Folliculitis Decalvans (Tufted Folliculitis, Central Centrifugal Cicatrizing Alopecia [CCCA])

Are You Confident of the Diagnosis?

What you should be alert for in the history

Folliculitis decalvans is a chronic inflammatory disease of the vertex of the scalp characterized by painful tender follicular papules and pustules (small or even pinhead-sized), often with associated scale and visible crusts. Eventually, the involved site(s) become an atrophic, shiny, occasionally depigmented, patch of total alopecia, absent of follicular orifices, that continues to expand as the peripheral destruction marches on (Figure 1, Figure 2).

When islands of distorted follicles and polytrichia appear, the process may be deemed tufted folliculitis. When areas of involvement merge with occipital lesions, there may be an overlap with folliculitis papillaris (acne keloid). Some authors consider folliculitis decalvans merely a more exuberant reaction, resulting eventually in central centrifugal cicatrizing alopecia (CCCA), as in a spectrum of inflammatory responses of the same disease process; however, folliculitis decalvans has enough unique defining features to separate it as an entity sui generis from CCCA.

Folliculitis decalvans is thus considered a primary scarring alopecia because the hair follicle is the primary target of the inflammatory process, not an innocent bystander caught in the deluge. It is considered by the North American Hair Research Society (NAHRS) a neutrophilic rather than lymphocytic primary alopecia because of the predominance of neutrophils in early lesions, although lymphocytes certainly play a role in older more evolved lesions.

Expected results of diagnostic studies

Routine laboratory tests, including chemotactic studies, have been negative, but a few reports of abnormal chemotaxis and T cell function, hypocomplementemia, or human T-lymphotrophic virus type 1 (HTLV-1) myelopathy in allegedly look-alike syndromes have been cited by Douwes. The histopathology of early lesions should exhibit neutrophils in the perivascular and perifollicular dermis, as well as in the follicular epithelium. Neutrophilic pustules in the follicular infundibula and ostia have been described. Spongiosis may be present in the follicle.

The final result is fibrosis, sometimes extending into the subcutis, with replacement of the follicle and the perifollicular area, and with elastolysis, in a wedge-shaped pattern on vertical sections, visible with von Weigert stain. Fungal stains are negative. Gram stains often show staphylococci. Ruptured and merging hair follicles trapped in fibrosis support the clinical picture of tufted folliculitis.

Diagnosis confirmation

There are several entities to be considered in the differential diagnosis of folliculitis decalvans:

– Erosive pustular dermatosis of the scalp is predominantly a disorder of the elderly, demonstrating rare pustules, many collapsed bullae, and broad shallow ulcers. The tiny pustules and miliary abscesses of folliculitis decalvans look entirely different.

– Bacterial folliculitis from organisms other than Staphylococcus must be considered and cultures obtained.

– Tinea capitis can certainly mimic folliculitis decalvans and cause scarring, even producing a tufted pattern; very inflammatory tinea may not easily present hyphae on scraping or culture. A culture of the brush or comb used in grooming may be of value.

– Keratotic papules, in addition to the pustules and scarring, involvement of areas other than the scalp, and presence of keratosis pilaris, are characteristic of folliculitis spinulosa decalvans, not folliculitis decalvans.

– Large nodules and sinus tracts, even in the presence of some follicular pustules, are more consistent with a diagnosis of perifolliculitis abscedens et suffodiens (dissecting cellulitis). Almost all cases will culture bacterially positive for Staphylococcus aureus.

Whether all cases in African and African-American blacks are part of the various oil-induced folliculitides (e.g. folliculitis papillaris capillitii, some traction alopecia, dermatitis cruris pustulosa et atrophicans) remains to be determined. As the condition is more common in African and African-American blacks, and associated with the use of lubricating oils and possible sequestration of S. aureus, the precise etiology remains to be proven.

Who is at Risk for Developing this Disease?

All racial groups and both sexes are susceptible, but American case reports disproportionately favor patients of African descent. Sillani reviewed the courses of thirteen Chinese patients (some with folliculitis decalvans, others with perifolliculitis abscedens) seen in just one year in one Chinese clinic.

Familial clustering, even in identical twins, has been reported but is distinctly rare, so a genetic component of high penetrance does not seem reasonable.

What is the Cause of the Disease?

Etiology

The cause is unknown.

Pathophysiology

Many authors, particularly Sperling, consider this disease a variant of follicular degeneration of unknown cause, with bacterial colonization occurring secondarily. It seems more likely that the papules and pustules are a direct effect (as are oil-induced folliculitides) of colonization of the scalp follicles by S. aureus, which is almost universally cultured when cultures are positive (the overwhelming majority of the cases).

Interestingly, these organisms are responsive to aminoglycosides, semi-synthetic penicillinase-resistant penicillins, as well as tetracyclines, sulfonamides, trimethoprim, rifampin, etc., suggesting again that, like oil-induced folliculitides, relatively non-virulent but easily recoverable staphylococci contribute to or cause the disease.

