Updates in Lichen plano pilaris

1. Updates in Lichen planopilaris f,.fatemi,MD Isfahan university of medical sciences

2. The incidence of LPP has increased significantly during the last decade and we may be facing an epidemic of the disease

4. CONCLUSION • cicatricial alopecia is more common in women. • The most common type is LPP. (69.7% of all patients). • Most of the cases were from 30-39 years old. • so early diagnosis and treatment is very important in these patients. • Other studies showed : • Higher prevalence rate of PCA in Caucasian

5. CLINICAL VARIENTS A-classic lichen plano pilaris

6. Objective signs • E • PFE • FK • PFS • Subjective symptoms • Pain • Pruritus • Soreness • (LPP scoring system) • Classic Lichen planopilaris (LPP) characterized by patchy cicatricial alopecia with objective signs & subjective symptoms. PFSive

9. Is still topical steroid as the first line treatment ??? • SR 1 • highest level of evidence: IV • total number of patients: 128 • global response rate: 53.9% [69 of 128] • response rate in monotherapy: 53.3% [49 of 92]) • SR2 • Improvement and stabilization were observed in almost one third of the topical steroid users . • They stated :Our findings oppose the previous systematic review that proposed topical corticosteroids as the first-line treatment modality for LPP patients

10. The response rates to treatment significantly decreased with subsequent therapies: • 18 of the 32 patients responded to first-line treatments, compared with 7 of 19 who received second-line treatment and only 1 of 9 patients who received third-line treatment. • Our recommendation : • Altough in some guidelines topical steroid cosider as the first line ,it is best to use it as adjunctive therapy with systemic modalities ,with the exception of the very limited cases.

11. Topical steroid twice daily for the first month , followed by an application once a day for 3 mo, and then every other day for 3 more months.

12. How about the systemic steroid ? • Although some authors have advocated the use of systemic oral corticosteroid therapy as a second-line treatment (prednisone 1 mg/kg/day for 15 days, tapered over 4 months),

13. SR1 • level of evidence: IV • Total number of patients: 15 • response rate in monotherapy: 71.4% SR2 • The only conducted study about the efficacy of oral corticosteroids monotherapy showed no improvement in the course of disease. The very high degree of relapse (around 80% of patients) after treatment suspension makes such a therapy little useful in the long-term period.

14. What about IL steroids ??? • SR 1 • highest level of evidence: IV • Total number of patients: 30 • Global response rate: 56.7% [17 of 30] • Response rate in monotherapy: 50.0% [13 of 24]) • SR2 • Improvement and stabilization in FFA was found in 83% of intralesional steroid users.

15. Our recommendation: • Because of not superiority of IL steroids VS topical steroids & regarding to its low patients compliance : • It is the best first line treatment for FFA.

16. which systemic drug ?

17. MTX as a promising treatment for LPP ….

19. Interestingly, this study revealed : • not only a significant superiority of MTX over hydroxychloroquine, • but also the very limited response of recalcitrant LPP to the latter medication (efficacy only on erythema degree), differently from methotrexate which showed efficacy on pruritus as well as on all the objective variables assessed in the study (erythema, perifollicular erythema, perifollicular scaling, spreading, and follicular keratosis). • However there are several reports showing positive results, with a response rate in monotherapy of 51.0% considering all the cases reported in the literature. • It is possible to speculate that the negative outcomes observed in the abovementioned clinical trial could be due to the fact that it was focused only on recalcitrant cases.

21. Refractory LPP defined as no response to treatment with topical agents/systematic medications in the past 6 months

22. Our recomendation: If you are sure in the diagnosis of LPP Start systemic therapy with MTX In obscured cases or the contraindicated situations for MTX start with HQ and If the patient didn’t respond after 3 mo switch to MTX if possible

23. CLINICAL VARIENTS B-frontal fibrosing alopecia

24. Frontal fibrosing alopecia (FFA) is considered a particular form of LPP, primarily affecting the hair follicles in the frontotemporal area of the scalp, with the hairline recession and eyebrow loss.

25. Epidemiology of ffa • Ten-fold increase in the number of cases seen annuallyover the last decade . • over 85% of the patients are white postmenopausal women. • from 18 to 87 years with the highest incidence in sixth decade. • In male and female patients.

26. Associated disorders : • A high incidence of early menopause, up to 17%,compared with an incidence of 6% in the general population. • Dyslipidemia, hypothyroidism, hypertension, and osteoporosis • Thyroid diseases are probably the main association (9% -23%). • Previous or concurrent lichen planus (LP) 2% to 17%. (Compared to LPP, in which LP is found in 28%-50% of cases.) • Associated with vitiligo, alopecia areata , rheumatoid arthritis, lupus erythematosus, and polymyositis , psoriasis , atopy.

