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Oral lichen planus (also termed "oral mucosal lichen planus"), is a form of mucosal lichen planus, where lichen planus involves the oral mucosa, the lining of the mouth. This may occur in combination with other variants of lichen planus. Six clinical forms of oral lichen planus are recognized:
- "Reticular", the most common presentation of oral lichen planus, is characterised by the net-like or spider web-like appearance of lacy white lines, oral variants of Wickham's straiae. This is usually asymptomatic.
- "Erosive/ulcerative", the second most common form of oral lichen planus, is characterised by oral ulcers presenting with persistent, irregular areas of redness, ulcerations and erosions covered with a yellow slough. This can occur in one or more areas of the mouth. In 25% of people with erosive oral lichen planus, the gums are involved, described as desquamative gingivitis (a condition not unique to lichen planus). This may be the initial or only sign of the condition.
- "Papular," with white papules.
- "Plaque-like" appearing as a white patch which may resemble leukoplakia.
- "Atrophic," appearing as areas. Atrophic oral lichen planus may also manifest as desquamative gingivitis.
- "Bullous," appearing as fluid-filled vesicles which project from the surface.
These types often coexist in the same individual. Oral lichen planus tends to present bilaterally as mostly white lesions on the inner cheek, although any mucosal site in the mouth may be involved. Other sites, in decreasing order of frequency, may include the tongue, lips, gingivae, floor of the mouth, and very rarely, the palate.
Generally, oral lichen planus tends not to cause any discomfort or pain, although some people may experience soreness when eating or drinking acidic or spicy foodstuffs or beverages. When symptoms arise, they are most commonly associated with the atrophic and ulcerative subtypes. These symptoms can include a burning sensation to severe pain. Lichen planus, particularly when concomitant oral or genital lesions occur, significantly affects patients’ quality of life.
Lichen planus affecting mucosal surfaces may have one lesion or be multifocal. Examples of lichen planus affecting mucosal surfaces include:
- "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.
- "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. Symptoms may include lower urinary tract symptoms associated with stenosis of the urethra, painful sexual intercourse, and itching. In females, "Vulvovaginal-gingival syndrome," is severe and distinct variant affecting the vulva, vagina, and gums, with complications including scarring, vaginal stricture formation, or vulva destruction. The corresponding syndrome in males, affecting the glans penis and gums, is the "peno-gingival syndrome". It is associated with HLA-DQB1.
The histology of lichen nitidus is significant for a "...localized granulomatous lymphohistiocytic infiltrate in an expanded dermal papilla with thinning of overlying epidermis and downward extension of the ridges at the lateral margin of the infiltrate, producing a typical 'claw clutching a ball' picture..."
Linear arrangements of these papules is common (referred to as a Koebner phenomenon), especially on the forearms, but may occasionally be grouped, though not confluent, on flexural areas. Generally, the initial lesions are localized, and remain so, to the chest, abdomen, glans penis, and flexor aspects of the upper extremities; however, less commonly, the disease process can (1) be strictly isolated to the palms and soles, presenting with many hyperkeratotic, yellow papules that may coalesce into plaques that fissure or “...sometimes a non-specific keratoderma resembling chronic eczema,” or (2) become more widespread, with papules widely distributed on the body—the extensor surfaces of the elbows, wrists, and hands, folds of the neck, submammary region in females, groin, thighs, ankles, and feet—and fusing into erythematous, minimally scaled plaques, with reddness that develops tints of violet, brown, and yellow.
LS can occur without symptoms. White patches on the LS body area, itching, pain, pain during sex (in genital LS), easier bruising, cracking, tearing and peeling, and hyperkeratosis are common symptoms in both men and women. In women, the condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening.
In males, the disease may take the form of whitish patches on the foreskin and its narrowing (preputial stenosis), forming an "indurated ring", which can make retraction more difficult or impossible. In addition there can be lesions, white patches or reddening on the glans. In contrast to women, anal involvement is less frequent. Meatal stenosis, making it more difficult or even impossible to urinate, may also occur.
On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.
Morpheaform sarcoidosis is a very rare cutaneous condition characterized by specific cutaneous skin lesions of sarcoidosis accompanied by substantial fibrosis, simulating morphea.
Erythrodermic sarcoidosis is a cutaneous condition and very rare form of sarcoidosis.
Hypopigmented sarcoidosis is a cutaneous condition characterized by areas of hypopigmented skin. It is usually diagnosed in darkly pigmented races and may be the earliest sign of sarcoidosis.
Papular sarcoid is a cutaneous condition characterized by papules, which are the most common morphology of cutaneous sarcoidosis.
