle grayish hair stumps
- Wood’s: (+) yellow-green fluoresence
Patelliform scales (scutula) - sulfuric-yellow concretions of hyphae and skin debris in the follicular orifices; malodorous
Favus
Deep, highly inflammatory fungal infection; (+) highly suppurative, boggy, nodular, deep folliculitis with fistulas and pus secretion
Kerion
Treatment for tinea capitis
Systemic: terbinafine, itraconazole, ketoconazole, griseofulvin, fluconazole
Topical sporicidal agents (to limit spread of spores): selenium, ketoconazole
Etiologies of traumatic hair loss (4)
Traumatic injuries
Traction alopecias (braided/weaved African-American hair, Sikh topknot, tight ponytails, rollers)
Trichotillomania
Pressure alopecia (d/t ischemia)
