Lichen planus

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and Keywords: Ruber planus; Wickham striae

Overview

Lichen planus is an inflammatory disease that affects the skin and the oral mucosa.

Historical Perspective

Classification

Lichen Planopilaris is the specific name given to lichen planus on the scalp that may cause permanent, scarring alopecia. If left untreated the scarring will cause permanent hair loss. The Cicatricial Alopecia Research Foundation is a non-profit organization that provides support and resources for people with lichen planopilaris.

Pathophysiology

Causes

Common Causes

The cause of lichen planus is not known; however, there are cases of lichen planus-type rashes (known as lichenoid reactions) occurring as allergic reactions to medications for high blood pressure, heart disease and arthritis. These lichenoid reactions are referred to as lichenoid mucositis (of the mucosa) or dermatitis (of the skin). Lichen planus has been reported as a complication of chronic hepatitis C virus infection and can be a sign of chronic graft-versus-host disease of the skin. It has been suggested that true lichen planus may respond to stress, where lesions may present on the mucosa or skin during times of stress in those with the disease.

Causes in Alphabetical Order

Differentiating lichen planus from other Diseases

The clinical presentation of lichen planus may also resemble other conditions, including:

A biopsy is useful in identifying histological features that help differentiate lichen planus from these conditions.

Epidemiology and Demographics

Age

Lichen planus in children is rare, and it occurs most often in middle-aged adults.

Gender

Lichen planus affects women more than men (at a ratio of 3:2).

Risk Factors

Some of the risk factors for lichen planus are as follows:

Natural History, Complications, and Prognosis

Lichen planus is usually not harmful and may get better with treatment. It usually clears up within 18 months. However it may last for weeks to months, and may come and go for years. If lichen planus is caused by a medication, the rash should go away once the medicine is stopped.

A possible complication that is associated with lichen planus is that mouth ulcers that are there for a long time may develop into oral cancer.

Diagnosis

Symptoms

The microscopic appearance of lichen planus is pathognomonic for the condition

  • Hyperparakeratosis with thickening of the granular cell layer
  • Development of a "saw-tooth" appearance of the rete pegs
  • Degeneration of the basal cell layer
  • Infiltration of inflammatory cells into the subepithelial layer of connective tissue

Other symptoms such as:

Physical Examination

The typical rash of lichen planus is well-described by the "5 P's": well-defined pruritic, planar, purple, polygonal papules. The commonly affected sites are near the wrist and the ankle. The rash tends to heal with prominent blue-black or brownish discoloration that persists for a long time. Besides the typical lesions, many morphological varieties of the rash may occur. The presence of cutaneous lesions is not constant and may wax and wane over time. Oral lesions tend to last far longer than cutaneous lichen planus lesions.

The following images show good examples of how lichen planus manifests on the body.

Skin

Trunk
Extremity
Oral cavity
Face
Lichen Planus Actinicus
Face
Trunk
Extremity
Lichen planus annular
Trunk
Lichen Planus Follicularis
Neck
Extremity
Scalp
Lichen Planus Linearis
Trunk
Extremity
Lichen Planus Verrucosus
Lichen Planus Vesicular
Trunk
Extremity
Mucosal Lichen Planus
Oral cavity

Differential diagnosis

Lichen planus must be differentiated from other diseases that cause ust be differentiated from other diseases that cause rash and eczema such as secondary syphilis and pityriasis rosea.

