Lichen planopilaris (LPP) is a primary lymphocytic scarring alopecia that causes inflammation, erythema, pruritus, dysesthesia, and alopecia that can be treatment resistant. After approval from the institutional review board, we performed a retrospective case analysis of alopecia due to LPP to assess possible therapeutic effectiveness.
All medical charts with International Classification of Diseases, Ninth Revision (ICD-9) diagnoses of alopecia (2004-2007) were analyzed: 674 cases were nonscarring (81%), and 159 were scarring alopecia (19%). Based on clinicopathologic correlation, LPP was diagnosed in 45 cases (28% scarring alopecia, 5% overall), and all patients were diagnosed by the same observer (J.C.E.). According to the criteria of the North American Hair Research Society,1 histologic evidence of lymphocytic scarring inflammation includes lupus erythematosus (LE), classic pseudopelade, central centrifugal cicatricial alopecia, alopecia mucinosa, keratosis follicularis spinulosa decalvans, and LPP.1
Lichen planopilaris and LE require close scrutiny for distinction. Histologic criteria used to distinguish LPP from LE were superficial infiltrate, lack of basement membrane thickening, excess mucin, epidermal thinning, or telangiectasia. Clinically, a diagnosis of LPP was favored over LE if the alopecia had perifollicular hyperkeratosis and erythema, lack of follicular plugging, ill-defined areas of involvement, and no evidence of perilesional hyperpigmentation. Data revealed that 42 of the 45 patients were white (93%), 31 were female (69%), and average age at the time of diagnosis was 51 years. Interestingly, 3 of the 45 patients were diagnosed with a frontal fibrosing alopecia variant with eyebrow loss, and 1 of 45 had Graham-Little-Piccardi-Lasseur syndrome. These numbers substantiate previously reported data regarding prevalence and patient characteristics of LPP.2
Twenty-nine patients with LPP met the criteria for analysis of treatment interventions. Those who did not return for follow-up, failed to obtain initial laboratory work, sought care elsewhere, or refused to undergo biopsy were not included in the analysis. Improvement was defined at follow-up as the absence of reported symptoms (pruritus, burning, and/or dysesthesia), lack of progression, reduction in erythema and follicular hyperkeratosis found on examination, and the ability to discontinue therapy. Most of the patients underwent treatment with supplemental topical steroids, topical minoxidil, 5%, and/or oral biotin with or without orthosilicic acid. A summary of instituted systemic immunosuppression approaches appears in the Table.
For 15 patients, an initial tetracycline therapeutic choice (doxycycline hyclate) was given based its relatively low adverse effect profile and lack of laboratory monitoring required: 27% of patients showed improvement (n = 4). Twenty-two of our patients took hydroxychloroquine sulfate during their therapy (some initially, others after doxycycline failure) with 9 of 22 showing improvement (41%). The Fisher exact test comparing these initial treatments did not find significant differences (P = .74). Of the patients for whom hydroxychloroquine treatment failed, 10 were treated with mycophenolate mofetil; none received this therapy without a failed trial of hydroxychloroquine. Three patients showed improvement under treatment with mycophenolate mofetil (30%). For those who did not improve, subsequent transition to methotrexate therapy in 1 patient was unsuccessful, and 2 of 3 patients who attempted acitretin treatment found success. The Fisher exact test comparing mycophenolate mofetil and acitretin found no significant difference (P = .51).
Many systemic agents have been used to treat LPP with limited success.3- 5 Our retrospective analysis corroborates that a consistent and successful therapy for refractory disease remains elusive.
Limitations to this study include its retrospective nature and end points that did not include percentage of scalp hair loss or hair counts. Clinically, we suggest offering patients stepwise therapy with doxycycline or hydroxychloroquine as first-line systemic agents. Our data do not suggest that one is superior. If needed, transition to mycophenolate mofetil or acitretin therapy can be made for relief of symptoms, but potential for adverse effects must be considered. In our experience, the risk-benefit ratios of other agents (eg, cyclosporine and thalidomide) preclude their use for this non–life-threatening condition. Evidence-based therapy with a prospective, multicenter, randomized, placebo-controlled study with set end points is needed.
Correspondence: Dr English, Department of Dermatology, University of Pittsburgh, 190 Lothrop St, Ste 145, Lothrop Hall, Pittsburgh, PA 15213 (email@example.com).
Author Contributions:Study concept and design: Spencer and English. Acquisition of data: Spencer, Hawryluk, and English. Analysis and interpretation of data: Spencer, Hawryluk, and English. Drafting of the manuscript: Spencer and Hawryluk. Critical revision of the manuscript for important intellectual content: Spencer, Hawryluk, and English. Administrative, technical, and material support: English. Study supervision: English.
Financial Disclosure: None reported.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Dermatology editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 8
Customize your page view by dragging & repositioning the boxes below.
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.