Pemphigus

Pemphigus is a rare group of autoimmune diseases that affect the skin and mucous membranes. It is a blistering disease in which autoantibodies are directed against Desmosglein - a protein, which acts like a glue to cement epidermal cells together.

When Desmogleins are targeted, the epidermal cells become detached from one another (acantholysis) and this produces blisters or lesions that fail to heal. These blisters can then progress to become sores that cover a significant area of the skin and the mouth.

Till the 1960s little was known about pemphigus.It was in 1971 that the autoimmune nature of this disease was published.

Pemphigus has a world wide occurrence and affects both males and females, of all ethnic groups.





Some people have a greater pre- ponderence to develop the disease such as those with a familial history and those with other auto- immune diseases such as Myasthenia gravis or thyroid cancer.

Drug-induced pemphigus vulgaris : Drugs reported most significantly in association with pemphigus vulgaris include penicillamine, captopril, cephalosporin, pyrazolones, nonsteroidal anti-inflammatory drugs (NSAIDs), and other thiol-containing compounds. Rifampin, emotional stress, thermal burns, ultraviolet rays, and infections (eg, coxsackievirus, Herpesviridae family) have also been reported as triggers for pemphigus vulgaris.




Clinical Presentation:

Mucous membranes typically are affected first in pemphigus vulgaris. Mucosal lesions may precede cutaneous lesions by weeks or months. Patients with mucosal lesions may present to dentists, oral surgeons, or gynecologists.

• Mucous membranes

  • Intact bullae are rare in the mouth. More commonly, patients have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. The erosions extend peripherally with shedding of the epithelium.
  • The mucous membranes most often affected in pemphigus vulgaris are those of the oral cavity, which is involved in almost all patients with pemphigus vulgaris and sometimes is the only area involved. Erosions may be seen on any part of the oral cavity. Erosions can be scattered and often are extensive. Erosions may spread to involve the larynx, with subsequent hoarseness. The patient often is unable to eat or drink adequately because the erosions are so uncomfortable.
  • In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of the cases.
  • Other mucosal surfaces may be involved, including the conjunctiva,esophagus (causes odynophagia and/or dysphagia),labia, vagina, cervix, vulva, penis, urethra, nasal mucosa, and anus.
• Skin

  • The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base, as shown in the images below.Early, small blister filled with clear fluid arises on healthy skin.Flaccid blister filled with clear fluid arises on healthy skin.
  • The blisters are fragile; therefore, intact blisters may be sparse. The contents soon become turbid, or the blisters rupture, producing painful erosions, which is the most common skin presentation and is shown in the image below. Erosions often are large because of their tendency to extend peripherally with the shedding of the epithelium.An erosion.
• Vegetating pemphigus vulgaris: Ordinary pemphigus vulgaris erosions may develop vegetation. Lesions in skin folds readily form vegetating granulations. In some patients, erosions tend to develop excessive granulation tissue and crusting, and these patients display more vegetating lesions. This type of lesion tends to occur more frequently in intertriginous areas and on the scalp or face. The vegetating type of response can be more resistant to therapy and can remain in one place for long periods.
• Pemphigus in pregnancy: Pemphigus vulgaris occurring in pregnancy is rare. When present, maternal autoantibodies may cross the placenta, resulting in neonatal pemphigus. Neonatal pemphigus is transient and improves with clearance of maternal autoantibodies.Treatment of pemphigus vulgaris in pregnancy is with oral corticosteroids; however, prednisone and its metabolites cross the placenta and have been associated with low birth weight, prematurity, infection, and adrenal insufficiency.
• Nikolsky sign: In patients with active blistering, firm sliding pressure with a finger separates normal-appearing epidermis, producing an erosion. This sign is not specific for pemphigus vulgaris and is found in other active blistering diseases.
• Asboe-Hansen sign: Lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin.
  
