Scarring Alopecia Clinical Presentation

History findings for the various types of scarring alopecia are as follows:

• Lichen planopilaris: Common symptoms include itching, burning, and pain. ^[2] Some authors report a co-occurrence of androgenetic alopecia in up to 30% of patients with lichen planopilaris.

• Central centrifugal cicatricial alopecia: Patients report slow onset of hair loss without symptoms.

• Pseudopelade: Patients report slow onset of hair loss without symptoms.

• Traction alopecia: Patients report slow onset of hair loss without symptoms.

• Lipedematous alopecia: Patients report slow onset of hair loss without symptoms.

• Alopecia mucinosa: Patients report dyshidrosis and dysesthesia.

• Chemotherapy alopecia: Alopecia may occur accompanied by a sensation of tingling. Cytotoxic effects on hair follicles result in temporary hair loss starting within 4 weeks of treatment initiation. This does not resolve until the telogen phase is complete and a new hair cycle begins, typically within 6 months. ^[29] Permanent chemotherapy-induced alopecia is defined as decreased or no regrowth 6 months after the end of therapy. ^[29] This alopecia is often reversible and is due to hair follicle cycle inhibition by the chemotherapy agents. This altered cycle and shedding is often called anagen effluvium. Reports of permanent alopecia typically follow high-dose chemotherapy and often also include subsequent bone marrow transplantation. Busulphan is the most common agent cited to cause permanent chemotherapy-induced alopecia. ^[29]

• Discoid lupus: Patients often have a history similar to persons with cutaneous discoid lupus, which may include a sensation of itchiness and tenderness in the region. Also see Lupus Erythematosus, Discoid.

• Acne keloidalis: Patients report of itching, burning, and tenderness.

• Acne necrotica: Patients report of itching, burning, and tenderness.

• Erosive pustular dermatosis: Patients often have no symptoms. If the scalp region is tender, suspect malignancy.

• Pressure alopecia: Patients usually report itching and dandruff. Note the image below.

  Recalcitrant scarring pressure alopecia several years after an ICU stay. Used with permission, rights retained, courtesy of Rashid M. Rashid, MD, PhD, Morzak Research Collaborative.

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• Senescent alopecia: Patients often have a normal full head of hair through age 40 years. A dramatic, diffuse thinning is then noted. No regional augmented area of alopecia is noted, as in androgenetic alopecia. Mild superimposed androgenetic alopecia may also be present. ^[30]

A hair-pull test can assist in the evaluation of all types of alopecia. An excess of loose anagen hairs can be indicative of active disease. All types of scarring alopecia show loss of follicular ostia. This is most easily seen with a magnification light or dermatoscope.

As detailed below, a full review of systems and a skin examination help in finding subclinical and atypical disease presentations.

Lichen planopilaris

The classic presentation of lichen planopilaris includes perifollicular erythema, perifollicular hyperkeratosis, and scalp erythema. Extracranial disease may be noted in up to 30% of patients. Signs of scalp inflammation can precede hair loss by many months, thus emphasizing the need for a thorough skin examination that includes a scalp examination. ^[30]

Graham-Little syndrome is another variant characterized by patchy cicatricial alopecia of the scalp, nonscarring alopecia of the axillary and pubic areas, and grouped spinous follicular papules that resemble lichen spinulosus or keratosis pilaris on the trunk and extremities.

Central centrifugal cicatricial alopecia

Signs of inflammation are not noted on physical examination. Although central centrifugal cicatricial alopecia classically is described to start on the crown and spread centrifugally, the discovery of cicatricial pattern hair loss as a possible alternate presentation indicates this is not the only course of progression.

Pseudopelade

Signs of inflammation are not noted on physical examination. Unlike central centrifugal cicatricial alopecia, pseudopelade often presents as small patches of scarring in the scalp. Some have suggested use of the term pseudopelade should be avoided.

Traction alopecia and trichotillomania

The scalp may have perifollicular erythema, scales, pustules, or a more subtle seborrheic picture. However, active inflammation is not required for the diagnosis. Broken hairs are common.

The region affected is determined by the etiology. With chignon alopecia, hair loss may be in the occipital area. With corn-rowing, the area most commonly affected is adjacent to the region that is braided. In patients who tie their beards into knots, areas of alopecia can be detected along the sides of the mandible. Note the image below.

In alopecia linearis frontalis, the distribution of hair loss follows a characteristic pattern in the temporal scalp, starting in the periauricular area and extending forward in a triangular manner. The involved area is approximately 1-3 cm in width in most cases.

With the long-term application of tensile forces, an irritant type of folliculitis develops. Follicular scarring and permanent alopecia may result. In some cases, peripilar hair casts form. The casts are fine, yellowish-white keratin cylinders smaller than 1 cm in diameter that ensheathe the hair follicle. Often, peripilar hair casts occur in isolation; however, they have also been known to occur in association with hyperkeratotic scalp disorders. The hair-loss pattern entirely depends on the specific grooming pattern of each patient. Marginal and nonmarginal types may be seen.

