​Kembara Xtra - Medicine - Lichen Planus

​Kembara Xtra - Medicine - Lichen Planus 
Lichen planus (LP) is an idiopathic eruption with distinctive shiny, flat-topped (Latin: planus, "flat"), purple (violaceous) papules and plaques on the skin. It is sometimes accompanied with distinctive mucous membrane lesions.
Itching could be quite bad.
Classic (regular) A relatively rare inflammatory illness of the skin and mucous membranes, LP can also affect the hair and nails.
– Small, flat, angular, reddish-purple, shiny, pruritic papules and/or plaques, also known as Wickham striae or gray-white puncta, are skin lesions that are most frequently found on the mucous membranes, the genitalia, the extensor surfaces of the lower extremities, and the flexor surfaces of the upper extremities. Lesions on the oral mucosa often manifest as elevated white lines arranged in a lace-like pattern, which are most frequently seen on the buccal mucosa.
– Onset might be sudden or gradual. Unpredictable course; may end spontaneously, repeat occasionally, or last for a long time. Drug-related LP
- Histopathologic and clinical features may resemble those of conventional LP. Wickham striae are typically absent in lesions (see the text that follows), and oral involvement is uncommon.
– Lesions typically don't start to manifest for months after the medicine is introduced.
- Lesions disappear when the inciting agent is stopped, frequently after a lengthy time.
Follicular LP variations, also known as lichen planopilaris, are most frequently found on the scalp and can cause alopecia scarring. - Annular: papules appear on the glans, penis, axillae, and oral mucosa and spread centrifugally as the core area heals. - Linear: could be a single finding
- Hypertrophic: thick plaques with hyperkeratotic itch on the dorsal legs and feet - Atrophic: uncommon, typically caused by healed lesions
- Bullous LP: The epidermis blisters as a result of severe dermal irritation.
LP pemphigoides (IgG autoantibodies to collagen 17) is a mix of LP and bullous pemphigoid. Nail LP: affects the nail matrix and causes lateral thinning, longitudinal ridging, and fissuring. Affected systems include the skin and exocrine.

Epidemiology Predominant sex: female > male Predominant age: 30 to 60 years; unusual in infants and the elderly In the United States, the prevalence is 450/100,000.

Pathophysiology and Etiology 

According to some theories, LP is an autoimmune reaction to self-antigens on injured keratinocytes that is T cell-mediated.
RISK ELEMENTS
exposure to specific chemicals or medications
Thiazides, furosemide, beta-blockers, sulfonylureas, antimalarial drugs, penicillamine, gold salts, and inhibitors of the angiotensin-converting enzyme. Rarely: photo-developing chemicals, dentistry materials, and tattoo colors

Accompanying Conditions 
LP and hepatitis C virus infection have been linked, especially in specific geographical areas (Asia, South America, the Middle East, and Europe). Patients with extensive LP presentations and those whose primary symptoms are oral should be tested for hepatitis.
In addition, it has been noticed that LP coexists with lichen nitidus, primary biliary cirrhosis, chronic active hepatitis, and chronic active hepatitis.
Additionally, LP has been observed more frequently than would be predicted by chance in conjunction with other immune-altering disorders.
- Vitiligo - Alopecia areata - Myasthenia gravis - Bullous pemphigoid
- Morphea and lichen sclerosus et atrophicus - Graft-versus-host reaction - Lupus erythematosus (LP overlap syndrome) - Ulcerative colitis

Despite having a variety of clinical symptoms, LP is most frequently identified by its outward manifestations.
Dermoscopy: The most frequent findings were radial capillaries, blue-gray granules, and polymorphic pearly white structures.
If there is any dispute about the diagnosis, a skin biopsy should be done.
HISTORY Only a small percentage of patients have LP in their families. Human leukocyte antigen B7 (HLA-B7) is more prevalent in affected families. A complete drug history should be taken.

clinical assessment 

 Skin (frequently with intense itching) - Papules: lesions in crops that are 1 to 10 mm in diameter, glossy, flat-topped (planar), and may have a fine scale.
– Typically, there are no crusts or excoriations to indicate scratching.
- Violaceous in color, with a white lace-like pattern (Wickham striae) on the papules' surface. If present, Wickham striae are almost pathognomonic for LP and are best observed following topical application of mineral oil.
- Polygonal or oval shapes. On the mucous membranes and trunk, anular lesions might develop. Polymorphic means that there may be different sizes and shapes.
- Organization: individual lesions may be dispersed, linear, or clustered. - Koebner phenomenon (isomorphic response): New lesions may appear at the locations of very small wounds, like burns or scratches.
Ventral surfaces of the wrists and forearms, dorsa hands, glans penis, dorsa feet, groin, sacrum, shins, and scalp are all affected. Lower legs may develop hypertrophic (verrucous) lesions, which can also be widespread.
- Postinflammatory hyperpigmentation: When lesions heal, they usually leave behind darkly pigmented macules.
 Mucous membranes (80% of patients have mucous membrane lesions without skin involvement, compared to 40–60% of patients with skin lesions) Milky-white streaks with an attractive, lacy pattern are most frequently asymptomatic, nonerosive, and observed on the buccal mucosa, however they can also show up on the tongue, gingiva, palate, or lips. - Less frequently, LP may be bullous and erosive.
Painful, particularly if ulcers are present Squamous cell carcinomas can arise from lesions (1–3%).
– Possible affected locations include the labia minora, vaginal vault, and perianal regions.
Hair/scalp - Lichen planopilaris (LP) manifests with keratotic plugs at the follicle orifice with a violaceous rim; this condition may cause hair follicles to atrophy and eventually die (scarring alopecia). ● Nails (10%)
- Nail matrix involvement may result in the creation of pterygium, proximal-to-distal linear grooves, and partial or total loss of the nail bed.

