This is open label single side study involvement 20 patient treated with Apremilast. Each enrolled patient may be evaluated at by a dermatologist using the Lichen Planopilaris Activity Index and Frontal Fibrosing Alopecia Index. Other measures include physician global assessment, dermatology quality of life and patients analogue score for pruritus. Pt will have visits at Week 0,2,4,8,12,16,20,24
Frontal fibrosing alopecia (FFA) is a chronic immune mediated inflammatory disease characterized by inflammation of the hair follicle and scaring hair loss. Clinically, FFA presents as a progressive recession of the hairline in a frontal temporal distribution. Evidence suggests that timely and effective management can prevent the permanent loss of hair. Unfortunately, most current treatment have been disappointing with poor efficacy or high risk profile. The available of a safe effective treatment for this disease remains an unmet need Apremilast is a novel phosphodiesterase 4 inhibitor currently FDA approved to treat psoriasis and is under investigation for other auto immune conditions. The medication has a good safety profile with no required laboratory monitoring. This study primarily aimsto determine whether Apremilast offers any benefit for this difficult to treatment population
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
20
Open label treatment with apremilast
Bellevue Dermatology
Bellevue, Washington, United States
RECRUITINGLichen Planopilaris index
The weights given to the symptoms (30%), signs (30%), anagen pull test (25%), and presence of spreading (15%) led to the equation: LPPAI (0-10) = (pruritus + pain + burning)/3 + (scalp erythema + perifollicular erythema + perifollicular scale)/3 + 2.5 (pull test) + 1.5 (spreading/2). Symptoms and signs are recorded on a 4-point scale. The anagen pull test involves grasping a small group of 10 to 20 hairs between the thumb, second finger, and third finger at the scalp end of the hair shafts, and pulling away from the scalp with a slow, firm perpendicular force to slide the fingers to the ends of the hair. The result is recorded both as a binary value (0 for no anagen hairs and 1 for the presence of anagen hairs) and as anagen hairs/total hairs pulled. Last is the assessment of spreading, recorded as 0 (no spreading) versus 1 (indeterminate) versus 2 (spreading). Our primary endpoint is percentage change from baseline
Time frame: Week 0 to 24, patient visit week0,2,4,8,12,16,20,24
Physicians global assessment
Physicians global assessment This is a a five point scale used to measure the severity of the disease at the time of the physicians evaluation. 0-Clear, 1-Almost Clear, 2-Mild, 3-Moderate, 4-Severe The lower the score the better the score
Time frame: Weeks 0 to 24, patient visit week 0,2,4,8,12,16,20,24
Visual Analogue Scale Pruritus
Patient draws a line that best represent the severity of itch: this is continuous scale from 0-10, 0 being no itching and 10 being worst possible itching. Patient is to draw a line along the scale that represent the itch.
Time frame: Weeks 0 to 24, patient visit week 0,2,4,8,12,16,20,24
Frontal Fibrosing Alopecia Index
This is a scoring system recently proposed by Holmes et al from the British Nail and Hair society. FFASI was compiled in two forms: FFASI and FFASI B. FFASI utilizes clinical images of the entire hairline, divided into four sections. Alopecia severity is graded 1-5 based on hairline recession. In order that hairline recession comprises the greatest proportion of the assessment, each grade is weighted. Nonscalp hair loss (eyebrow, eyelash, limb and flexural) are scored, as are associated features (facial papules; cutaneous, nail and mucosal lichen planus;and generalized scalp lichen planopilaris). Scores for hairline recession, inflammatory band, nonscalp loss and associated features may be combined to give a maximum score of 100. FFASI B uses the same format, but rather than grading alopecia it permits user-defined measurement of each hairline section. We will look at mean change in FFASI compared to baseline
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Time frame: Weeks 0 to 24, patient visit weeks 0,2,4,8,12,16,20,24
Dermatology Quality of Life (DLQI) (Appendix E) Dermatology Quality of Life (DLQI) (Appendix E) Dermatology Quality of Life (DLQI)
This is a questionnaire which measures how much a subjects skin problems affect his life over the last week. It is a series of 10 questions regarding various daily activities and each question consist of 4 responses from Very much, A lot, A little to Not at all. 0-being not at all or not relevant , 1-a little, 2-a lot, 3-very much in addition question 7, 3-prevent work or studying, the scores are added together for total score. Interpretation of score: 0-1 No affect on patient's life, 2-4 small affect on patient's life, 6-10 moderate affect on patient's life, 11-20 large on affect on patien's life, 21-30 extreme affect on patient's life
Time frame: Weeks 0 to 24, patient visit weeks 0,2,4,8,12,16,20,24