  
  
  
  Histological Findings-Histopathology demonstrates an intradermal blister. The earliest changes consist of intercellular edema with loss of intercellular attachments in the basal layer. Suprabasal epidermal cells separate from the basal cells to form clefts and blisters. Basal cells are separated from one another and stand like a row of tombstones on the floor of the blister, but they remain attached to the basement membrane. Blister cells contain some acantholytic cells. Histopathology can help differentiate pemphigus vulgaris from pemphigus foliaceous, which demonstrates a more superficial epidermal cleavage.
  Tzanck preparation is a smear taken from the base of a blister or an oral erosion that contains acantholytic cells. Blistering is preceded by eosinophilic spongiosis in some patients. The superficial dermis has a mild, superficial, mixed inflammatory infiltrate, which includes some eosinophils.
  
  
  
  

  Medication Summary

  The aim of treatment is to reduce the inflammatory response and autoantibody production. While target-specific therapy is not available, non–target-specific treatments currently are used. The most commonly used medications are corticosteroids.

  The introduction of corticosteroids has reduced mortality greatly, but significant morbidity remains. Immunosuppressants should be considered early in the course of disease, as steroid-sparing agents. Mycophenolate mofetil and azathioprine are the usual agents considered as initial choices.Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring.

Herpes Zoster

Varicella-zoster virus (VZV) is the agent causing varicella, otherwise known as chickenpox, the common childhood infection. Following resolution of chickenpox, VZV lies dormant in the spinal dorsal root ganglia until a decrease in cellular immunity triggers the reactivation of the virus, resulting in herpes zoster, otherwise known as shingles. 

Shingles is a syndrome characterized by a painful, vesicular rash that is usually restricted to a unilateral dermatomal distribution. At times, especially in the immunosuppressed patient, the infection may spread and produce severe systemic illness, with involvement of multiple visceral organs and multiple dermatomes (disseminated zoster). 

Shingles usually has a benign course, but complications may occur, ranging from mild to life threatening. In properly selected patients, early treatment with antivirals and corticosteroids has been shown to decrease the duration of symptoms and to possibly prevent or ameliorate some complications. 

The clinical manifestations of herpes zoster can be divided into the preeruptive phase (preherpetic neuralgia), acute eruptive phase, and chronic phase (postherpetic neuralgia).

VZV reactivation causes inflammation in the dorsal root ganglion, accompanied by hemorrhagic necrosis of nerve cells. The result is neuronal loss and fibrosis. The distribution of the rash corresponds to the sensory fields of the infected neurons within a specific ganglion. The anatomic location of the involved dermatome often determines the specific manifestations (eg, herpes zoster ophthalmicus [HZO], causing ocular complications, when the trigeminal ganglion is involved).

Pathophysiology-

Central nervous system involvement

While zoster is classically described in sensory (dorsal root) ganglia, it can spread to affect any portion of the nervous system.

Involvement of the anterior horn cells can produce muscular weakness, cranial nerve palsies, diaphragmatic paralysis, neurogenic bladder, and colon pseudo-obstruction.

Wider involvement of the spinal cord can produce Guillain-Barré syndrome, transverse myelitis, and myositis.

In severely ill or immunocompromised patients, general central nervous system (CNS) involvement can be observed in the form of meningoencephalitis or encephalitis. Such presentations are indistinguishable from other forms of meningoencephalitis, although other evidence of acute zoster usually is present.Cerebrospinal fluid (CSF) studies frequently reveal pleocytosis without elevated protein. These infections can be life threatening.

Herpes zoster ophthalmicus

This is potentially the most devastating form of acute zoster.

Herpes zoster ophthalmicus (HZO) results from the reactivation of varicella-zoster virus (VZV) in the trigeminal (fifth cranial) nerve. Any branch of the nerve may be affected, although the frontal branch within the first division of the trigeminal nerve is most commonly involved. This branch innervates nearly all of the ocular and periocular structures.