Disease Rash Characteristics Signs and Symptoms Associated Conditions Images
Cutaneous T cell lymphoma/Mycosis fungoides[2]
Pityriasis rosea[3]
  • Pink or salmon in color, which may be scaly; referred to as "herald patch"
  • Oval shape
  • Long axis oriented along the cleavage lines
  • Distributed on the trunk and proximal extremities
  • Squamous marginal collarette and a “fir-tree” or “Christmas tree” distribution on posterior trunk
  • Secondary to viral infections
  • Resolves spontaneously after 6-8 weeks
By James Heilman,MD - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16305230
Pityriasis lichenoides chronica
  • Recurrent lesions are usually less evenly scattered than in cases of psoriasis
  • Brownish red or orange-brown in color
  • Lesions are capped by a single detachable, opaque, mica-like scale
  • Often leave hypopigmented macules
Nummular dermatitis[6]
  • Lesions commonly relapse after occasional remission or may persist for long periods
  • Pruritus
Secondary syphilis[7]
  • Round, coppery, red colored lesions on palms and soles
  • Papules with collarette of scales
By James Heilman,MD - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16305230
Bowen’s disease[8]
  • Erythematous, small, scaly plaque, which enlarges erratically over time
  • Scale is usually yellow or white and it is easily detachable without any bleeding
  • Well-defined margins
By Klaus D. Peter, Gummersbach, Germany - Own work (own photograph), CC BY 3.0 de, https://commons.wikimedia.org/w/index.php?curid=6839115
Exanthematous pustulosis[10]
By See below - (2010). "Acute generalized exanthematous pustulosis: an unusual side effect of meropenem". Indian J Dermatol 55 (2): 176–7. DOI:10.4103/0019-5154.62759. PMID 20606889. PMC: 2887524., CC BY 1.0, https://commons.wikimedia.org/w/index.php?curid=52979729
Hypertrophic lichen planus[12]
Di James Heilman, MD - Opera propria, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=11509003
Sneddon–Wilkinson disease[14]
  • Flaccid pustules that are often generalized and have a tendency to involve the flexural areas
  • Annular configuration
Small plaque parapsoriasis[18]
  • Erythematous plaques with fine scaly surface
  • May present with elongated, finger-like patches
  • Symmetrical distribution on the flanks
  • Known as digitate dermatosis
  • Lesions may be asymptomatic
  • May be mildly pruritic
  • May fade or disappear after sun exposure during the summer season, but typically recur during the winter
Intertrigo[20]
Langerhans cell histiocytosis[21]
  • Scaling and crusting of scalp
Tinea manuum/pedum/capitis[25]
  • Scaling, flaking, and sometimes blistering of the affected areas
  • Hair loss with a black dot on scalp in case of tinea capitis
Seborrheic dermatitis
By Roymishali - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=27267929

Head

Oral lichen planus may present in one of three forms.

  • The reticular form is the most common presentation and manifests as white lacy streaks on the mucosa (known as Wickham's striae) or as smaller papules (small raised area). The lesions tend to be bilateral and are asymptomatic. The lacy streaks may also be seen on other parts of the mouth, including the gingiva (gums), the tongue, palate and lips.
  • The bullous form presents as fluid-filled vesicles which project from the surface.
  • The erosive form presents with erythematous (red) areas that are ulcerated and uncomfortable. The erosion of the thin epithelium may occur in multiple areas of the mouth, or in one area, such as the gums, where they resemble desquamative gingivitis. Wickham's striae may also be seen near these ulcerated areas. This form may undergo malignant transformation.

Throat

Rarely, lichen planus shows esophageal involvement, where it can present with erosive esophagitis and stricturing. It has also been hypothesized that it is a precursor to squamous cell carcinoma of the esophagus.

Genitals

Lichen planus may also affect the genital mucosa - vulvovaginal-gingival lichen planus. It can resemble other skin conditions such as atopic dermatitis and psoriasis.

Clinical experience suggests that Lichen planus of the skin alone is easier to treat as compared to one which is associated with oral and genital lesions.

Treatment

Currently there is no cure for lichen planus but there are certain types of medicines used to reduce the effects of the inflammation. Lichen planus may go into a dormant state after treatment. There are also reports that lichen planus can flare up years after it is considered cured.

Medical Therapy

Pharmacotherapy

Acute Pharmacotherapies
Chronic Pharmacotherapies

Surgery

Prevention

References

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