88: Cicatricial Alopecia Flashcards
What are the two main types of cicatricial alopecia and their causes?
- Primary cicatricial alopecia: Caused by idiopathic inflammatory diseases that destroy hair follicles.
- Secondary cicatricial alopecia: Caused by cutaneous inflammatory processes of the scalp skin or physical trauma that injures the skin and its appendages.
What is the role of pluripotent hair follicle stem cells in cicatricial alopecia?
Pluripotent hair follicle stem cells are responsible for:
- Renewal of the upper part of the hair follicle and sebaceous glands.
- Restoration of the lower cyclical component of the follicles at the onset of a new anagen period.
What are the clinical features of primary cicatricial alopecia?
- Central and parietal scalp involvement before progressing to other sites.
- Isolated alopecic patches with atrophy and lack of follicular ostia.
- Inflammatory changes such as diffuse or perifollicular erythema, follicular hyperkeratosis, pigment changes, tufting, and pustules.
What diagnostic methods are used to confirm cicatricial alopecia?
- 10-fold magnifying dermatoscope: To assess the presence or absence of follicular ostia, perifollicular erythema, and follicular hyperkeratosis.
- Scalp biopsy: Confirms scarring alopecia, with specific techniques for processing tissue samples.
What is the primary goal of treatment for cicatricial alopecia?
The primary goal of treatment is to stop the inflammation and further progression of the disease. If hair loss is extensive, camouflage techniques or hair restoration surgery may be considered.
Describe the primary and secondary causes of cicatricial alopecia.
Primary cicatricial alopecia is caused by idiopathic inflammatory diseases that destroy the hair follicle through a folliculocentric inflammatory process. Secondary cicatricial alopecia is caused by cutaneous inflammatory processes or physical trauma that injures the skin and its appendages.
What is the most common cause of inflammatory cicatricial alopecia?
Chronic cutaneous lupus erythematosus (discoid lupus erythematosus) is the most common cause of inflammatory cicatricial alopecia, often associated with lichen planopilaris (LPP).
What are the clinical presentations of Frontal Fibrosing Alopecia (FFA)?
FFA presents with frontal, band-like or circumferential scarring alopecia, often with some hairs spared in the original frontal hairline. It may also show follicular hyperkeratosis and perifollicular erythema in a band-like pattern, and alopecia of the eyebrows is frequently seen.
What is the management and treatment for classic Lichen Planopilaris (LPP)?
Management for classic LPP includes:
- Intra-lesional triamcinolone acetonide at 10 mg/mL every 4 to 6 weeks.
- Topical class I or class II corticosteroids.
- For FFA, ILSI 2.5 mg/mL injected 1 cm behind the hairline.
- Topical minoxidil or tacrolimus for FFA.
- Oral corticosteroids as bridge therapy in the first weeks of treatment.
What are the histopathological features of Lichen Planopilaris (LPP) and Frontal Fibrosing Alopecia (FFA)?
LPP and FFA share similar histopathological features, including:
- Lymphocytic infiltrate and interface dermatitis predominantly found in and around the upper permanent part of the hair follicle.
- Vascular plexus is not affected by inflammation, and mucin deposits are absent.
- Direct immunofluorescence (DIF) shows globular cytoid depositions of IgM, and rarely IgA, IgG, or C3, in the dermis around the infundibulum.
What is the typical age range for women affected by Classic Pseudopelade of Brocq?
Classic Pseudopelade of Brocq typically affects women between the ages of 30 and 50 years.
What are the clinical features of discoid lupus erythematosus (DLE)?
DLE presents as one or more erythematous, atrophic, and alopecic patches on the scalp, with features like follicular hyperkeratosis, hyperpigmentation, hypopigmentation, and telangiectasia. Active lesions may be sensitive or pruritic and worsen after UV light exposure.
What is the first-line treatment for moderately active classic lichen planopilaris (LPP)?
The first-line treatment for moderately active classic LPP lesions is intralesional triamcinolone acetonide at a concentration of 10 mg/mL every 4 to 6 weeks, often combined with topical class I or class II corticosteroids.
