Begrijpen en Behandelen van Lichen Planopilaris - Huidziekten onder de Loep

What is the Evidence?

Chew, A, Bashir, S, Wain, M, Fenton, DA, Stefanato, CM. “Expanding the spectrum of frontal fibrosing alopecia: A unifying concept”. J Am Acad Dermatol. vol. 63. 2010. pp. 653-60. (Extensive review of FFA showing the destruction is more generalized than thought even without evidence of clinical inflammation.)

Chiang, C, Sah, D, Cho, B, Ochoa, BE, Price, VH. “Hydroxychloroquine and lichen planopilaris: Efficacy and introduction of Lichen Planopilaris Activity Index scoring system”. J Am Acad Dermatol. vol. 62. 2010. pp. 387-92. (Landmark evaluation system even though subjective and with a personally weighted scale shows symptomatic and visible improvement of at least 25% in 83% of patients by 1 year.)

Cho, BK, Sah, D, Chwalek, J, Roseborough, I, Ochoa, B, Chiang, C. “Efficacy and safety of mycophenolate mofetil for lichen planopilaris”. J Am Acad Dermatol. vol. 62. 2010. pp. 393-7. (Even hydroxychloroquine-resistant patients showed improvement by LPPAI index within just 6 months in 10 of 12 instances with dosing of 500mg twice a day for 1 month, then 1000mg orally twice a day for many months.)

Garcovich, S, Manco, S, Zampetti, A, Amerio, P, Garcovich, A. “Onset of lichen planopilaris during treatment with etanercept”. Br J Dermatol. vol. 158. 2008. pp. 1161 (A case report of a 56-year-old Italian woman with psoriatic arthritis who responded to etanercept, however, by week 32 she developed severe and painful LPP of her scalp.)

Harries, M, Paus, R. “Scarring alopecia and the PPAR-γ Connection”. J Invest Dermatol. vol. 129. 2009. pp. 1066-70. (A fascinating review of preliminary data based on a mouse model showing sebaceous gland dysfunction and downregulation of PPAR-gamma gene and changes in LPP patients showing abnormalities in similar pathways—pathogenesis of LPP?)

Karnik, P, Tekeste, Z, McCormick, TS, Gilliam, AC, Price, VH, Cooper, KD. “Hair follicle stem cell-specific PPARc deletion causes scarring alopecia”. J Invest Dermatol. vol. 129. 2009. pp. 1243-57. (Hair follicle stem cell-specific PPARc deletion or downregulation causes scarring alopecia of mice and women.)

RESULTS

A total of 31 Caucasian patients (20 females and 11 males) with a median age of 40 years (range 23–66 years) were recruited. None of the patients had a family history of FD or LPP. The median age of onset of the condition was 34 years (range 22–51 years). The associated dermatological comorbidities were: androgenetic alopecia (17 patients, 54.8%), seborrhoeic dermatitis on the scalp (9 patients, 29.0%), atopic dermatitis in 2 patients and vulvar lichen sclerosus in 1 patient.

Clinical presentation of folliculitis decalvans and lichen planopilaris phenotypic spectrum. A unique cicatricial plaque of alopecia with hair tufts of 6 or more hair follicles.

Trichoscopy revealed hair tufting and perifollicular white scaling in all cases ( Fig. 2 ) (the remaining tricho-scopic features are summarized in Table I ).

Table I

Trichoscopic features of patients with folliculitis decalvans and lichen planopilaris phenotypic spectrum

Trichoscopy of folliculitis decalvans and lichen planopilaris phenotypic spectrum. Loss of follicular openings and hair tufting. White perifollicular scales and ingrowing hairs are visible.

After statistical analysis, the duration of the condition was the only factor associated with grade III forms of FDLPPPS (p = 0.026). The presence of white dots on trichoscopy was higher in grade I–II cases than in severe forms of the disease (p = 0.015). No other clinical factors were correlated with the severity of the disease.

DISCUSSION

Despite these options, the literature has limited evidence on treatments for this challenging disease. Reports mostly involve small groups of patients and frequently reveal varied or poor clinical response. 2 Amongst individuals who do respond to therapy, hair regrowth within alopecic patches is exceedingly rare. Evidence in the literature is limited to several case reports, including a 27-year-old male with regrowth after initiation of oral tofacitinib and dapsone, and a 42-year-old woman with regrowth using low level light therapy. 5,6 Due to the lack of large, randomized controlled trials guiding therapeutic management, dermatologists may choose to offer patients well tolerated, alternative treatments including low-dose naltrexone and PRP, as is described in our case report.

Low-dose naltrexone (LDN), a μ-opioid antagonist with antiinflammatory properties, has been successfully utilized in the treatment of patients with LPP, leading to reduced symptoms of pruritus, decreased evidence of scalp inflammation, and slowed disease progression. 7 However, a randomized controlled trial published by Lajevardi et al found that while LDN at 3 mg daily improves the severity of LPP, the improvements are not superior to those achieved with topical clobetasol. 8 Similarly, literature on the use of PRP as an adjunctive treatment for LPP suggests only mild benefit. In a case study of 10 LPP patients treated with PRP, 3 demonstrated reduced inflammation and decreased hair loss, but none exhibited hair regrowth. 9

This case was remarkable given the patient’s dramatic hair regrowth in the setting of cicatricial alopecia. Though our patient was initially treated with traditional therapies, consistent with treatment algorithms proposed in the literature, her clinical response was observed only after the addition of more novel interventions, including low-dose naltrexone and PRP. 2 This case highlights the importance of remaining flexible and diligent in therapeutic approaches to LPP and the need for more robust literature regarding prognosis and treatment options for LPP.