Lichen planus

Definition :Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. 

FEATURES :It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale (Wickham's striae ) dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa.

Lichen planus lesions are so called because of their "lichen-like" appearance

Classification :

Lichen planus may be categorized as affecting mucosal or cutaneous surfaces.

• Cutaneous forms are those affecting the skin, scalp, and nails.
• Mucosal forms are those affecting the lining of the gastrointestinal tract (mouth, pharynx, esophagus, stomach, anus), larynx, and other mucosal surfaces including the genitals, peritoneum, ears, nose, bladder and conjunctiva of the eyes. 

• Types:

• Papular Papular form is the classic cutaneous lichen planus (CLP) lesion characterized by shiny, red or purple-colored, flat-topped papule. Lesions may have a thin, transparent, and adherent scale. Fine whitish points or lacy lines (Wickham's striae) may be seen on the surface of well-developed papules.[1]
• Annular 'Ring-shaped' lesions that develop gradually from single small pigmented spots into circular groups of papules with clear, unaffected skin in the center. The ring-like lesions may very slowly enlarge, co-join and morph into larger irregular (serpentine) bands, sometimes accompanied by lines (See Linear, below). Annular CLP is uncommon and classically involves the male genitalia (glans penis and penile shaft), groin, axilla and also the extremities.[1]
• Linear Papules are arranged in a line (the "Blaschko line").[15] This pattern may develop secondary to trauma (koebnerization) or, uncommonly, as a spontaneous, isolated eruption, usually on the extremities, and rarely on the face.[16]
• Hypertrophic This pattern is characterized by hyperkeratotic thick pruritic red-brown to purple-gray plaques with follicular accentuation. Hypertrophic CLP commonly involves the extremities, especially the interphalangeal joints and the anterior legs in a symmetrical distribution.[1] This form is also known as "lichen planus verrucosus".
• Atrophic This morphology is characterized by the presence of a few well-demarcated, white-bluish papules or plaques with central superficial atrophy. Atrophic CLP is the clinical endpoint of chronic annular or hypertrophic LP with atrophic lesions. The use of potent topical corticosteroids for a long-term may predispose the patient to developing atrophic lesions.[1]
• Bullous This morphology is characterized by the development of vesicles and bullae with the skin lesions. This is a rare variant of lichen planus, and also known as "vesiculobullous lichen planus."
• Actinic Rare form presenting as nummular patches or plaques with a hypopigmented halo surrounding a hyperpigmented center. Actinic CLP is more prevalent in African Americans, Indians, and Middle-Eastern individuals and commonly affects the sun-exposed areas.[1]
• Ulcerative This morphology is characterized by chronic, painful bullae and ulceration of the feet, often with cicatricial sequelae evident. This is a rare variant of lichen planus.
• Pigmented This morphology is characterized by hyperpigmented, dark-brown macules in sun-exposed areas and flexural folds. This is a rare variant of lichen planus.
• Follicular Characterized by follicular, flat, elevated or hemispherical erythematous papules with or without keratoses presenting in groups or disseminated. The Graham‐Little‐Piccardi‐Lasseur syndrome, seen in a familial pattern and also predominantly in women, is characterized by the appearance of follicular LP on the trunk with LP follicularis decalvans on the scalp. Follicular LP on the scalp is more likely to lead to scarring alopecia.[14]
• Inverse Characterized by extensive erythematous lesions with poorly defined borders and in part with lichenification. Inverse LP typically affects the axillae, inguinal creases, limb flexures and submammary region. Pigmentation of the individual lesions at these inverse locations are typical. Additionally, keratotic papules and erosions with a bizarre configuration can occur

• Skin

• Variants of cutaneous lichen planus are distinguished based upon the appearance of the lesions and/or their distribution.^[20]Lesions can affect the:

  • Extremities (face, dorsal hands, arms, and nape of neck).^[a] This is more common in Middle Eastern countries in spring and summer, where sunlight appears to have a precipitating effect.^[16][21][22]
  • Palms and solesfirst-line treatment typically involves either topical or systemic corticosteroids,^[7]and removal of any triggers.^[65] Without treatment, most lesions will spontaneously resolve within 6–9 months for cutaneous lesions,^[7] and longer for mucosal lesions.
  • Intertriginous areas of the skin. This is also known as "inverse lichen planus."^[16]
  • Nails^[23] characterized by irregular longitudinal grooving and ridging of the nail plate, thinning of the nail plate, pterygium formation, shedding of the nail plate with atrophy of the nail bed, subungual keratosis, longitudinal erthronychia (red streaks), and subungual hyperpigmentation.^[24] A sand-papered appearance is present in around 10% of individuals with nail lichen planus.^[23]
  • Hair and Scalp. The scalp is rarely affected by a condition known as lichen planopilaris, acuminatus, follicular lichen planus, and peripilaris, characterised by violaceous, adherent follicular scale with progressive scarring alopecia. While lichen planus and lichen planopilaris may occur together, aside from sharing the term ‘lichen’ and revealing inflammation on skin biopsy, there is neither established data on their co-occurrence nor data to suggest a common etiology. Lichen planopilaris is considered an orphan disease with no definitive prevalence data and no proven effective treatments.^[25][26]

  • Mucous membranes

  • Lichen planus affecting mucosal surfaces may have one lesion or be multifocal.^[32]Examples of lichen planus affecting mucosal surfaces include:^[32]

    • Esophageal lichen planus, affecting the esophageal mucosa. This can present with difficulty or pain when swallowing due to oesophageal inflammation, or as the development of an esophageal stricture. It has also been hypothesized that it is a precursor to squamous cell carcinoma of the esophagus.^[12][33]
    • Genital lichen planus, which may cause lesions on the glans penis or skin of the scrotum in males, and the vulva or vagina in females.^[8] Symptoms may include lower urinary tract symptoms associated with stenosis of the urethra, painful sexual intercourse, and itching.^[8] In females, Vulvovaginal-gingival syndrome, is severe and distinct variant affecting the vulva, vagina, and gums, with complications including scarring, vaginal stricture formation,^[34] or vulva destruction.^[35] The corresponding syndrome in males, affecting the glans penis and gums, is the peno-gingival syndrome.^[16] It is DQB1.

