LICHEN PLANOPILARIS

Lichen planopilaris (follicular lichen planus) affecting the scalp
Short Summary

A 50 years old female complained about appearance of detached pruritic symptomatology affecting the trunk and the scalp. The patient carried out numerous dermatology specialist examinations on the following 3 years. Her allergy tests results were negative and she diagnosed with a seborrhoeic dermatitis affecting the scalp. As the patient did not achieve any result with the prescribed therapies ,she carried out biopsy. The biopsy was consistent with a “Lichen Planopilaris.”

Patient's Questions

1) Do you confirm the diagnosis?
2) What therapy do you suggest?
3) Do you think it will be possible to resolve completely or partially the current scalp lesions that can be seen in the enclosed photos?
4) Could you advise any healthcare centre of excellence with regard to the pathology the patient suffers from?
 

Medical Background

50 years old, female.

Past illness: pre-eclamptic toxaemia.

Chronic diseases: arterial hypertension.

In July 2006, approximately, appeared detached pruritic rush affecting the trunk and the scalp. The patient carried out dermatology specialist examination with indications to carry out blood tests with negative allergy tests results. At the follow-up visit, carried out by the same doctor, the following therapy was prescribed: Zirtec, Flubason, Seba Med oil for bath and shower, Atoderm emulsion. On the further visit with the same dermatologist it was reported that the patient showed an incoercible itching involving the scalp (the apex) with current evidence of superficial follicular pustules picture. A further pharmacological therapy with Bassado associated with lactic cultures and Kestine, besides the use of Boric water at 3%, was advised.

As the patient did not achieve any result from this treatment, she visited another specialist who diagnosed her with a seborrhoeic dermatitis affecting the scalp and prescribed further therapy with Triatop shampoo, Mellis bioshampoo, Localyn glicole and Zirtec tablets. A further examination, 2 weeks later, was carried out by the same dermatologist, who prescribed: Restiv-oil and Bio-thymus lotion. A month later, the same specialist (always confirming the seborrhoeic dermatitis), changed the therapy to: Liperol shampoo, Bio-Thymus lotion and Elocon lotion. Three months later, the same dermatologist changed the therapy for the seborrhoeic dermatitis again in the following way: Anacaps 1 tablet daily for 1 month; Liperol Shampoo once a week; Psorin Scalp Fluid Shampoo once a week; Kelnol Zine Lotion once daily. At last, month later, the dermatologist, carried out the last visit with prescription of a new therapeutic scheme: Anacaps 1 tablet daily for 1 month; Psorin scalp fluid once weekly administration; Deltacrin solution for midweek administrations; Biothymus intensive vials to use by means of rubbings.

As the patient did not achieve any result, she went to another medical centre, whose medical report gave evidence of a hair loss with small sclero-atrophic patches, and adviced her to carry out biopsy and hepatitis markers tests. Moreover, a therapy with shampoo and lotions and immunostimulants, was prescribed. The hepatitis markers were negative, while the biopsy was consistent with a “Lichen Planopilaris”.

The patient went back to the previous dermatologist with the diagnosis detected by biopsy and she underwent therapy with: Liperol shampoo twice a week, Losalen lotion once daily application on the scalp for 3 weeks, Bio-thymus pro Active lotion twice a week, Immugen-R 1 tablet daily for 1 month. On clinical follow-up visit, which confirmed a persisting Follicular Lichen and, therefore, further specific therapy with: Deflan 12 mg in the morning for 10 days – then 6 mg a day for 5 days, Ranidil 75 mg in the evening, Liperol Shampoo twice a week, Olux foam one application a day for 15 days. Further follow-up,two weeks later, with diagnosis of Follicular Lichen involving the scalp with patches of pseudopelade of Broq: the patient was advised to continue with Deflan 6 mg  daily for 7 days – then suspension of Deflan and intake of Sursum 400-1 tablet a day for 1 month; Ranidil 75 mg in the evening;  Liperol Shampoo, Localyn glicole once daily application on the scalp.

On examination to another specialist who confirmed the therapy with Deflan: 1 tablet daily for 10 days, then ½ tablet daily for 15 days, then ¼ tablet daily for 15 days, then every other day for a further 15 days. Specialist visit was carried out with diagnosis of Cicatricial Alopecia with indication to suspend the current therapy and to repeat the cutaneous biopsy. The biopsy confirmed the diagnosis of “Cicatricial Alopecia on inflammatory basis, consistent with Lichen Planus Pilaris”. At this point a new cortisone therapy was started: Medrol 16 mg, 1 tablet on Monday, Tuesday, Thursday and Friday.