What are the histopathological features of frontal fibrosing alopecia (FFA)?
FFA shows lymphocytic infiltrate and interface dermatitis predominantly in the upper permanent part of the hair follicle. The vascular plexus is not affected by inflammation, and mucin deposits are absent.
What are the clinical features of Graham-Little syndrome?
Graham-Little syndrome is characterized by lesions of classic LPP on the scalp, nonscarring alopecia of the axillae, pubic area, and eyebrows, as well as keratosis pilaris of the trunk and extremities.
What are the clinical features of lichen planopilaris (LPP)?
LPP starts at the crown and vertex, presenting with perifollicular erythema and follicular hyperkeratosis. Alopecic areas are often smaller, irregularly shaped, and interconnected, leading to a reticulated clinical pattern.
What is the most common form of primary cicatricial alopecia in women of African descent?
Central Centrifugal Cicatricial Alopecia (CCCA) is the most common form, characterized by a skin-colored patch of scarring alopecia on the crown that progresses centrifugally to the parietal areas.
What are the major pathogenic factors associated with Central Centrifugal Cicatricial Alopecia?
The major pathogenic factors include:
- Autosomal mode of inheritance
- Chemical hair grooming practices
- Traction-inducing hairstyles
What are the management options for Central Centrifugal Cicatricial Alopecia?
Management options include:
- Early screening
- Natural, less traumatizing hair care practices
- Topical and intralesional corticosteroids
- Tetracycline
- Hydroxychloroquine
- Immunosuppressive medications (e.g., mycophenolate mofetil, cyclosporine)
- Antiandrogens
- Wigs and hairpieces
What distinguishes Alopecia Mucinosa from primary cicatricial alopecia?
Alopecia Mucinosa is not a primary cicatricial alopecia because the hair follicle is not replaced by a true scar. It is characterized by mucin deposition in the outer root sheath and can occur idiopathically or in the setting of cutaneous T-cell lymphoma or mycosis fungoides.
What is the treatment approach for Alopecia Mucinosa?
The treatment approach includes:
- Complete workup to rule out underlying malignancy (e.g., mycosis fungoides)
- Oral corticosteroids
- Minocycline
- Isotretinoin
- Topical and intralesional corticosteroids
- Dapsone
- Indomethacin
- Light therapy
What is Folliculitis Decalvans and its associated pathogen?
Folliculitis Decalvans is characterized by erythematous alopecic patches and is predominantly associated with Staphylococcus aureus. It often presents with follicular pustules and hyperkeratosis.
What are the management strategies for Folliculitis Decalvans?
Management strategies include:
- Bacterial cultures and antibiotic sensitivity testing
- Antibiotics (e.g., minocycline, erythromycin, cephalosporins)
- Rifampin in combination with clindamycin
- Oral fucidic acid
- Topical antibiotics (e.g., mupirocin)
- Intralesional triamcinolone acetonide
What is Dissecting Folliculitis and its demographic prevalence?
Dissecting Folliculitis, also known as perifolliculitis capitis abscedens et suffodiens, is more commonly seen in young men between 18 and 40 years of age, particularly affecting African American men more than white men.
What are the clinical features of central centrifugal cicatricial alopecia (CCCA)?
CCCA presents as a skin-colored patch of scarring alopecia on the crown, gradually progressing centrifugally to the parietal areas. Symptoms include perifollicular hyperpigmentation, polytrichia, pain, itching, tenderness, and ‘pins-and-needle’ sensations.
What are the clinical features of classic pseudopelade of Brocq (PPB)?
PPB presents as small flesh-toned alopecic patches with irregular margins, often described as ‘footprints in the snow.’
What are the clinical features of alopecia mucinosa in late lesions?
Late lesions of alopecia mucinosa show replacement of the entire pilosebaceous unit by pools of mucin.
What are the clinical features of alopecia mucinosa in early lesions?
Early lesions of alopecia mucinosa show mucin deposition in the outer root sheath.
What are the common clinical features of Acne Keloidalis Nuchae?
