Lichen Planopilaris



Lichen planopilaris (LPP) is a rare chronic scarring folliculitis of the scalp of unknown cause.



It is a primary scarring alopecia.



The  follicle itself is targeted by inflammatory cells and scarring occurs around the follicular unit. 



Because T cells are the overwhelmingly present inflammatory cell.



It is a lymphocytic primary scarring alopecia.



It is part of the lichen planus complex.



Typical lesions of lichen planus are not seen in the scalp.



About 30% of patients may present with typical cutaneous or mucosal lesions of lichen planus elsewhere. 



Predominantly affects the vertex and occasionally the parietal scalp alone.



Other presentations—frontal fibrosing alopecia (FFA) and Piccardi–Lassueur–Graham–Little syndrome (PLGLS ) are part of the lichen complex.



Most patients, 70-80% are 


women, and almost all are postmenopausal.



The average age is 53.



Almost all patients with LPP are Caucasian, although all races are affected.



Hair loss develops on the scalp and progresses in an  irregular pattern with eventual merging of hairless patches. 



Occasionally a more diffuse uniform alopecic pattern of the central vertex with peripheral spreading occurs. 



Nonscalp lesions of typical lichen planus may be concurrent.



Findings: Perifollicular erythema and scaling, with loss of follicular ostia.



The follicular lesions are papules, and not pustules, which may be red or violaceous.



These manifestations are measurable criteria for following the course of the disease and its treatment



It is usually associated with pruritus and pain, severe burning sensation and tenderness are common. 



The accepted diagnostic test is a positive anagen pull test.



By pulling on 10 to 20 hairs with moderate traction there will be a  show release of many hairs with a great preponderance of anagen, not telogen, hairs.



Clinical confusion with cutaneous lupus exists.



Frontal fibrosing alopecia (FFA) is a rare  variant of the disease making up about 8% to 15% of all LPP cases.



Frontal fibrosing alopecia affects mostly, but not solely, postmenopausal women presenting with an expanding band of alopecia along the frontal hairline. 



Alopecia of eyebrows, occurs in 20% of cases.



FFA shows a loss of ostia and the presence of perifollicular scale and erythema not evident in alopecia areata.



There is an inflammatory infiltrate of lymphocytes with variable perifollicular fibrosis and absence of thickened basement membrane as confirmed by a periodic acid–Schiff stain.



Eventually the inflammation results in loss of sebaceous glands, arrector pili muscles, perifollicular fibrosis, and permanent destruction of the follicle with replacement with perfollicular hyalinization in the upper and lower dermis.



It has no consistently abnormal laboratory or hormonal findings.



Frontal fibrosing alopecia is an even more rare disease affecting mostly, but not solely, postmenopausal women.



b-FGF and TGF-β induce activation of fibroblasts and are abundantly produced.



Treatment options: 















Intralesional: Corticosteroids (triamcinolone acetonide 3.5 to 10mg/cc)
























Mycophenolate mofetil






Pioglitazone therapeutically at 15mg daily in combination with mycophenolate mofetil 500 to 1000mg orally twice daily have been used with effectiveness.



Hydroxychloroquine showed 83% improvement in patients with LPP.



Hydroxychloroquine supplemented by topical tacrolimus, and minoxidil are first line treatments.



Mycophenolate mofetil at 500mg twice daily exhibits  positive results.




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