History

Fact Explanation
There are Violaceous, itchy papules occurring at the ventral aspect of wrists and legs . On the palms and soles, there are yellow colored, firm papules. This is considered to be an Immune mediated disease. CD8+ T cells recognize antigens on basal keratinocytes and cause lysis of the cells.[1] Note:There are subtypes/variants in this disease(Annular,Atrophic,Bullous,Follicular, Hypertrophic,Ulcerative) and specific features vary according to the varients.[1]
Asymptomatic, white colored, lacy network or papules arise on the buccal mucosa, tongue, gums and lips This is present in 50% of the patients.Also, this may be the only presenting feature.[1] Desquamative gingivitis results in diffuse inflammation and peeling of the gums.[3] As a complication, the ulcerative form in the mouth can give rise to Squamous cell carcinoma.[1]
Nail changes (longitudinal grooves/ destruction of nail and nail bed) The damage is permanent[1] .Nail damage and epithelial growth on nails(causing Pterygium) can be seen[2]. There may be onycholysis present. There may be shedding, arrested growth, or total disappearance of the nail.[3]
Patchy alopecia This is a permanent type of alopecia[1](cicatricial alopecia)[2].This is due to the fibrosis accompanying inflammation.It results in damaging the hair follicle[2].
Painless or painful whitish lesions with inflammation in genitalia(vulval/ vaginal/Penile) In Vulval lichen planus, there are painless, white colored streaks.In the Erosive type, the mucosa of the labia minora and introitus become bright red in color due to inflammation. The labia minora can get adhered to each other or to the labia majora .This type is painful and can interfere with intercourse.[3] In Vaginal lichen planus, it causes a desquamative vaginitis, resulting in a vaginal discharge. There is also contact bleeding present.[3] In Penile lichen planus,Papules are arranged as a ring arround the glans.This is the commonest presentation.[3]
Chronic course with changes in the lesions The lesions lasts for months to years(Eruptions last about a year and hypertrophic variant lasts longer). As the lesions resolve, hyper-pigmented( Brown/ gray colored ) discrete macules are develop[1].
Drug history It tends to cause asymptomatic or itchy purple colored,flat papules on trunk and oral mucosa.The common drugs are: Gold, hydroxychloroquine,Captopril,quinine,thiazide diuretics,[3]
Past history or family history of auto-immune diseases There may be multiple autoimmune diseases present in one patient.Diseases such as Alopecia areata, vitiligo, ulcerative colitis and contact allergy to Mercury( in oral lichen planus) are common[1]
Associated features of Hepatitis C infection Lichen planus is not the result of Hepatitis per-Se but it is observed in patients with Hep-C.This is probably due to immune related molecular mimicry.[3]
Recurrence Recurrence occur due to its autoimmune etiology.This occurs in one in six patients.[1]
References
  1. WELLER, Richard P.J.B. HUNTER, A.A.Jhon. SAVIN John A. DAHL, Mark V. Clinical Dermatology. 4th ed.Oxford:Blackwell publishing Ltd.2008. pg72-75
  2. ROBIN, Graham-Brown. BURNS Tony. Lecture notes:Dermatology.10th ed.West Sussex:WILEY-BLACKWELL .2011.Pg118,120, 134-136
  3. Lichen planus.DermNet NZ[Online].DermNet New Zealand Trust.2013[viewed 6 May 2014].Available form:http://dermnetnz.org/scaly/lichen-planus.html

Examination

Fact Explanation
Multiple, well demarcated violaceous papules with flat surfaces,and polygonal in shape.Common sites are wrists and ankles. When the lesions are examined closely, there is a streaky white colored pattern on the surface called Wickham's striae.
Nail changes (longitudinal grooves/ destruction of nail and nail bed) This is permanent[1] . Nail damage and epithelial growth on nails(causing Pterygium) can be seen[2]. There may be onycholysis present. There may be shedding, arrested growth, or disappearance of the nail.[3]
Patchy alopecia This is a permanent type of alopecia[1](cicatricial alopecia)[2].This is due to the fibrosis accompanying inflammation, that results in damaging the hair follicle[2].
Painless or painful whitish lesions with inflammation in genitalia(vulval/ vaginal/Penile) In Vulval lichen planus, there are painless, white colored streaks present.In the Erosive type, the mucosa of the labia minora and introitus become bright red color due to inflammation. The labia minora can get adhered to each other or to the labia majora .This type is painful and can interfere with intercourse.[3] In Vaginal lichen planus, it causes a desquamative vaginitis, resulting in a vaginal discharge. There is also contact bleeding present.[3] In Penile lichen planus,Papules are arranged as a ring arround the glans.This is the commonest presentation.[3]
signs suggestive of auto-immune diseases There may be multiple autoimmune diseases present in one patient.Diseases such as Alopecia areata, vitiligo(hypeopigmented macules) , ulcerative colitis(Aphthous ulcer,uveitis, Episcleritis, arthritis, Sacroiliitis, Erythema nodosum,Pyoderma gangrenosum,Clubbing) and contact allergy to Mercury( in oral lichen planus)[1]
Associated features of Hepatitis C infection Lichen planus is not the result of Hepatitis per-Se but it is observed in patients with Hep-C.This is probably due to immune related molecular mimicry.[3] Features of complicated Hep-C ex-cirrhosis(ascitis, Jaundice) can be observed.
References
  1. WELLER, Richard P.J.B. HUNTER, A.A.Jhon. SAVIN John A. DAHL, Mark V. Clinical Dermatology. 4th ed.Oxford:Blackwell publishing Ltd.2008. pg72-75
  2. ROBIN, Graham-Brown. BURNS Tony. Lecture notes:Dermatology.10th ed.West Sussex:WILEY-BLACKWELL .2011.Pg118,120, 134-136
  3. Lichen planus.DermNet NZ[Online].DermNet New Zealand Trust.2013[viewed 6 May 2014]. Available form :http://dermnetnz.org/scaly/lichen-planus.html

