Management of Primary Cicatricial Alopecias: Options for Treatment

M.J. Harries; R.D. Sinclair; S. MacDonald-Hull; D.A. Whiting; C.E.M. Griffiths; R. Paus

Disclosures

The British Journal of Dermatology. 2008;159(1):1-22. 

In This Article

Neutrophilic group

Folliculitis decalvans (FD) is a rare, progressive purulent folliculitis that may involve any hair-bearing site, although it is most common on the vertex of the scalp. Usually there is only a single focus of disease; however, additional foci may evolve over years.[251] It is characterized initially by painful follicular pustules that become crusted. Patches of alopecia develop from an expanding zone of folliculitis, eventually resulting in a central area of scarring. Unlike CCLE and LPP the scar is indurated and boggy rather than atrophic, at least in the early stages. Multiple hair tufts may be found emerging from a common dilated follicular opening, giving the appearance of a doll's hair (Figure 4). Staphylococcus aureus is frequently cultured and has been implicated in the pathogenesis of this disorder.[252,253] No malfunction in systemic or local immune response has, however, been demonstrated.[253]

Boggy scarring alopecia with hair tufting and occasional pustules (diagnosis = folliculitis decalvans).

Histopathology reveals perifollicular neutrophilic inflammation around the upper follicle, which later develops into a more mixed inflammatory infiltrate of neutrophils, lymphocytes and plasma cells. Follicular rupture ensues, resulting in granuloma formation around exposed hair shaft fragments.

Treatment.

First-line Treatment.

Oral antibiotic±topical antibiotic (Level of evidence = C)

Single-agent oral (or topical) antibiotics should be chosen based on organism cultures and antibiotic sensitivity tests. Antistaphylococcal antibiotics are the most commonly used treatments and are reported to improve FD; however, the condition often recurs quickly on stopping therapy.[254,255,256,257,258,259,260,261] Topical nadifloxacin ointment applied twice daily improved two patients, with no expansion of alopecia being observed after 1year of follow-up.[262]

Second-line Treatment.

Oral rifampicin and oral clindamycin (Level of evidence = C)

Powell and Dawber originally reported 18 patients with FD who were treated with a 10-week course of rifampicin (300mg twice daily) and clindamycin (300mg twice daily) given in combination. Fifteen of the 18 patients achieved a prolonged treatment-free remission without relapse after one or more courses of this treatment. One patient failed to respond and was subsequently found to have rifampicin-resistant S.aureus in the lesional skin. The same authors have subsequently reported a further 20 patients with FD treated in the same way, again with good results.[252,253] Other researchers have also reported good rates of clearance and prolonged periods of remission with this therapy.[263,264,265,266,267] It should be noted that clindamycin has a propensity for inducing Clostridium difficile diarrhoea. If diarrhoea develops during treatment, or if the risk of C.difficile infection is deemed too great, possible substitutes for clindamycin include doxycycline, ciprofloxacin or clarithromycin.[253] Oral rifampicin combined with topical antibiotics has also had reported success.[254,268] Monotherapy with rifampicin is not recommended due to rapid emergence of resistant organisms.[253]

Third-line treatment.

Oral fusidic acid±oral zinc (Level of evidence = E)

Oral zinc (Level of evidence = E)

Dapsone (Level of evidence = E)

Sulfamethoxazole-trimethoprim (Level of evidence = E)

Surgical excision (Level of evidence = E)

Laser epilation (Level of evidence = E)

Radiotherapy epilation (Level of evidence = E)

Shave scalp (Level of evidence = E)

Oral L-tyrosine (Level of evidence = E)

Oral steroids PLUS low-dose isotretinoin (Level of evidence = E)

Oral retinoids (Level of evidence = E)

Topical ciclosporin (Level of evidence = E)

Intramuscular immunoglobulin (Level of evidence = E)

Oral fusidic acid (for 3weeks) combined with oral zinc (continued for 6months) improved three patients. Two remained clear at 1year follow-up.[269] Karakuzu et al.[270] also reported benefit; however, others have had unsatisfactory responses even using zinc at superphysiological doses.[254,271] Fusidic acid as monotherapy has also been reported as beneficial; however, the treatment regimens were not supplied.[272,273] Resistance may also develop with prolonged, or multiple, treatment courses.