At the follow-up visit two months later, the specialist advised to continue the therapy with Medrol ; Elocon lotion in the evening ; Methotrexate 5 mg twice a day on Monday , 5mg once a day on Tuesday; Levofolene 7.5 mg on Wednesday at 8 pm and 7.5 mg on Thursday at 8 am. The specialist also recomended to review  transaminases, GGT, creatinine, azotemia, for possible therapy of combination with MTX. The blood tests were within normal limits.

The patient continued by following the therapeutic scheme for a few months, monitoring the functional parameters of liver and kidneys; later the Diprosalic was added.

In February 2009 appeared non-infiltrated erythema on the face (cheekbone area and forehead) causing strong itching ,described as teleangectasia and signs of folliculitis. Month later, she carried out an immunology visit with prescription of further blood tests. These medical tests have not highlighted notable abnormalities, in particular we report that the immunology examinations were negative - anti-nuclear antibodies, anti-native DNA antibody, extractable nuclear antigens (ENA), antiphospholipid antibodies (LA, B2GPI, aCI), fractions of the complement C3 and C4. The dermatologic assessment and the biopsy are consistent with a picture of facial rosacea to be treated by a dermatologist taking into consideration the previous dermatologic problems (lichen planus) that have caused a severe alopecia involving the scalp with relevant psychological problems.

At the last dermatologic follow-up visit  the clinical picture related to the Lichen Planofilaris is stable with blood tests within normal limits.

Expert's Opinion

This is a second opinion based on the attached clinical information. I have not performed a clinical examination of the patient. This second opinion has been requested on behalf of the patient by Medical-Opinion.

A 50 year old woman with a diagnosis of lichen planopilaris (LPP) is presented. Her present medical illness spans over 3 years, beginning with symptoms of scalp pruritus and culminating with cicatricial patches of alopecia on her scalp. During this period four working diagnoses have gradually been assigned: scalp pruritus (though not specifically written), seborrheic dermatitis, LPP (or follicular lichen planus) and rosacea.

The patient was treated with a variety of topical and oral medications. These initially included topical steroidal, antiseptic and bacteriostatic, anti-fungal, keratolytic and several zinc/amino acid/vitamin/plant extracts formulations. Anti-histamines were initiated orally and eventually oral steroid tablets and methotrexate were prescribed.

The patient underwent skin biopsy more than a year after the inception of her symptoms. At that time period there was " evidence of a hair loss with small sclero-atrophic patches". The histology report (specific details are not provided) was consistent with LPP. Hepatitis-associated lichen planus was ruled out.

A second skin biopsy was taken confirming the diagnosis. The histology report (again, explicit details are not provided) depicted "Cicatricial Alopecia on inflammatory basis, consistent with Lichen Planus Pilaris". By then, diffuse and prominent patches of cicatricial alopecia appeared.

After carefully reviewing the patient medical records (though lacking some histologic and precise anamnestic and chronological details) and prudently viewing the enclosed clinical pictures, I concur with the diagnosis of LPP leading to pseudopelade of Brocq. The rosacea which followed later was confirmed by biopsy and was most likely induced by the treatment (steroids). The main differential diagnosis of LPP - cutaneous lupus erythematosus/discoid lupus erythematosus (DLE), was ruled out based on histological and laboratory tests.

The patient's pictures portray irregular patches of alopecia, some of which are atrophic , scarred, porcelain-colored with evidence of follicular involvement consisting of mild perifollicular hyperkeratosis and scales.

Lichen planopilaris is an inflammatory, cicatricial alopecia with several patterns of hair loss. It affects women more often than men and may run an insidious or fulminant course. Patients with indolent scalp disease often report at first scalp pruritus and tenderness. Typically, overtime several scattered foci of partial hair loss emerge with associated perifollicular erythema, follicular spines and scarring. LPP is the most common cause of pseudopelade of Brocq - a rare clinical syndrome of scarring alopecia and fibrosis, in which distinct pathologic features are absent and that is the end stage of follicular fibrosis.

It is my experience that once hair loss has appeared and skin biopsy is confirmatory for LPP, aggressive oral steroid therapy should be initiated. The disease can be a particularly difficult condition to treat and alternative treatment regimens include intra-lesional steroids, oral anti-malarial drugs, systemic retinoids, and low-dose weekly methotrexate. These latter treatments should be used with caution, as they may themselves cause some degree of alopecia.

As mentioned above, the disease can be very resistant to treatment, but in my opinion the given treatment was too little too late. I suspect that having reached end-stage fibrosis no treatment would be beneficial, though if some scalp areas exhibit active inflammation, I would try intra-lesional steroids.

I would be happy to see the patient in my practice if she wishes to travel to Israel. I would also recommend professor Mark R. Pittelkow from the department of dermatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. If I can be of any further help to you, please contact me, through Medical-Opinion.

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