Tufted Hair Folliculitis

Updated: Aug 07, 2019
  • Author: Elizabeth CW Hughes, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Tufted hair folliculitis is a rare, progressive pattern of scarring alopecia that affects the scalp. [1] 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.

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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. [2]

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Etiology

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]

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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.

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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.

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