Ichthyosiform sarcoidosis is a cutaneous condition resembling ichthyosis vulgaris or acquired ichthyosis, with fine scaling usually on the distal extremities, by caused by sarcoidosis.
Urticarial erythema multiforme is an unusual reaction virtually always associated with antibiotic ingestions, characterized by skin lesions that consist of urticarial papules and plaques, some of which clear centrally forming annular lesions, but with no true urticarial lesions.
Ulcerative sarcoidosis is a cutaneous condition affecting roughly 5% of people with sarcoidosis.
Annular sarcoidosis is a cutaneous condition characterized by papular skin lesions arranged in annular
patterns, usually with a red-brown hue.
A papulosquamous disorder is a condition which presents with both papules and scales, or both scaly papules and plaques.
Examples include psoriasis, lichen planus, and pityriasis rosea.
Porokeratosis may be divided into the following clinical types:
- "Plaque-type porokeratosis" (also known as "Classic porokeratosis" and "Porokeratosis of Mibelli") is characterized by skin lesions that start as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques. Sometimes they may show gross overgrowth and even horn-like structures may develop. Skin malignancy, although rare, is reported from all types of porokeratosis. Squamous cell carcinomata have been reported to develop in Mibelli's type porokeratosis over partianal areas involving anal mucosa. This was the first report mentioning mucosal malignancy in any form of porokeratosis.
- "Disseminated superficial porokeratosis" is a more generalized processes and involves mainly the extremities in a bilateral, symmetric fashion. In about 50% of cases, skin lesions only develop in sun-exposed areas, and this is referred to as "disseminated superficial actinic porokeratosis"
- "Porokeratosis palmaris et plantaris disseminata" is characterized by skin lesions that are superficial, small, relatively uniform, and demarcated by a distinct peripheral ridge of no more than 1mm in height.
- "Linear porokeratosis" is characterized clinically skin lesions are identical to those of classic porokeratosis, including lichenoid papules, annular lesions, hyperkeratotic plaques with central atrophy, and the characteristic peripheral ridge.
- "Punctate porokeratosis" is a skin condition associated with either classic porokeratosis or linear porokeratosis types of porokeratosis, and is characterized by multiple, minute, and discrete punctate, hyperkeratotic, seed-like skin lesions surrounded by a thin, raised margin on the palms and soles.
- "Porokeratosis plantaris discreta" is a skin condition that occurs in adults, with a 4:1 female preponderance, characterized by a sharply marginated, rubbery, wide-based papules. It is also known as "Steinberg's lesion". It was characterized in 1970.
Lichen striatus is defined by:
The papules could be smooth, flat topped or scaly. The band of lichen striatus varies from a few millimeters to 1-- 2 cm wide and extends from a few centimeters to the complete length of the extremity. By and big, the papules are unilateral and single on an extremity along the lines of Blaschko.
There is a diffuse, gray-white, milky opalescent appearance of the mucosa which usually occurs bilaterally on the buccal mucosa. Less often, the labial mucosa, the palate or the floor of mouth may be affected. The surface of the area is folded, creating a wrinkled, white streaked lesion. Apart from the appearance, the lesion is entirely asymptomatic.
Lichen aureus (also known as "lichen purpuricus") is a skin condition characterized by the sudden appearance of one or several golden or rust-colored, closely packed macules or lichenoid papules.
Distress due to the discomfort and pain of Lichen Sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help.
According to the National Vulvodynia Association, which also supports women with Lichen Sclerosus, vulvo-vaginal conditions can cause feelings of isolation, hopelessness, low self-image, and much more. Some women are unable to continue working or have sexual relations and may be limited in other physical activities. Depression, anxiety, and even anger are all normal responses to the ongoing pain LS patients suffer from.
Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the stratum corneum with a thin or absent granular layer.
Papular mucinosis is chronic and may be progressive. The dermal layer of the skin breaks out into small and solid bumps, usually conical in shape and measured from 2 to 4 mm or sometimes flat-topped papules. Unlike pustules, these bumps do not contain pus. Instead they contain mucin, a substance of mucus, the body's natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticarial, nodular, or sometimes annular lesions may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella, results in longitudinal folding and gives the appearance of a leonine facies.
In scleromyxedema, symptoms can occur on larger part of the body. Erythema and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy, inflammatory polyarthritis, central nervous system symptoms, esophageal aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.
