Continuing Education Activity

Tufted hair folliculitis is an uncommon, progressive condition associated with cicatricial (scarring) alopecia that affects the scalp. Tufted hair folliculitis is considered a clinicopathologic feature rather than a distinct entity, since it may be observed in association with several scarring conditions. This activity describes the pathophysiology, evaluation, and management of tufted hair folliculitis and highlights the role of the interprofessional team in the management of these patients.

Objectives:

• Identify the etiology of tufted hair folliculitis.
• Describe the presentation of tufted hair folliculitis.
• Outline the management options available for tufted hair folliculitis.
• Explain interprofessional team strategies for improving care coordination and communication to advance the treatment of tufted hair folliculitis and improve outcomes.

Introduction

Tufted hair folliculitis is an uncommon, progressive clinical manifestation associated with scarring (cicatricial) alopecia which affects the scalp. This condition derives its name from the presence of groups of five to 30 hairs emerging from a unique, dilated follicular opening. Tufted hair folliculitis is considered a clinicopathologic feature than a distinct entity rather, since it may be observed in association with several scarring conditions.[1][2]

Etiology

The etiology of tufted hair folliculitis is most often unknown. This clinical aspect can be observed in association with several types of scarring alopecia. It is likely that this finding represents an ultimate stage of follicular injury which can be caused by several different mechanisms. This pattern of hair loss has been reported with scars from trauma, burn, or surgery, acne keloidalis nuchae, folliculitis decalvans, dissecting cellulitis of the scalp, tinea capitis, lichen planus, and pemphigus vulgaris. Tufted hair folliculitis has also been reported with use of some drugs, such as cyclosporin, trastuzumab, and lapatinib. Some authors think that tufted hair folliculitis is a result of preexistent folliculitis, while others believe it is a particular type of folliculitis decalvans since the two conditions share similar histopathological findings.[3][4]

Epidemiology

Tufted hair folliculitis is a rare condition. It has been reported most often in adults, primarily in individuals in the fourth and fifth decade of life. However rare cases of tufted hair folliculitis have been reported in younger patients, among them, two were seen in Gobello Nevus syndrome. Extreme ages reported ranges from ten to 68 years. There is no racial predilection for tufted hair folliculitis, but a male predominance is observed with a male to female ratio of 2.7:1.

Pathophysiology

Tufted hair folliculitis is the manifestation of a fibrosis-induced gathering of adjacent follicular structures, as well as a follicular retention of telogen phase hairs over multiple cycles. Some authors hypothesized that tufted hair folliculitis might result from nevoid scalp structures that undergo inflammation and scarring. Staphylococcal colonies are frequently cultured from tufted hair folliculitis lesions, but the role of this organism in the pathogenesis is unclear. Although some authors think that tufted hair folliculitis may result from a local immune response disorder, no anomalies in the host immune response to pathogens have been demonstrated, since CD4 and CD8 counts, as well as the ratio T-cell/B-cell, were found to be normal.[5][6]

Histopathology

Biopsy specimens from tufted hair folliculitis lesions demonstrate scarring fibrosis in the upper dermis, resulting in a convergence of the follicular infundibula (the upper portion of the hair follicle) into a single channel. The lower portions of the hair follicle, including the bulb, are not affected by the scarring process and remain separate. As seen in folliculitis decalvans, a perifollicular neutrophilic inflammatory infiltrate is often observed in tufted hair folliculitis. In some other cases, the inflammatory infiltrate is mixed, containing, in addition to neutrophils, lymphocytes, and plasmocytes.

History and Physical

Patients are having tufted hair folliculitis note pain, itching, and/or swelling of the scalp with slowly progressive hair loss. Perifollicular crusting and scaling are also frequently reported. The process usually starts in a specific portion of the scalp which enlarges gradually.  

The predominant clinical finding in tufted hair folliculitis is clusters of anagen hairs emerging from the scalp, and having a pattern of "doll's hair." Circumscribed areas of scarring alopecia containing no hair follicles alternate and are mixed with the tufts of hair. Parietal and occipital areas are involved with predilection in tufted hair folliculitis, but the entire scalp may be affected. The beard is rarely involved. Though pustules are not present, it may be possible to express purulent exudate from the dilated follicular openings. Depending on the duration of the condition and the underlying cause of the tufting, there may be erythema, edema, tenderness, and/or bogginess of the scalp. A case of tufted hair folliculitis having a linear pattern has been reported.

Evaluation

Hair and scalp dermoscopic examination (trichoscopy) of areas containing tuft hair folliculitis exhibits tufts of hair emerging from a unique follicle. The hair follicles are surrounded by scaling, and by a white coloration in a "starburst pattern" predictive of fibrosis process. Blood vessels may be observed and are located between the hair follicles.

A scalp biopsy may be helpful to determine the cause of the tufted hair folliculitis. If the patient has an active primary scarring hair alopecia, such as lichen planus, the typical pathologic changes associated with that condition will be present. Scalp biopsy can also indicate the extent of scarring and the degree of active inflammation, which can have a prognostic information about the condition. Bacterial cultures often yield Staphylococcus, but this organism is not seen in all cases. Nor does treating the Staphylococcus lead to resolution of the condition. A fungal culture is commonly negative.

Treatment / Management

Treatment of tufted hair folliculitis is not codified, and there is no satisfactory therapy for this condition. If a cause can be identified for tufted hair folliculitis, treatment is aimed at the underlying disease. Otherwise, therapy should be directed toward decreasing discomfort and improve appearance. Topical treatment, such as shampoos containing salicylic acid or tar derivatives, can decrease scaling but are not sufficient. Topical steroids can decrease erythema, edema, and itching but their effect is minimal and not persistent. Systemic antistaphylococcal agents may be used in tufted hair folliculitis if evident clinical signs of infection, such as purulent exudate, are present. Prolonged treatment with oral rifampicin (300 mg daily) associated with oral clindamycin (300 mg daily) was found to give good results in tufted hair folliculitis. Topical nadifloxacin has been used in one patient with good clinical results but need further evaluation on large series of patients. Zinc sulfate, methotrexate, and isotretinoin are not efficient. Surgical excision of the plaques of scarring alopecia has been reported to give good cosmetic results in patients with tufted hair folliculitis. Caution is recommended, however, as post-surgical scarring can be a cause of this condition.[7][8][9][10]

Differential Diagnosis

• Acne keloidalis Nuchae (KAN)

• Dissecting cellulitis of the scalp

• Folliculitis decalvans

• Kerion

• Lichen planus

Pearls and Other Issues

Tufted hair folliculitis usually has a chronic progressive course with alternating periods of exacerbation and recovery. The condition is usually not life-threatening. However, it may have a negative psychological effect due to the cosmetic disfigurement that it may cause.

Enhancing Healthcare Team Outcomes

The management of tufted hair folliculitis is best done with an interprofessional team that also includes nurses and pharmacists.

Treatment of tufted hair folliculitis is not codified, and there is no satisfactory therapy for this condition. If a cause can be identified for tufted hair folliculitis, treatment is aimed at the underlying disease. Otherwise, therapy should be directed toward decreasing discomfort and improve appearance. To improve outcomes, clinicians should not aggressively pursue surgical options as they often lead to more harm than good.

The outcomes for most patients are guarded. The condition is known to cause significant emotional distress and hence a mental health nurse should be consulted.