Tufted Hair Folliculitis 

Updated: May 03, 2016
Author: Elizabeth CW Hughes, MD; Chief Editor: William D James, MD 

Overview

Background

Tufted hair folliculitis is a rare, progressive pattern of scarring alopecia that affects the scalp. Its characteristic feature is the presence of groups of 10-15 hairs emerging from a single follicular opening. The cause of this disorder is unknown. Tufts of hair associated with scars have been described in association with several other forms of alopecia. It is probable that tufted hair folliculitis represents an advanced stage of follicular injury seen in several types of scarring alopecia.

Pathophysiology

Tufted hair folliculitis affects hair follicles of the scalp. Biopsy specimens demonstrate convergence of follicular infundibula, with multiple hairs emerging from a single follicular opening, while the lower portions of the hair follicle are separate and unaffected by the scarring process. Staphylococcal organisms frequently are cultured from lesions of tufted hair folliculitis, but their role in pathogenesis is unclear.[1]

Epidemiology

Frequency

Tufted hair folliculitis is rare.

Race

No racial predilection is recognized for tufted hair folliculitis.

Sex

No sex predilection is recognized for tufted hair folliculitis.

Age

Tufted hair folliculitis has been reported only in adults. It has been reported primarily in individuals in the fourth and fifth decades of life.

Prognosis

Tufted hair folliculitis is a chronic condition. The patient may experience intermittent flares and periods of quiescence. Fortunately, morbidity is low and limited to local discomfort and cosmetic concerns. Mortality from tufted hair folliculitis has not been reported.

 

Presentation

History

Patients with tufted hair folliculitis report hair loss that develops slowly, often over years. The hair loss frequently is accompanied by pain or swelling of the affected scalp. Patients frequently complain of crust and scales adherent to the scalp and hair. The ability to express pus from the follicular orifice is a frequent, but not constant, finding. This process usually is limited to a single area of the scalp that enlarges gradually. If the patient has another form of scarring alopecia accompanied by tufted hair, such as acne keloidalis, the history will reflect the predominant cause of hair loss.

Physical

The most prominent feature of this disorder is the presence of tufts of 8-15 hairs that appear to emerge from a single follicular orifice in a "doll's hair" pattern. Adjacent to and intermingled with the tufts are areas of scarring alopecia, with complete loss of follicles. The area of tufts and scarring is somewhat well circumscribed and may be accompanied by varying degrees of edema, erythema, and tenderness. Boggy plaques have been described. There may be crust adherent to the scalp or hair, often in a collarette around the most proximal portion of the hair. Pustules are not common, but pus may be expressed from the follicular openings. Several reports have noted that a high percentage of telogen hairs are obtained when tufts of hair are forcibly extracted.[2]

Tufted hairs. Multiple hairs emerging a dilated fo Tufted hairs. Multiple hairs emerging a dilated follicular orifice with surrounding scarring alopecia.

Causes

Tufted hair folliculitis probably represents an advanced stage of follicular damage common to several different forms of scarring alopecia. As such, the condition is a clinicopathologic pattern and not a distinct disease. In most cases, the cause of idiopathic tufted hair folliculitis is unknown, but several theories about the exact mechanism of hair tuft formation exist.

In the original report of this entity, Smith and Sanderson suggested that new follicular epithelium forms around groups of hair shafts that remain after destruction of the upper portion of the follicle.[3] Many authors believe in a variation of this theory. The variation suggests that tufts form when inflammation and scarring in the papillary and upper reticular dermis contracts the interfollicular dermal tissue, causing separate follicles to converge.

Perifollicular inflammation is presumed to lead to retention of telogen hairs, compounding the appearance of tufting. On the other hand, Tong and Baden proposed that tufts of hair represent a nevoid malformation.[4]

The precise role of Staphylococcus aureus in this condition is also unclear. S aureus frequently, but not invariably, is cultured from lesions of tufted hair folliculitis. It is likely that the organism is a secondary invader, but still may contribute to the progression of disease. However, some authors postulate that infection is the primary process; toxins elaborated by S aureus trigger an inflammatory process in the superior dermis, leading to scarring.

Case reports describe tufted folliculitis in association with medication use, specifically with cyclosporine[5] and lapatinib.[6] Such associations are rare, however, and the pathophysiology in these cases has not been fully explained.

Tufts of hair amid areas of scarring, giving the classic appearance of tufted hair folliculitis, have been described in patients with a number of different disorders, including scars from surgery or trauma, acne keloidalis, folliculitis decalvans,[7, 8] dissecting cellulitis of the scalp, lichen planus, Melkersson-Rosenthal syndrome and hidradenitis suppurativa,[9] and pemphigus vulgaris.[10, 11]

 

DDx

Differential Diagnoses

 

Workup

Laboratory Studies

A skin biopsy specimen can be obtained from the affected area. See Histologic Findings.

Bacterial culture of purulent exudate, biopsy specimen, or plucked hair, including antibiotic sensitivity, may be helpful in guiding treatment.

Fungal cultures are not positive. If a fungal organism is identified, consideration should be given to an alternate diagnosis.

Extensive laboratory tests generally are not revealing and are of little value.

Histologic Findings

The epidermis shows hyperkeratosis, with parakeratosis, overlying a hyperplastic epidermis. Follicular plugging may be observed.

In the papillary and upper reticular dermis a perifollicular, mixed inflammatory cell infiltrate is present, including lymphocytes, plasma cells, and neutrophils. In areas of follicular rupture, giant cells that may contain fragments of hair shafts are present.

In areas of tufting, the outer root sheath in the infundibular section of the follicle is thinned. The normal spacing of the hairs is disrupted, resulting in convergence the upper portion of the follicles. Multiple hairs are seen emerging from a single follicular opening. Large numbers of telogen hairs may be identified in the tufts.

The deep reticular dermis and subcutis are normal, showing undisturbed anagen hair bulbs.

In cases of hair tufting associated with other causes of scarring hair loss, the histologic features reflect the primary cause of the alopecia.

Note, however, that one cannot rely solely on histology to make the diagnosis. Mirmirani et al demonstrated that cicatricial alopecias cannot be distinguished reliably from one another on the basis of histology findings.[12] Histologic findings must be interpreted in light of the clinical findings and bacterial culture results.

 

Treatment

Medical Care

No uniformly successful treatment for tufted hair folliculitis exists. Overall, direct therapy toward decreasing patient discomfort and improving appearance. Institute topical treatment directed at decreasing scaling. Shampoos containing tar derivatives often are used. Oral antistaphylococcal antibiotics may be helpful, particularly if a large amount of purulent exudate is present.

Surgical Care

Treatment of tufted hair folliculitis by excision of the areas of scarring has been described.

Long-Term Monitoring

Because tufted hair folliculitis is a chronic condition that lacks a universally successful treatment, patients generally require periodic long-term follow-up. The intensity of treatment and use of oral antibiotics depend on the patient's symptoms.