    Differential diagnosis 

    • Skin
    • Lichenoid drug eruption
    • The cutaneous manifestations resemble idiopathic lichen planus.
    • Chronic graft-versus-host disease
    • The history of preceding hematopoietic cell transplant is helpful for diagnosis
    • Psoriasis
    • Atopic dermatitisc
    • Cutaneous lupus erythematos
    • Discoid lupus erythematosus 

    • Treatment 

    first-line treatment typically involves either topical or systemic corticosteroids,[7]and removal of any triggers.[65] Without treatment, most lesions will spontaneously resolve within 6–9 months for cutaneous lesions,[7] and longer for mucosal lesions

    Abstract

    A 34-year-old female patient with the chief complaint of burning sensation in the oral cavity associated with generalized pruritis, scalp and skin lesions diagnosed as Bullous lichen planus and treated with systemic prednisolone, levamisole, benzydamine oral rinse. Patient is in follow up since 1 year and free of lesions. Here we report the case and review current modalities in the management of oral lichen planus.

    Keywords: Bullous lichen planus, pruritis, burning sensation

    Case Report

    A 34-year-old Indian female patient reported to the Department of Oral Medicine and Radiology with the chief complaint of burning sensation of oral cavity since 4 days. Burning sensation was insidious in onset, preceded by eruption of fluid filled vesicles that used to burst within few seconds, continuous, severe intensity aggravated on having spicy food substances and no relieving factors, episodes of such occurrence of blisters 10-15 times per day. She was also associated with generalized body itching and mild fever. History of recurrent episodes of burning sensation since 8 years but this time associated with severity. Her medical history revealed occurrence of dermal and scalp lesions, loss of hair, generalized pruritis for which she was taking treatment by a dermatologist since 10 years and presently was on the following medications: pimecrolimus (pacroma cream 0.01%), halobresol propionate cream (halox cream 0.05%), and ketoconazole shampoo (Keto Az shampoo). History of anal hemorrhoids treated 4 years back. Her past dental history revealed that extraction of mobile teeth and gingival flap surgery was done 8 years back.

    No significant family history. She was married since 13 years blessed with two children (girls) and stays in a joint family, often her life was associated with stress and tension as reported by the patient. Vegetarian by diet. No history of any deleterious habits. She brushed once daily in horizontal manner using brush and paste. General physical examination revealed that she is moderately built and nourished, appeared depressed and sad. Depression Anxiety and Stress Scale [DASS] score indicated moderate depression, mild anxiety, and moderate stress. Scalp had isolated central area of hair loss, solitary ulcerative lesion measuring about 3 ×4 cm, margins were irregular, floor covered with yellow slough, tender on palpation. Nails present with mild longitudinal ridging. Black healed pigmented lesions of varying sizes were evident on legs, hands, and back 

    

    On intra-oral examination solitary mixed red and white lesions, irregular in shape, measuring about 2 ×3 cm i.r.t right posterior buccal mucosa extending anteriorly from mesial of 45 posteriorly distal to 48, superiorly from occlusal plane of 14 inferiorly up to buccal vestibule i.r.t 45 46 47 48 regions, surface presented with bullae filled with clear fluid measuring about 1 ×0.8 cm, margins are irregular with pseudo membrane slough in the posterior buccal mucosal region, surrounding buccal mucosa was normal 

    

    
    

    Hard palate showed solitary white lesion measuring about 1.5 ×1 cm irregular in shape, extending anteriorly from mid-palatal raphe posteriorly to line joining distal surface of 13 23, white papules, interspersed with striae, surrounding mucosa was normal.
    

    Gingiva was observed with generalized gingival inflammation, erythematous, bleeding on probing, tender, lower right side of posterior gingiva had typical white striae. Generalized pockets were present. Hard tissue examination reveals teeth 15 14 13 12 11 21 22 23 3 24 25 27 48 47 46 45 44 43 42 41 31 32 33 34 35 38. 38 – Grade III mobility. Considering history and clinical features Bullous lichen planus (LP) was considered as provisional diagnosis with the differential diagnosis phemphigus vulgaris, Bullous phemphigoid, mucus membrane phemphigoid, Lichenoid contact stomatitis.
    

    She was treated with Tab levamisole (Vermisol) 150 mg once daily for 3 days, Tab prednisolone (Wysolone) 10 mg 4 times a day for 10 days, benzydamine oral rinse 0.15% (Tantum oral rinse), ranitidine 150 mg (Histac EVT) once daily for 10 days. On second visit after 10 days, patient reported with 50% reduction in burning sensation and also episodes of occurrence of bullae was reduced from 10–15 times/day to 7–8 times/day. The dose of wysolone was tapered. On third visit after 15 days patient reported with complete reduction of burning sensation and was enjoying the taste of food. Episodes of bullae occurrence was 4 times in 15 days. On fourth visit after 15 days lesions on right and left buccal mucosa had healed for remaining white lesions, triamcinolone (caziq) was prescribed for topical application. On fifth visit her lesions on right and left buccal mucosa had healed, her scalp lesions too were healed and erythematous gingiva was much reduced. Patient was referred to Department of Periodontics for oral hygiene prophylaxis. Patient is still under follow up since a year and free of lesions.