Differential Diagnoses

Fact Explanation
Psoriasis There are well demarcated, erythematous Plaques with thick scales, where the lesions are most commonly found on extensor surfaces[2]
Pityriasis rosea This condition is common in young patients.A larger herald patch precedes the others.Oval, pink colored lesions with itchiness and fine scaling is present.The characteristic' Fir tree' pattern (along ribs) is present.[1]
Pityriasis lichenoides There are 2 types. Acute type is characterized by papules developing in to necrotic areas and then healing with scaring. The chronic type presents with multiple small scaly papules with a distinctive single gray scale(mica scale) [1]
Discoid lupus erythematosis Plaques are erythematous, scaling, and become atrophied.There is telengectasia and a papualr lesion with a hyperpigmented border and a hypopigmented center.Characteristically the lesions are prominent in sun exposed areas such as face and scalp.[1]
Tinea corporis There is severe itching .Also, a well demarcated area with elevated erythematous border and healed, scaling center can be observed.There may be associated nail deformities and tinea cruris.
Eczema There is prominent excoriations and lichenification of skin, commonly observed on flexor surfaces[1]
Lichen simplex chronicus Single or multiple plaques with lichenification in easily scratched areas[2] can be observed
Thrush This is due to candida infections of the oral mucosa.White colored plaque on oral mucosa that can be easily removed is a characteristic feature.common in patients with immuno-deficiencies or prolonged steroid usage. [2]
Leukoplakia This is a premalignent condition.The White adherent plaque on the mucosa will not rub off.[2]
References
  1. WELLER, Richard P.J.B. HUNTER, A.A.Jhon. SAVIN John A. DAHL, Mark V. Clinical Dermatology. 4th ed.Oxford:Blackwell publishing Ltd.2008. pg72-75
  2. USATINE RP, TINITIGAN M. Diagnosis and treatment of lichen planus. Am Fam Physician [online] 2011 Jul 1, 84(1):53-60 [viewed 09 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21766756

Investigations - for Diagnosis

Fact Explanation
Skin biopsy This is usually a clinical diagnosis[1], but a biopsy will conform the diagnosis. A punch biopsy is taken from the affected area (skin/mouth).Typical “saw-tooth” pattern of epidermal hyperplasia, hyperparakeratosis vacuolar changes in basal layer,and T-Cell infiltration at the dermal-epidermal junction can be identified.[2]
Direct immunofluorescence study In direct immunofluorescence studies immunoglobulins were deposited at the cytoid bodies and fibrin was deposited at dermoepidermal junction. This test may be helpful in diagnosing patients with inadequate clinical and histological evidence. [3]
References
  1. WELLER, Richard P.J.B. HUNTER, A.A.Jhon. SAVIN John A. DAHL, Mark V. Clinical Dermatology. 4th ed.Oxford:Blackwell publishing Ltd.2008. pg72-75
  2. USATINE RP, TINITIGAN M. Diagnosis and treatment of lichen planus. Am Fam Physician [online] 2011 Jul 1, 84(1):53-60 [viewed 09 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21766756
  3. KULTHANAN K, JIAMTON S, VAROTHAI S, PINKAEW S, SUTTHIPINITTHARM P. Direct immunofluorescence study in patients with lichen planus. Int J Dermatol [online] 2007 Dec, 46(12):1237-41 [viewed 09 May 2014] Available from: doi:10.1111/j.1365-4632.2007.03396.x