Ramsay Hunt syndrome

Also known as herpes zoster oticus, geniculate neuralgia, or herpes zoster auricularis, Ramsay-Hunt syndrome is caused by VZV reactivation involving the facial and auditory nerves. 

Vesicular eruptions may manifest on the pinna, tragus, or tympanic membrane or in the auditory canal, as well as anywhere in the facial nerve distribution.

The patient may experience hearing impairment, nystagmus, vertigo, or facial nerve palsy (mimicking Bell palsy).Patients may lose taste sensation in the anterior two thirds of the tongue.

Ramsay Hunt syndrome may go unnoticed and be difficult to diagnose, especially in elderly patients.

Etiology-

VZV is an enveloped, double-stranded deoxyribonucleic acid (DNA) virus belonging to the Herpesviridae family. In humans, primary infection with VZV occurs when the virus comes into contact with the mucosa of the respiratory tract or conjunctiva. From these sites, it is distributed throughout the body via mononuclear cells in the blood stream. In tissue, VZV spreads from cell to cell via direct contact to produce its effects.

After primary infection, the virus migrates along sensory nerve fibers to the satellite cells of dorsal root ganglia where it becomes dormant. This dormancy may be permanent, or the virus may become reactivated by conditions of decreased cellular immunity, resulting in herpes zoster.

Exactly why VZV reactivates from latency is not fully understood. However, cell-mediated, VZV-specific immunity has been shown to be a major factor in determining reactivation of VZV. Cell-mediated, VZV-specific immunity decreases with age and in patients with certain malignancies. These groups have much higher rates of herpes zoster. Patients with hypogammaglobulinemia (a defect of humoral, but not cellular, immunity) do not have a higher rate of zoster. This supports the concept of an important role for cell-mediated immunity in the pathogenesis of VZV infection.

No identifiable environmental, genetic, or social risk factors exist for the development of the reactivation of VZV.

Clinical Features-

Prodromal pain precedes the rash in approximately 70-80% of patients; it is typically confined to the same dermatomal distribution as the rash. In immunocompetent patients, herpes zoster is generally restricted to one dermatome, with possible limited involvement of adjacent dermatomes due to normal variations in innervation. Many patients describe the pain as "burning," "throbbing," or "stabbing." It may be severe, mild, constant, rare, or felt as another sensation such as pruritus. The involved area may be tender to palpation.

After 48-72 hours, or longer in some cases, the rash develops. Initially, and briefly, maculopapular lesions rapidly transition to vesicles in 1-2 days. New vesicles tend to form over 3-5 days, sometimes coalescing to form bullae. The lesions then rupture and release their contents, ulcerate, crust over, and dry, over 7-10 days. As with chickenpox, once crusting occurs, the lesions are no longer infectious.

Depending on the dermatome involved, additional physical examination findings may include the following:

• Corneal ulcers, conjunctivitis
• Regional lymphadenopathy
• Cranial nerve palsies
• Peripheral facial nerve palsy
• Delirium, confusion, coma (in patients with meningoencephalitis)
• Loss of taste in the anterior tongue (in Ramsay Hunt Syndrome)

Symptomatic involvement of multiple dermatomes or bilateral aspects of the same dermatome (ie, crossing the midline) may indicate disseminated disease or another etiology such as herpes simplex virus (HSV) infection.

Symptoms and lesions tend to resolve over 10-15 days. However, lesions may require up to 1 month to completely heal. Scarring and hyperpigmentation or hypopigmentation at lesion sites may persist for a long period or may be permanent. Pain duration is variable but is usually less than 1 month. Pain lasting longer than 1 month is referred to, by definition, as postherpetic neuralgia.

Less than 20% of patients have systemic symptoms, such as headache, fever, malaise, or fatigue, at any point during a case of herpes zoster.