Awareness of trichotillomania mimics is also important. This diagnosis can easily mimic other conditions such as patchy alopecia areata, especially if history findings do not suggest otherwise. Often, examination and differentiation can be difficult. ^[7]

Alopecia mucinosa

Hair-bearing areas in the head and neck region are commonly involved. Examination findings include alopecia, prominent follicles, and indurated plaques with fine scale. The physical presentation can be one of many polymorphous types, with no specific pattern recognized. In fact, reports have included mimics of ophiasis, alopecia areata patches, and frontal fibrosing alopecia. Furthermore, because affected hair shafts are fragile and break easily, alopecia mucinosa can present with the now less-specific “black dots” sign of tinea capitis. Thus, no clinical criteria exist for differentiation of patients with benign alopecia mucinosa from those with malignancy-associated alopecia mucinosa.

Keratosis pilaris atrophicans

Keratosis pilaris decalvans involves widespread follicular hyperkeratosis with variable progression to atrophy, cicatricial alopecia of the scalp, and photophobia.

In folliculitis spinulosa decalvans, scalp pustules develop after puberty, often with bacterial infection. This condition may be related to a mutation in MBTPS2. ^[31]

In keratosis pilaris atrophicans faciei, follicular hyperkeratosis develops on the eyebrows but spares the scalp.

With atrophoderma vermiculata, follicular hyperkeratosis involves the cheeks but spares the scalp. Close examination is required in less dramatic cases in order to rule out nonfollicular mimics such as multiple minute digitate hyperkeratosis (also known as spiny hyperkeratosis) and other differential diagnoses related to spiny diseases.

Chemotherapy alopecia

Chemotherapy-induced alopecia can be diffuse or focal. Clinical signs of inflammation are lacking. ^[32]

Discoid Lupus

In adults, discoid lupus erythematosus affects females more often than males. An increased prevalence among African Americans has been reported in the United States. ^[33] The onset of disease is typically between ages 20 and 40 years. Approximately 10% of adults with discoid lupus erythematosus develop systemic lupus erythematosus (SLE); the likelihood is significantly higher in children and adolescents (26%-31%). ^[34] Similarly, 10% of systemic lupus erythematosus patients develop discoid lupus erythematosus. The course of systemic disease is often severe, with renal or neurologic involvement. However, when systemic lupus erythematosus precedes the onset of discoid lupus erythematosus, systemic disease tends to be relatively mild. ^[34]

Involvement of the scalp is common in adults with discoid lupus erythematosus (approximately 50%), and half the patients have the initial onset on the scalp. ^[35]

Patients often report pruritus. The initial erythematous papule or plaque undergoes centrifugal spread and a coin-shaped (discoid) erythematous plaque forms, with follicular plugging and adherent scale. The “carpet tack” sign may be elicited with retraction of the scale. Eventually, erythema diminishes and atrophy, telangiectases, hypopigmentation or depigmentation, and loss of follicular ostia become prominent.

If disease is localized, spontaneous remission occurs within 4 years in approximately 50% of affected persons. Intralesional recurrences are common, along with other complications such as aggressive squamous cell carcinoma with a high metastatic rate.

Folliculitis decalvans

Folliculitis decalvans often starts with a pinpoint erythematous follicular pustule or papule with pain or pruritus. Multiple lesions soon appear, with formation of a large abscess. Clinical findings may include round- to irregularly-shaped, atrophic, flesh-colored or ivory-white areas of scarring alopecia. These areas spread peripherally and can be multifocal. The course is chronic and slowly progressive. Tufted folliculitis may be present.

Perifolliculitis capitis abscedens et suffodiens

Papules progress to painful, bulbous, firm or fluctuant nodules. Pressure on affected sites can result in expression of seropurulent exudate. Long-standing, untreated disease progresses from nonscarring to scarring alopecia. Lymphadenopathy may be present. Perifolliculitis capitis abscedens et suffodiens can resemble and be confused with kerion.

Acne keloidalis

Acne keloidalis is often noted along the posterior hairline and neck. It is mainly found in nonwhite males, although females and whites are also affected. Onset occurs after adolescence. Pinpoint, smooth, follicular papules are evident early in disease and may be crusted, umbilicated, or pustular and contain hair. These papules may coalesce to nodules or keloidal plaques. Abscesses, sinuses, polytrichia, tufted hair folliculitis, foul-smelling discharge, and pain are possible findings.

Acne necrotica

The varioliformis variant is a chronic, relapsing disorder of the adult anterior hairline. Similar findings may be noted in a seborrheic distribution. Examination findings include pruritic, tender, reddish-brown papules or pustules that umbilicate and then undergo central necrosis. These lesions ultimately leave a round hemorrhagic crust that eventually sheds. Varioliform scars result, appearing as focal areas of cicatricial alopecia. A few lesions typically appear with each outbreak. Aggravation in the summer has been reported.

The miliaris variant is not often seen intact because pruritus can lead to near-neurotic excoriation of the lesions.

Erosive pustular dermatosis

Patients develop large, asymptomatic or tender, well-demarcated, boggy, crusted plaques with exudative erosions and pustules. Episodic pustular flares, with slow enlargement over years, are common. Cicatricial alopecia is common, as is bacterial colonization.