Desquamative gingivitis involves lesions of the free and attached gingiva. Unlike plaque-induced inflammation of the gums (normal marginal gingivitis), desquamative gingivitis extends beyond the marginal gingiva, involving the full width of the gingiva and sometimes the alveolar mucosa. The term "full width gingivitis" usually refers to the oral lesions of orofacial granulomatosis however. The color is another dissimilarity between typical marginal gingivitis and desquamative gingivitis, in the latter it is dusky red. Plasma cell gingivitis is another form of gingivitis which affects both the attached and free gingiva.
Erythroderma is generalized exfoliative dermatitis, which involves 90% or more of the patient's skin. The most common cause of erythroderma is exacerbation of an underlying skin disease, such as psoriasis, contact dermatitis, seborrheic dermatitis, lichen planus, pityriasis rubra pilaris or a drug reaction. Primary erythroderma is less frequent and is usually seen in cases of cutaneous T-cell lymphoma, in particular in Sézary's disease.
The most common causes of exfoliative dermatitis are best remembered by the mnemonic device ID-SCALP. The causes and their frequencies are as follows:
- Idiopathic - 30%
- Drug allergy - 28%
- Lymphoma and leukemia - 14%
- Atopic dermatitis - 10%
- Psoriasis - 8%
- Contact dermatitis - 3%
- Seborrheic dermatitis - 2%
Differential diagnosis in patients with erythroderma may be difficult.
Localized granuloma annulare has a tendency towards spontaneous resolution. Localized lesions have been treated with potent topical corticosteroids.
There are several types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, Intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus.
- Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack "Desmoglein 3". Sores often originate in the mouth, making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management.
- Pemphigus foliaceus (PF) is the least severe of the three varieties. "Desmoglein 1", the protein that is destroyed by the autoantibody, is found in only the top dry layer of the skin. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema
- Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.
- The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer, usually lymphoma and Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population. Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.
- Endemic pemphigus foliaceus, including the Fogo Selvagem, the new variant of endemic pemphigus folaiceus in El Bagre, Colombia, South America, and the Tunisian endemic pemphigus in North Africa.
Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.
Verrucous lupus erythematosus (also known as "Hypertrophic lupus erythematosus") presents with non-pruritic papulonodular lesions on the arms and hands, resembling keratoacanthoma or hypertropic lichen planus.
Erythroderma (also known as "Exfoliative dermatitis," "Dermatitis exfoliativa") is an inflammatory skin disease with erythema and scaling that affects nearly the entire cutaneous surface.
In ICD-10, a distinction is made between "exfoliative dermatitis" at L26, and "erythroderma" at L53.9.
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Desquamative gingivitis is a descriptive clinical term, not a diagnosis. Dermatologic conditions cause about 75% of cases of desquamative gingivitis, and over 95% of the dermatologic cases are accounted for by either oral lichen planus or cicatricial pemphigoid. The exact cause of desquamative gingivitis cannot be determined about a third of cases.
- Oral lichen planus
- Cicatricial pemphigoid or less commonly bullous pemphigoid
- Pemphigus vulgaris
- Linear immunoglobulin A disease
- Dermatitis herpetiformis
- Lupus erythematosus
- Chronic ulcerative stomatitis
- Chronic bacterial, fungal, and viral infections
- Reactions to medications, mouthwashes, and chewing gum
Rare causes include:
- Crohn’s disease
- Sarcoidosis
- Leukemia
- factitious (self inflicted) lesions
- Squamous cell carcinoma (can be mistaken for desquamative gingivitis)
Pityriasis amiantacea (also known as "Tinea amiantacea") is an eczematous condition of the scalp in which thick tenaciously adherent scale infiltrates and surrounds the base of a group of scalp hairs. It does not result in scarring or alopecia.
Pityriasis amiantacea was first described by Alibert in 1832. Pityriasis amiantacea affects the scalp as shiny asbestos-like (amiantaceus) thick scales attached in layers to the hair shaft. The scales surround and bind down tufts of hair. The condition can be localised or covering over the entire scalp. Temporary alopecia and scarring alopecia may occur due to repeated removal of hairs attached to the scale. It is a rare disease with a female predilection.
Pityriasis amiantacea can easily be misdiagnosed due its close resemblance to other scalp diseases such as psoriasis, seborrhoeic dermatitis or lichen planus. However in pityriasis amiantacea the scales are attached to both the hair shaft and the scalp. Pityriasis amiantacea may be present with other inflammatory conditions such as atopic dermatitis or seborrhoeic dermatitis and sebaceous scales and alopecia can occur. According to the dermatology text Bolognia this condition is most often seen in psoriasis, but may also be seen in secondarily infected atopic dermatitis, seborrheic dermatitis, and tinea capitis.