S. aureus is thought by some to act as a superantigen, but interleukin-1beta (IL-1β) and tumor necrosis factor-alpha (TNF-α), apparent in large amounts in superantigen-evolved inflammation, were found only weakly expressed in Chiarini’s studies of folliculitis decalvans; the studies did find that basic fibroblast growth factor (b-FGF) and transforming growth factor-beta (TGF-β), which induce activation of fibroblasts, were abundant and could be contributory to the eventual scarring.

Systemic Implications and Complications

No systemic diseases of consequence are related to this condition.

Squamous cell carcinoma of the scalp vertex has been found in one Australian patient with moderate thinning of hair density and scarring but no history of skin ulcers; he had Fitzpatrick photoscale Type II skin and had had unprotected intense sun exposure during his course of alopecia. Lack of a typical Marjolin ulcer makes his sun exposure a more likely causative culprit, despite absence of solar elastosis on his biopsy. Folliculitis decalvans, then, does not predispose to cancer.

Treatment Options

SCALP CARE

Discontinue greasy emollients and “sheen” producers—use siloxane polymers, not petrolatum. Increase frequency of scalp cleansing.

TOPICAL ANTIBIOTICS AND ANTIBACTERIALS

Antibacterial shampoos
Povidone-iodine
Intranasal mupirocin
Topical erythromycin or clindamycin solution or sisomicin
Topical fusidic acid

ORAL ANTIBIOTICS

Penicillinase-resistant penicillins
Macrolides: erythromycin, roxithromycin, clarithromycin
Tetracyclines: minocycline, doxycycline
Clindamycin
Fluoroquinolones: ciprofloxacin, levofloxacin
Dapsone
Rifampin (especially with clindamycin)
Fusidic acid plus zinc

OTHER SYSTEMIC AGENTS

L-tyrosine
Topical desoximetasone (for erlotinib rash)
Intramuscular immunoglobulin(IGIM)
Isotretinoin 0.5mg/kg
Methylprednisolone 40mg/day plus isotretinoin 10mg/day

HAIR REMOVAL

Closely shaved head
Long-pulsed ruby laser
Long-pulsed Nd:YAG laser
Radiation therapy

Optimal Therapeutic Approach for this Disease

One must first determine that the patient does not have scalp folliculitis due to an acneiform or rosaceiform diathesis, and in fact has painful papules and pustules leading to hair loss and apparent loss of follicles.

Bacterial cultures and sensitivities should be taken, and if hair loosening or loss are present, cultures should be made from samples taken from scrapings, pluckings of hair, and grooming instruments. If frank scarring is evident, biopsies should be performed on pustules, not taken from the scarred or tufted areas, and examined using routine H&E and periodic acid-Schiff (PAS) stains. Vertical and horizontal sectioning will provide a better view of the level and type of inflammation.

Therapy touted as optimal in 1999 by Powell, based on eighteen patients and re-emphasized with support of additional success by that author in later publications, involved a combined approach of rifampicin 300mg orally, twice daily, and clindamycin 300mg orally, twice daily, for 10-week courses that occasionally required additional courses. This therapy resulted in very prolonged, possibly complete, remissions. Both agents are individually excellent antistaphylococcals; the combination is intended to reduce the otherwise high risk of development of resistance to rifampin.

The staphylococci isolated from a variety of scalp and oil-induced folliculitides have low virulence and there has been no suggestion of increasing resistance, at least in culture, to antibiotics after their prolonged use. What is peculiar is the easy return of staphylococci to the same environment. Many other antibiotics have been used (since these staphylococci have broad sensitivity), but these have met with varied success, often with only temporary remissions, and not to the levels described by Powell.

It seems reasonable, therefore, to couple the combination therapy with topical preparations such as antibacterial shampoos and povidone-iodine cleansers, and to advise avoidance of greasy scalp treatments as an ongoing maintenance regimen. The cure affected in one patient by merely shaving the hair seems to enhance the concept of ecologic control of the scalp environs.

Application of mupirocin to the nares to reduce staphylococcal carriage is sensible, although the nose may not be the sole orifice of carriage. Treatment with rifampin 300mg orally, twice daily and clindamycin 300mg orally, twice daily with food should be continued for a full 10 weeks, regardless of the rate of clearance, since that is the standard against which to measure. If complete remission occurs, then only topical maintenance should be used, and the patient should be followed monthly for a few months, then discharged. If total remission has not occurred, then more 10-week courses should be prescribed.

Symptomatic relief may be aided by use of topical steroid solutions, applied twice daily. Oral steroids have been used in florid cases, with claimed control, particularly in combination with oral isotretinoin. IGIM, given monthly at 165mg/2cc and 660mg for 40-60kg, and 825mg for 60-80kg, as reported in a limited Korean trial, produced an alleged 100% improvement.