Dapsone (75–100mg daily) for 4–6months, followed by a maintenance dose of 25mg daily, resulted in prolonged clearance in two patients.[274] Others have also reported benefit.[275] Whiting has reported success with sulfamethoxazole-trimethoprim, but specific details were not supplied.[8]

Excision is reported as effective in three cases.[276,277,278] Laser[279] or radiotherapy[280] epilation of the entire scalp may improve FD; however, scarring and permanent hair loss are potential side-effects. One patient noticed disease improvement with scalp shaving.[281] Oral l-tyrosine (1.5g twice daily for 10weeks) reduced erythema in one patient.[282] Combination treatment with oral steroids and isotretinoin helped two patients.[271,283]

Oral 13-cis retinoic acid (1mgkg−1 daily for 20weeks) induced remission in one patient, but relapse occurred with treatment withdrawal.[255] All three patients treated with retinoids in one series failed to respond[280,281,284] and FD was observed developing in one patient already taking retinoids for Darier's disease.[263] One patient treated with topical applications of ciclosporin (diluted in 1-methyl-2-pyrrolidinone and ethylic acid; final conc. = 1.5%), applied twice daily (1mLcm−2 of scalp) for 1month then daily for 3months, achieved complete remission at 1year and developed some sporadic hair regrowth.[285]

All three patients with FD treated with intramuscular human immunoglobulin (12.38mgkg−1 monthly) showed a 50–70% response rate.[286]

Dissecting cellulitis of the scalp (DCS) is an uncommon suppurative disease of the scalp. It is characterized by painful fluctuant nodules, abscesses and interconnecting sinus tracts, which evolve into scarring alopecia. It predominantly affects black men in their second to fourth decades and runs a chronic, relapsing course.[287] DCS is associated with acne conglobata and hidradenitis suppurativa.[288] These conditions all have the common pathogenic mechanism of follicular hyperkeratosis causing follicular occlusion. Retention of follicular products ensues predisposing to secondary bacterial infection and follicular rupture. Keratinous debris is extruded into the dermis, resulting in a foreign-body reaction and eventual scarring.

Treatment.

First-line treatment.

Oral isotretinoin (Level of evidence = D)

Several case reports have claimed benefit with isotretinoin (1mgkg−1 daily). Treatment duration of between 6 and 11months is advocated as lesions may take some time to flatten. Long-term, treatment-free remission may result.[289,290,291,292,293,294,295,296,297,298,299,300] Combination therapy with oral isotretinoin and 2-weekly ILTAC injections (40mgmL−1) resulted in a 2-year remission in one patient.[301] In severe cases the addition of oral corticosteroids is recommended.[8]

Second-line treatment.

Oral antibiotics (Level of evidence = E)

Aspiration PLUS intralesional triamcinolone acetonide (Level of evidence = E)

Oral zinc (Level of evidence = E)

Topical antibiotics PLUS topical retinoids (Level of evidence = E)

Oxytetracycline (1g daily)[302] and trimethoprim (100mg twice daily)[303] have been reported to improve individual patients; however, others were unsuccessful with antibiotic therapy.[304] Antibiotics combined with repeated aspiration resulted in improvement in two patients[305] and oral antibiotics combined with oral corticosteroids have also been shown to be beneficial.[306,307] Aspiration of lesions followed by ILTAC (every 4 or 5days) improved three patients; however, quick relapse occurred on stopping therapy.[304]

Oral zinc sulphate, 400mg daily for 12–22weeks, resulted in remission in two patients. One quickly relapsed on stopping treatment whereas the other remained disease free for 5
years.[308,309]

Combination of topical clindamycin gel with topical isotretinoin gel for 2months, followed by isotretinoin gel alone for a further 8months, resulted in a 1-year remission in one patient.[310] After 12months follow-up one patient's condition had 'improved significantly' with oral clindamycin (600mg three times daily for 6weeks) combined with topical isotretinoin.[311]

Third-line treatment.

Alternate day oral corticosteroids (Level of evidence = E)

Colchicine (Level of evidence = E)

Dapsone (Level of evidence = E)

Laser epilation (Level of evidence = C)

Radiotherapy epilation (Level of evidence = C)

Excision and skin grafting (Level of evidence = D)

Carbon dioxide laser (Level of evidence = E)

Alternate day low-dose corticosteroids[303,312] and colchicine (0.6mg twice daily for 2months)[313] have helped individual patients. Halder claims dapsone is 'very effective' without supplying patient details.[287] Others have found no benefit of dapsone in DCS.[245,313] Laser epilation and radiotherapy epilation have both been reported as successful.[245,313–316] Permanent hair loss may ensue; however, it is argued that this side-effect is preferable to active disease. Excision with split thickness grafting,[317,318,319,320,321] marsupialization[322] and incision and drainage (combined with isotretinoin)[323] are reported as effective in a few cases. Combination treatment using tissue expansion, radical excision and oral isotretinoin (perioperatively and for 3months postoperatively) resulted in complete remission at 1year follow-up in one patient.[324] Carbon dioxide laser excision of nodules was reported as beneficial in one case.[325]

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