Management - General Measures

Fact Explanation
Patient education Inform the patient about the nature of the disease, complications and prognosis.LP is a self limited condition and patient should be aware of it.
Skin and genital area management Wash with plain warm water avoid soap/shampoo[1],[4]. Use a plain emollient[4]/ aqueous cream and use a good lubricant in intercourse. Try to wear light undergarments as possible[1],[4]. Visit a dermatologist for further issues. [Note: Management of skin in both genital and other areas is similar in relation to cleaning]
Select suitable food Avoid spices and acidic food[3]
Avoid alcohol, tobaco Avoid taking alcohol, tobaco or spirits because it irritates , reduces healing rate and increases the risk of malignency . Use a mouthwash which doesn't contain alcohol / or use chlorhexidine mouthwash[2]
Improve oral hygiene Use a toothpaste that is mild and non irritating[2]. Regular visits to the dentist[2] helps in maintaining gum health and he can identify any pre-malignent lesions
References
  1. UK National Guideline on the Management of Vulval Conditions.Clinical Effectiveness Group.British Association Sexual Health and HIV.2014.[viewed 7 May 2014] Available form:http://www.bashh.org/BASHH/Guidelines/BASHH/Guidelines/Guidelines.aspx?hkey=faccb209-a32e-46b4-8663-a895d6cc2051
  2. SCHLOSSER BJ. Lichen planus and lichenoid reactions of the oral mucosa. Dermatol Ther [online] 2010 May-Jun, 23(3):251-67 [viewed 09 May 2014] Available from: doi:10.1111/j.1529-8019.2010.01322.x
  3. LE CLEACH L, CHOSIDOW O. Clinical practice. Lichen planus. N Engl J Med [online] 2012 Feb 23, 366(8):723-32 [viewed 09 May 2014] Available from: doi:10.1056/NEJMcp1103641
  4. Erosive Lichen planus.DermNet NZ.Derm [online]Net New Zealand Trust.2013[viewed 9 May 2014].Available form:http://dermnetnz.org/site-age-specific/erosive-lichen-planus.html

Management - Specific Treatments

Fact Explanation
Topical steroids These agents relive the inflammation and reduce the size of the plaques.[1] Topical high-potency corticosteroids and intralesional corticosteroids are commonly used to minimize scaring alopecia.[2]
Systemic steroids This method is recommended only for severe conditions such as nail lesions or erosive oral lesions.[1]
Photochemotherapy This reduces pruritus and improve healing.[1]
Oral cyclosporin/ or Acitretin This is considered in patients with severe or resistant types.[1],[2]
Antihistamines These drugs reduce the itch [1],[2]and improves healing and well being
intralesional triamcinolone acetonide Hypertrophic lesions are treated with intralesional injections[2]
For genital lichen planus Triamcinolone ointment is firstline treatment. Topical tacrolimus and clobetasol are used in vulvovaginal (erosive lichen planus) LP.Topical lidocaine relives pain.[2]
High-potency topical steroids This is a very effective treatment and it is used as first line treatment for oral LP.This is also used in mucosal erosive lichen planus.[2]
Systemic corticosteroids(oral prednisone) This is only used in severe oral lichen planus and LP with other mucocutaneous sites.[2]
Topical calcineurin inhibitors, such as tacrolimus/ pimecrolimus These are second-line therapy for oral lesions.[2]
Carbon-dioxide laser evaporation If the condition is not responding to topical corticosteroids, this method can induce a long-term remission of symptoms. It can also be considered as first-line measure in patients with painful oral lesions.[2]
Tetracycline This was found to be useful in the treatment of gingival lesions.[3],[5]
Metronidazole Oral lesions show a good response to this drug.[4],[5]
References
  1. WELLER, Richard P.J.B. HUNTER, A.A.Jhon. SAVIN John A. DAHL, Mark V. Clinical Dermatology. 4th ed.Oxford:Blackwell publishing Ltd.2008. pg72-75
  2. USATINE RP, TINITIGAN M. Diagnosis and treatment of lichen planus. Am Fam Physician [online] 2011 Jul 1, 84(1):53-60 [viewed 09 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21766756
  3. LODI G, SCULLY C, CARROZZO M, GRIFFITHS M, SUGERMAN PB, THONGPRASOM K. Current controversies in oral lichen planus: report of an international consensus meeting. Part 2. Clinical management and malignant transformation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod [online] 2005 Aug, 100(2):164-78 [viewed 09 May 2014] Available from: doi:10.1016/j.tripleo.2004.06.076
  4. RASI A, BEHZADI AH, DAVOUDI S, RAFIZADEH P, HONARBAKHSH Y, MEHRAN M, PIRAN P, DEHGHAN N. Efficacy of oral metronidazole in treatment of cutaneous and mucosal lichen planus. J Drugs Dermatol [online] 2010 Oct, 9(10):1186-90 [viewed 09 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20941941
  5. ASCH S, GOLDENBERG G. Systemic treatment of cutaneous lichen planus: an update. Cutis [online] 2011 Mar, 87(3):129-34 [viewed 09 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21488570