Herpes zoster may develop without the typical rash, as aseptic meningitis or zoster sine herpete, which is a condition defined as pain and paresthesias along a dermatome without the development of visible cutaneous involvement

Treatment-

Episodes of herpes zoster are generally self-limited and resolve without intervention. However, effective treatments do exist and can reduce the extent and duration of symptoms, and possibly the risk of chronic sequelae (ie, postherpetic neuralgia) as well. Treatment is of most benefit in those patient populations at risk for prolonged or severe symptoms, specifically, immunocompromised people and persons older than 50 years. The benefit of treating younger and healthier populations is unclear.

Uncomplicated zoster does not require inpatient care. Patients at high risk for disseminated zoster may benefit from intravenous (IV) acyclovir. Patients with disseminated zoster usually require admission for IV acyclovir. Inpatient care is also recommended for any patient demonstrating disseminated disease or ophthalmic or meningoencephalopathic involvement.

The use of steroids in conjunction with an antiviral for uncomplicated herpes zoster is controversial.

The addition of oral corticosteroids has been evaluated in patients treated with acyclovir in 2 controlled studies. Steroids were found to accelerate the resolution of acute neuritis and provide a clear improvement in quality-of-life measures in comparison to those patients treated with antivirals alone. The use of oral steroids had no effect on the development or duration of postherpetic neuralgia. Oral steroids have not been studied with valacyclovir or famciclovir, so the benefit is unknown.

Nonoral forms of adjunctive steroid therapy in acute herpes zoster have also been studied. A study involving a single epidural injection of steroids and local anesthetics given in conjunction with a standard regimen of oral antivirals and analgesics was found to modestly improve zoster-associated pain for 1 month over treatment without steroids. As above, no effect in preventing postherpetic neuralgia was noted.

In light of the potential adverse effects of and contraindications to corticosteroid use, current expert opinion suggests limiting their involvement to cases of moderate to severe zoster pain, or in which significant neurological symptoms (such as facial paralysis) or CNS involvement is present (and use of corticosteroids is not otherwise contraindicated).

The optimal duration of steroid therapy is not known. If prescribed, it seems reasonable for steroids to be used concurrently with antiviral therapy. The duration of steroid use should not extend beyond the period of antiviral therapy. Steroids should not be given alone (without antiviral therapy), owing to concern about the promotion of viral replication.

Treatment of complicated herpes zoster-

Individuals with altered cell-mediated immunity, due to an immunosuppressive condition (eg, HIV, cancer) or treatment (eg, extended corticosteroid use), are at increased risk for herpes zoster. Further, herpes zoster presentations in the immunocompromised population can be complicated by disseminated disease and visceral organ involvement.

Antiviral therapy has been demonstrated to halt progression and dissemination of acute herpes zoster in immunocompromised patients, even when initiated more than 72 hours after rash onset.As such, current expert opinion recommends the use of antiviral therapy in all immunocompromised zoster patients who present prior to full crusting of all lesions.

Intravenous acyclovir remains the drug of choice for selected populations of immunocompromised patients, as follows:

• Patients with evidence of disseminated disease or visceral organ involvement
• Patients with ophthalmic involvement
• Patients with advanced HIV/AIDS with active opportunistic infections or prominent wasting
• Transplant recipients soon after transplantation or when being treated for rejection

Patients without such risk factors can be treated with oral antivirals. Data on adjunctive therapy with corticosteroids are lacking, and this therapy is not currently recommended. Antiviral therapy should be continued until resolution of all lesions.

Herpes simplex

Herpes simplex viruses are ubiquitous, host-adapted pathogens that cause a wide variety of disease states. Two types exist: herpes simplex virus type 1 (HSV-1) and type 2 (HSV-2). Both are closely related but differ in epidemiology. HSV-1 is traditionally associated with orofacial disease, while HSV-2 is traditionally associated with genital disease; however, lesion location is not necessarily indicative of viral type.

Up to 80% of herpes simplex infections are asymptomatic. Symptomatic infections can be characterized by significant morbidity and recurrence. In immunocompromised hosts, infections can cause life-threatening complications.