- Skin-colored follicular papules, pustules, and plaques
- Keloid-like scarred lesions in the occipital scalp
- Acute inflammation with neutrophilic or lymphocytic infiltration
- Chronic granulomatous inflammation around the isthmus and lower infundibulum
What is the treatment of choice for Acne Keloidalis Nuchae?
- Monthly intralesional triamcinolone acetonide (10 to 40 mg/mL) alone or combined with topical 2% clindamycin or oral tetracyclines
- Class I or class II topical steroids alone or in combination with topical antibiotics for mild cases
- Surgical excision for extensive keloidal lesions
What are the characteristics of Erosive Pustular Dermatosis?
- Occurs in elderly women
- Presents as suppurative, necrotic, erosive papule or plaque
- Histology of early lesions is non-specific
- Older lesions show extensive, chronic, mixed inflammatory infiltrate in the dermis and later dermal fibrosis
What are the potential causes of Secondary Cicatricial Alopecia?
- Permanent hair loss caused by other scalp conditions not related to the hair follicle
- Possible causes include congenital defects, trauma, inflammatory conditions, infections, neoplasms, and rarely, drugs
What is Tinea Capitis and its common causative agent?
- Tinea Capitis is an infection with dermatophytes species
- Trichophyton tonsurans accounts for approximately 90% of cases in the United States and United Kingdom
What are the signs of Endothrix infections in Tinea Capitis?
- Spores within the hair shaft
- Hair breaks off directly at the skin surface, presenting as ‘black dots’
- Hair loss, inflammation, and scaling may be minimal, often dismissed as seborrheic or atopic dermatitis
What are the clinical implications of Favus in Tinea Capitis?
- Favus presents with patelliform scales (scutula) and sulfuric-yellow concretions of hyphae and skin debris in the follicular orifices
- Exhibits a distinct malodorous smell
- Can lead to scarring hair loss
What is the pathogenesis of dissecting folliculitis?
The pathogenesis involves follicular occlusion, seborrhea, androgens, secondary bacterial overpopulation, and an abnormal host response to bacterial antigens.
What are the clinical features of dissecting folliculitis in initial lesions?
Initial lesions of dissecting folliculitis present as erythematous, follicular papules and pustules, mostly on the vertex and occipital scalp.
What is the treatment of choice for acne keloidalis nuchae?
The treatment of choice is monthly intralesional triamcinolone acetonide (10 to 40 mg/mL) alone or combined with topical 2% clindamycin or oral tetracyclines.
What is the treatment of choice for acne keloidalis nuchae?
The treatment of choice is monthly intralesional triamcinolone acetonide (10 to 40 mg/mL), alone or combined with topical 2% clindamycin or oral antibiotics like tetracyclines.
What are the key features of dissecting folliculitis?
Dissecting folliculitis is characterized by fluctuating nodules, abscesses, and sinuses that frequently discharge pus. It often starts on the vertex and occipital scalp and can lead to chronic, relapsing hypertrophic or keloidal scars.
What are the management options for erosive pustular dermatosis?
Management includes class I or class II topical steroids with or without topical antibiotics, systemic antibiotics, and oral isotretinoin.
What are the clinical features of acne necrotica?
Acne necrotica presents as umbilicated, pruritic, or painful papules that undergo central necrosis, leading to varioliform or smallpox-like scars.
What are the clinical features of acne keloidalis nuchae?
Acne keloidalis nuchae presents as skin-colored follicular papules, pustules, and plaques, with keloid-like scarred lesions in the occipital scalp.
What are the clinical features of dissecting folliculitis in advanced stages?
Advanced stages of dissecting folliculitis show interconnecting sinus tracts lined by squamous epithelium, follicular perforation, and perifollicular and deep dermal abscesses.
What are the clinical features of erosive pustular dermatosis?
Erosive pustular dermatosis presents as suppurative, necrotic, erosive papules or plaques, often seen in elderly women.
What are the clinical features of acne necrotica scars?
Acne necrotica scars are varioliform or smallpox-like scars resulting from necrotic papules.
What are the clinical features of secondary cicatricial alopecia?