The single case report of shaving resulting in complete remission suggests that the presence of hair, at least above the ostium, contributes to persistence of this disease. This lends support to the varieties of techniques of hair removal, such as lasers and even x-irradiation, long used for a variety of inflammatory types of alopecia.

Shaving is innocuous (at least for a male); laser hair removal, particularly using a long-pulse 1064nm Nd:YAG laser, may be safe and effective, but is clearly very painful (and demanding extensive local anesthesia) when used at 28J/cm2, 3-millisecond duration, 12mm spot size, and dynamic cryogen spray cooling set at a 50-millisecond spray and a 20-millisecond delay.

Surgical scalping and grafting, as utilized for grossly disfiguring perifolliculitis abscedens, does not seem warranted in this disease. The resultant scarring from folliculitis decalvans, though flat, may be disfiguring and proper hair styling should be attempted with a stylist to mask the absence of hair. Men should consider shaving the head for uniformity. Hairpieces will cover the affected area, but raise the question of exacerbation by sweating and sequestration of more Staphylococcus.

Patient Management

Patients must be educated about the chronic nature of the condition and risk of scarring, and should be advised that the best treatment combines appropriate local scalp care with topical and systemic antibiotics to reduce staphylococcal infection and carriage.

In cases associated with prominent inflammation, the use of topical and/or systemic steroids should be considered, as should the use of isotretinoin. The pros and cons of epilating procedures, with either lasers or radiation, must be discussed.

If a patient can be brought to a complete remission with only topical maintenance , then the patient can be followed monthly for a few months and then discharged. If total remission has not occurred, additional courses of full therapy should be implemented.

Surgery, as utilized for dissecting cellulitis or severe acne keloidalis, is not recommended for folliculitis decalvans, as it may be grossly disfiguring. Because the cosmetic appearance of the condition may be of great concern to the patient, referral to a hair stylist, shaving the scalp (in men), or use of hairpieces, should be considered.

Unusual Clinical Scenarios to Consider in Patient Management

Erlotinib has been given at 150mg per day for 6 months for lung-cancer-induced folliculitis decalvans; however, the disease remitted on antibiotics and topical steroids while erlotinib was continued. Pustules and erythema disappeared, and much of the hair returned.

Although truncal folliculitis is very common in epidermal growth factor receptor tyrosine kinase inhibitor (EGFr-TKI) therapy for cancer, disease limited to the scalp is very rare and is not associated with positive skin cultures.

What is the Evidence?

Brooke, R, Griffiths, C. “Folliculitis decalvans”. Clin Exp Dermatol. vol. 26. 2001. pp. 120-2. (A 3-year follow-up of remissions from Powell’s original studies.)

Chiarini, C, Torchia, D, Bianchi, B. “Immunopathogenesis of folliculitis decalvans: clues in early lesions”. Am J Clin Pathol. vol. 130. 2008. pp. 526-4. (A beginning theory of pathogenesis, based on stainable markers of cytokines.)

Otberg, N, Kang, H, Abdullateef, A, Shapiro, J. “Folliculitis decalvans”. Dermatologic Therapy. vol. 21. 2008. pp. 238-44. (A classic review.)

Parlette, E, Kroeger, N, Ross, EV. “Nd:YAG laser treatment of recalcitrant folliculitis decalvans”. Dermatol Surg. vol. 30. 2004. pp. 1152-4. (Case report with excellent cosmetic remission by eight Nd:YAG laser treatments.)

Powell, J, Dawber, R, Gatter, K. “Folliculitis decalvans including tufted folliculitis: clinical, histological and therapeutic findings”. Br J Dermatol. vol. 140. 1999. pp. 328-33. (Classic article describing folliculitis decalvans and tufted folliculitis with consideration of effective therapy.)

Powell, J. “Folliculitis decalvans and tufted folliculitis are specific infective diseases that may lead to scarring but are not a subset of central centrifugal scarring alopecia”. Arch Dermatol. vol. 137. 2001. pp. 373(Point and counterpoint about the role of S. aureus in scarring alopecia.)

Sillani, C, Zhang, B, Zhao, Y. “Effective treatment of folliculitis decalvans using selected antimicrobial agents”. Int J Trichology. vol. 2. 2010. pp. 20-3. (This study of thirteen Chinese patients confirms that, although this condition is typically observed in patients of African descent, the condition is not limited to a specific racial group.)

Sperling, L. “A new look at scarring alopecia”. Arch Dermatol. vol. 136. 2000. pp. 235-42. (A seminal article on new categories for scarring alopecia.)

Stefanato, C. “Histopathology of alopecia: a clinicopathological approach to diagnosis”. Histopathology. vol. 56. 2010. pp. 24-38. (Illustrative review of alopecia histologic workup.)

Walker, SL, Smith, HR, Lun, K, Griffiths, WAD. “Improvement of folliculitis decalvans following shaving of the scalp”. Br J Dermatol. vol. 142. 2000. pp. 1245-6. (Did shaving alter the milieu for Staphylococcus and prevent its recurrence?)

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