Pathophysiology-

The prevalence of HSV infection worldwide has increased over the last several decades, making it a major public health concern. Prompt recognition of herpes simplex infection and early initiation of therapy are of utmost importance in the management of the disease.

HSV (both types 1 and 2) belongs to the family Herpesviridae and to the subfamily Alphaherpesvirinae. It is a double-stranded DNA virus characterized by the following unique biological properties

• Neurovirulence (the capacity to invade and replicate in the nervous system)
• Latency (the establishment and maintenance of latent infection in nerve cell ganglia proximal to the site of infection): In orofacial HSV infections, the trigeminal ganglia are most commonly involved, while, in genital HSV infection, the sacral nerve root ganglia (S2-S5) are involved.
• Reactivation: The reactivation and replication of latent HSV, always in the area supplied by the ganglia in which latency was established, can be induced by various stimuli (eg, fever, trauma, emotional stress, sunlight, menstruation), resulting in overt or covert recurrent infection and shedding of HSV. In immunocompetent persons who are at an equal risk of acquiring HSV-1 and HSV-2 both orally and genitally, HSV-1 reactivates more frequently in the oral rather than the genital region. Similarly, HSV-2 reactivates 8-10 times more commonly in the genital region than in the orolabial regions. Reactivation is more common and severe in immunocompromised individuals.

Dissemination of herpes simplex infection can occur in people with impaired T-cell immunity, such as in organ transplant recipients and in individuals withAIDS.

HSV is distributed worldwide. Humans are the only natural reservoirs, and no vectors are involved in transmission. Endemicity is easily maintained in most human communities owing to latent infection, periodic reactivation, and asymptomatic virus shedding.

HSV is transmitted by close personal contact, and infection occurs via inoculation of virus into susceptible mucosal surfaces (eg, oropharynx, cervix, conjunctiva) or through small cracks in the skin. The virus is readily inactivated at room temperature and by drying; hence, aerosol and fomitic spread are rare.

HSV-1 infections transmitted via saliva are common in children, although primary herpes gingivostomatitis can be observed at any age.

Clinical presentation-

The clinical course of herpes simplex infection depends on the age and immune status of the host, the anatomic site of involvement, and the antigenic virus type. Primary herpes simplex virus (HSV)–1 and HSV-2 infections are accompanied by systemic signs, longer duration of symptoms, and higher rate of complications. Recurrent episodes are milder and shorter. Both HSV-1 and HSV-2 can cause similar genital and orofacial primary infections after contact with infectious secretions containing either HSV-1 (usually oral secretions) or HSV-2 (usually genital secretions).

• Acute herpetic gingivostomatitis
  

  • This is a manifestation of primary HSV-1 infection that occurs in children aged 6 months to 5 years. Adults may also develop acute gingivostomatitis, but it is less severe and is associated more often with a posterior pharyngitis.
  • Infected saliva from an adult or another child is the mode of infection. The incubation period is 3-6 days.
  • Clinical features include the following:
    • Abrupt onset
    • High temperature (102-104°F)
    • Anorexia and listlessness
    • Gingivitis (This is the most striking feature, with markedly swollen, erythematous, friable gums.)
    • Vesicular lesions (These develop on the oral mucosa, tongue, and lips and later rupture and coalesce, leaving ulcerated plaques.)
    • Tender regional lymphadenopathy
    • Perioral skin involvement due to contamination with infected saliva
  • Course: Acute herpetic gingivostomatitis lasts 5-7 days, and the symptoms subside in 2 weeks. Viral shedding from the saliva may continue for 3 weeks or more.
• Acute herpetic pharyngotonsillitis
  