Secondary cicatricial alopecia is caused by permanent hair loss due to scalp conditions unrelated to the hair follicle, such as congenital defects, trauma, inflammatory conditions, infections, neoplasms, and drugs.
What are the systemic antifungal treatments for scalp conditions and how does the choice depend on the type of fungus?
Systemic antifungal treatments include:
- Terbinafine
- Itraconazole
- Ketoconazole
- Griseofulvin
- Fluconazole
The choice of systemic antifungal agent depends on the type of fungus involved in the scalp condition.
What types of traumatic hair loss can occur due to mechanical insult to the scalp?
Traumatic hair loss can be classified into three types:
- Acute trauma
- Prolonged traction
- Pressure
What are the characteristics and potential outcomes of traumatic injuries to the scalp?
Characteristics of traumatic injuries include:
- Fine streaks of hair loss in the injured area.
- Irregular and large patches of hair loss if the wound borders undergo contusion or destruction.
- Can occur after scalp surgery, especially after extensive scalp reduction or large donor strip harvesting.
Potential outcomes:
- Usually reversible but can also be permanent.
- Traumatic birth induced alopecia is infrequent and may result from mechanical extractor marks, tears, or infections.
What is traction alopecia and what are its common causes?
Traction alopecia is caused by prolonged traction on the hair, leading to follicular atrophy. Common causes include:
- Tight ponytails
- Braids
- Heavy dreadlocks
- Extensive use of rollers
It is more commonly seen in African American women from hair braiding and weaving procedures, and in Sikh boys with tight hairstyles.
What is trichotillomania and how does it manifest in individuals?
Trichotillomania, or hair pulling disorder, is characterized by:
- A compulsion to pull out, twist, or break one’s own hair.
- Two forms:
- Infantile: Early-onset trichotillomania starts in early childhood, typically resolves spontaneously or with simple interventions.
- Late-onset: May present with single or multiple asymmetrical areas of hair loss, not smoothly devoid of hairs, displaying short or bristly anagen hair.
Treatment focuses on education of the patient and/or parents, and addressing underlying psychopathology in late-onset cases.
What is pressure alopecia and what causes it?
Pressure alopecia occurs when a patient is unconscious and completely immobile for a certain length of time. It results from ischemia caused by the pressure of the body weight on a specific scalp area, leading to ischemic injury and potentially permanent hair loss.
What are the two forms of trichotillomania?
The two forms are infantile trichotillomania, which starts in early childhood and may resolve spontaneously, and late-onset trichotillomania, which is of short duration and may require simple interventions.
What are the clinical features of tinea capitis caused by ectothrix infection?
Ectothrix infection presents as alopecic patches with inflammation, scaling, and brittle grayish hair stumps. It may show yellow-green fluorescence under Wood light.
What are the management options for traction alopecia?
Management includes discontinuing injurious hairstyles, adopting less traumatic hair care practices, and hair transplantation if there is sufficient donor hair supply.
What are the clinical features of pressure alopecia?
Pressure alopecia occurs after prolonged immobility, resulting in ischemic injury and permanent hair loss in the affected scalp area.
What are the clinical features of kerion in tinea capitis?
Kerion is a deep, highly inflammatory fungal infection characterized by boggy, nodular, deep folliculitis with fistulas and pus secretion.
What are the clinical features of trichotillomania?
Trichotillomania presents as single or multiple asymmetrical, occasionally geometrically shaped areas of hair loss on the scalp or other body areas, with short or bristly anagen hair.
What are the clinical features of favus in tinea capitis?
Favus presents as patelliform scales (scutula), sulfuric-yellow concretions of hyphae and skin debris in the follicular orifices, and emits a distinct malodorous smell.
What are the clinical features of traumatic hair loss?
Traumatic hair loss presents as fine streaks of hair loss in the injured scalp area, which may result in irregular and large patches of hair loss if the wound borders undergo contusion or destruction.
What are the clinical features of tinea capitis caused by endothrix infection?
Endothrix infection presents as hair breaking off directly at the skin surface, appearing as ‘black dots,’ with minimal hair loss, inflammation, and scaling.