Skin: Lichen simplex chronicus; Eczematous dermatitis; Psoriasis; Discoid lupus erythematosus; Other lichenoid eruptions (those that resemble LP); Other lichenoid eruptions
- Lichen nitidus - Self-inflicted dermatoses - Pityriasis rosea
 Leukoplakia, oral hairy leukoplakia, and oral mucous membranes
- Aphthous ulcers - Candidiasis - Squamous cell cancer (especially in ulcerative lesions)
- Secondary syphilis - Herpetic stomatitis
 Genital mucous membranes - Pemphigus vulgaris, bullous pemphigoid, and Behçet illness (all rare); Psoriasis (penis and labia); Nonspecific balanitis; Zoon balanitis; Fixed drug eruption (penis); Candidiasis (penis and labia); Scarring alopecia (central centrifugal cicatricial alopecia) affects the hair and scalp.

Laboratory Results 
Liver function tests, serology for hepatitis, and, if indicated by history
Skin biopsy and direct immunofluorescence can aid to identify LP from discoid lupus erythematosus in diagnostic procedures/other.
Interpretation of Tests
Basement membrane thinning with "sawtoothing"; dense, band-like (lichenoid) lymphocytic infiltrate of the upper dermis; vacuolar degeneration of the basal layer; hyperkeratosis and irregular acanthosis; increased granular layer; lower epidermis-found degenerative keratinocytes known as colloid or Civatte bodies; melanin pigment in macrophages.


Management 

Although LP can cure on its own, patients who may be experiencing significant symptoms or who are bothered by its aesthetic look typically want therapy.

General Actions 
Asymptomatic oral lesions don't need to be treated; the aim is to get rid of the lesions and stop the itching.
No treatment is curative for oral LP.
- The purpose of treatment is to reduce the risk of oral cancer, heal ulcerative lesions, and relieve discomfort.

The First Line of Medicine

● Skin (3),(4) Topical steroids with ultrahigh and high potencies should be applied twice daily for two to four weeks. Other options include fluocinonide 0.05% or triamcinolone acetonide 0.05% when an obstruction is present. - Intralesional corticosteroids for refractory and hypertrophic lesions, such as triamcinolone [Kenalog] at a concentration of 5 to 10 mg/mL. - "Soak and smear" technique: this method may prevent the need for systemic steroids and result in a rapid relief of symptoms in as little as 1 to 2 days.
By soaking, the stratum corneum can be hydrated and the anti-inflammatory steroid in the ointment can reach deeper layers of the skin. Because water cannot pass through oily materials, ointment smearing holds water in the skin.
The afflicted area is promptly covered with a thin layer of the steroid ointment containing clobetasol or another superpotent topical steroid after soaking for 20 minutes in a bathtub filled with lukewarm plain water.
If needed, soak and smear procedures can last up to 5 days. The treatments are most effective when performed at night since the greasy ointment on the skin gets on pajamas rather than on daily clothing and remains on the skin while you sleep. If necessary, a topical steroid cream is then used during the day.
Mucous membranes - For oral, unpleasant, erosive LP
0.05% clobetasol propionate ointment or topical corticosteroids (0.1% triamcinolone [Kenalog] in Orabase) BID
Corticosteroids intralesional BID

Child Safety Considerations

Due to their bigger skin surface-to-weight ratios, children may absorb a quantity of topical steroid that is proportionately greater.

Second Line Mucous membranes and skin
Oral steroids are only used in cases of abrupt aggravation, severe or extensive disease, or when topical steroids are ineffective.
Prednisone at 30 to 60 mg/day for 3 to 6 weeks, then decrease over the course of 4 to 6 weeks. In some cases of recalcitrant acne, isotretinoin (10 mg), acitretin (30 mg), or alitretinoin (30 mg PO daily) may be added. Pay close attention for any resulting dyslipidemia.
Topical retinoids: tretinoin 0.05% twice a day; isotretinoin 0.1% twice a day; Topical calcipotriol; Metronidazole; Sulfasalazine; Methotrexate; Cyclosporine; Hydroxychloroquine; Dapsone; Thalidomide; Narrow-band UVB (NBUVB) and broad-band UVB (BBUVB): especially when system

Caution 

When pregnant, stay away from oral and topical retinoids.

Further Therapies 

Antihistamines, such as hydroxyzine 25 mg PO every six hours, have little effect on itching but may help with drowsiness before night.

Patient Follow-Up Monitoring
routine checks for erosive and ulcerative lesions in the mouth


Patient education: Oral, ulcerative, or erosive LP: annual follow-up to check for cancer  Maintain proper dental and oral health.
Avoid drinking alcohol, smoking, and using tobacco products, especially if the patient has atrophic or erosive lesions.
Avoid dry, crispy meals like corn chips, pretzels, and toast to prevent mucosal stress.

The condition may spontaneously resolve in a few weeks, but it may also last for years, especially in cases of oral lesions and hypertrophic lesions on the shins.
There is a propensity towards relapse.
 Recurrence occurs in 12–20% of cases, particularly in those with widespread involvement.

Complications include alopecia, nail deterioration, and oral or genital squamous cell carcinoma.

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