27. FFA has also been described in patients following hair transplantation and face-lift surgery • An association between beta-blockers and NSAIDS • A possible protective effect of ACEIs

28. The LPP & FFA are the same ?? • Several studies showed that in FFA : • Lymphocytes selectively affect the HF of the frontal margin and eyebrows • Involves hair follicles of different types: terminal, intermediate, and vellus • involves hair follicles different stages of the cycling (from anagen to telogen) . The reason for this selection is still unknown.

29. Etiology: • HORMONAL INFLUENCE ? • GENETIC FACTORS ? • Microbial antigens or superantigens? • ENVIRONMENTAL TRIGGERS • Hair care products? • Sunsreens? • Fregrance & cosmetics?

30. PPARγ DEFICIENCY & SEBACEOUS GLAND DESTRUCTION • Peroxisome proliferator-activated receptor gamma (PPARγ) is a member of the nuclear receptor supergene family that regulates the expression of genes involved in : • Lipid homeostasis • Inflammatory responses • Has a crucial role in maintaining the pilosebaceous unit. • Recent gene expression studies identified: • A deficiency in PPARγ-mediated signaling in LPP patientsand suggested that loss of this function may trigger pathogenesis of LPP. • Several authors reported the efficacy of PPARγ-agonists in treatment of LPP. The deficiency of PPARγhas yet to be studied in FFA

31. Recently the possible link between aryl hydrocarbon receptor (AHR) over expression in CA was suggested. • Since the ligand for the AHR is activated by dioxin, the possibility that dioxin-like substances can trigger the disease discussed recently, but remains unproven.

33. Dioxin-like chemicals are environmental pollutants that are ingested mostly with food of animal origin: Meat, dairy products, or fish predominate • These toxins persist in the environment and are very slowly eliminated from the human body. • Chronic low dose exposure may lead to the accumulation of dioxins in lipid-rich regions such as sebaceous glands, causing loss of PPARγ expressionand thereby scarring alopecia in susceptible individuals.

34. Sensitive groups • Some people may be exposed to higher levels of dioxins because of : • Their diet (such as high consumers of fish in certain parts of the world) • Their occupation (such as workers in the pulp and paper industry, in incineration plants, and at hazardous waste sites).

35. What should consumers do to reduce their risk of exposure? • Trimming fat from meat • consuming low fat dairy products • A balanced diet (adequate amounts of fruits, vegetables, cereals)

36. photoallergic reaction to pyridoxine hydrochloridefound in a wide variety of detergents, soap & skin & hair care products, needs to be evaluated as potential trigger for scarring alopecia. • Up to date their role remains unclear.

37. Clinical picture

38. Although the frontal area is most commonly affected, may appear on other sites, such as the temporo-parietal as well as the retroauricular and occipital areas. • May involving the entire hairline and leaving only a band of hair on the top of the scalp, which is described as “clown alopecia”. • Increased venous vasculature on the temples is also a common observation

39. The striking feature is the unnatural appearance of the hairline caused by loss of both terminal and vellus hair • “lonely hair“ is a useful diagnostic clue • Eyebrow diminution, a characteristic feature . • Hair loss from the lateral third of the eyebrows is typical, but in some cases, there may be diffuse thinning or total loss of eyebrows • Eyelash involvement was also reported

40. The skin between the old and new hairlines is atrophic

41. patients with the pseudo “fringe sign” had the best prognosis • patients with the “diffuse pattern” presented with the worst.

42. CLINICAL VARIENTS C-facia lichenplanopilaris

43. Recently (2007)there are case reports of : involvement of facial vellus hairs , characterized by roughening of the facial skin. The involvement of facial vellus hairs, clinicallypresenting as : pinpoint, skin-colored papules & A decrease or absence of vellus hairs

44. Histopathologic features of facial papules might not be limited to perifollicular inflammation, and that structural changes involving elastic fibers and sebaceous glandscould be responsible for their clinical formation. • An abnormal elastic framework could be responsible for remodeling the shape of sebaceous lobules and ducts, leading to the “pop out” of sebaceous glands and the formation of facial papules.

45. We believe that this diagnosis is often missed and may be more prevalent then it appears from the literature. • We as dermatologists have to be aware of this entity and learn to recognize these subtle clinical changes. • Further follow-up needs to early diagnosis and treatment of FFA.

46. 14% of FFA patients present with facial papules. the involvement of facial vellus hairs in FFA is thought to be associated with a more severe form of FFA and higher disease activity. Lichen planus pigmentosus (LPPigm), a macular variant of lichen planus, may be seen in association with FFA, especially in dark-skinned patients

47. In the original description, LPPigm preceded hair loss in all subjects and was therefore suggested as a herald sign of FFA. • In our patients Usually facial LPP also proceededthe onset of hair loss so : It would be advisable to monitor patients with facial vellus involvement or LPPigm more closely, to detect any alterations in scalp or eyebrow hair early, and evaluate the need for systemic treatment.

50. treatment