Tufted Hair Folliculitis Clinical Presentation

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.

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 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, and pemphigus vulgaris.^{[9, 10]}