  • In adults, oropharyngeal HSV-1 infection causes pharyngitis and tonsillitis more often than gingivostomatitis.
  • Fever, malaise, headache, and sore throat are presenting features.
  • The vesicles rupture to form ulcerative lesions with grayish exudates on the tonsils and the posterior pharynx.
  • Associated oral and labial lesions occur in fewer than 10% of patients.
  • HSV-2 infection can cause similar symptoms and can be associated with orogenital contact or can occur concurrently with genital herpes.
• Herpes labialis/Cold sore
  

  • This is the most common manifestation of recurrent HSV-1 infection. A prodrome of pain, burning, and tingling often occurs at the site, followed by the development of erythematous papules that rapidly develop into tiny, thin-walled, intraepidermal vesicles that become pustular and ulcerate. In most patients, fewer than two recurrences manifest each year, but some individuals experience monthly recurrences.
  • Maximum viral shedding is in the first 24 hours of the acute illness but may last 5 days.

  
  

  
  

  Physical-

This section describes physical examination findings of the herpetic lesion as it relates to primary and recurrent lesions of cutaneous or mucosal HSV infection. This can be related to either oral or genital infection.

• Primary mucocutaneous HSV infections
  

  • Some primary infections are asymptomatic.
  • Primary (first-episode) infections manifest within several days of exposure to secretions containing viable virus.
  • Often painful, the lesions quickly progress to vesicles and can continue to erupt over 1-2 weeks.
  • The lesions are prominent and are often present internally on the mucosal surface of the oral or genital area, as well as on the surrounding skin.
  • Constitutional symptoms (fever, malaise, myalgias, and anorexia) are often prominent. Weight loss is not uncommon and is due either to illness or dysphagia (in primary gingivostomatitis).
  • Individual vesicles on mucosal surfaces break down rapidly, forming shallow painful ulcers (usually < 8-10 mm in diameter). They may be covered with a white exudate that can be confused with mucosal candidiasis. Those on cutaneous surfaces remain as vesicles longer, only to evolve into crusted ulcers that heal within 5-7 days.
• Recurrent mucocutaneous HSV infections
  

  • Following the establishment of latency in the corresponding sensory nerve ganglion cells, HSV can cause recurrent infection that can be subclinical (manifesting as viral excretion without lesions) or overt (manifesting as mucosal or cutaneous lesions with viral excretion).
  • Oral recurrences are often triggered by recognizable stimuli such as pyrexia (fever blisters and cold sores), stress, or sunburn. Genital recurrences are more likely to be linked to stress rather than to pyrexia. Females may relate a relationship to the menstrual cycle.
  • Localized burning or paraesthesias may precede recurrent lesions. Unlike primary infection, constitutional symptoms are minimal in most cases.
  • Recurrences last 3-7 days and can occur numerous times per year or once or twice in a lifetime. Overall, the number of yearly recurrences tends to decrease over time.
  • Although recurrent HSV infections may last much longer (>30 d) in immunocompromised hosts, such as individuals with AIDS, frequent recurrences are not necessarily a sign of an altered immune system.
  • Because recurrences can be clinically unrecognizable, transmission to susceptible individuals can occur in the absence of overt lesions. In genital HSV infections, barrier protection should be used regardless of existing lesions, even in the absence of a history of genital HSV infection.

  Causes-

  
  

  • HSV is transmitted via close personal contact.
  • HSV infection occurs via inoculation of virus into susceptible mucosal surfaces (eg, oropharynx, cervix, conjunctiva) or through small cracks in the skin.
  • The virus is inactivated readily at room temperature and by drying; hence, aerosol and fomitic spread are rare.
  • HSV-1 is transmitted chiefly by contact with infected saliva, whereas HSV-2 is transmitted sexually or from a mother's genital tract infection to her newborn. However, lesion location does not always indicate viral type.
    Treatment-
    Overall, medical treatment of herpes simplex virus (HSV) infection is centered around specific antiviral treatment. While the same medications are active against HSV-1 and HSV-2, the location of the lesions and the chronicity (primary or reactivation) of the infection dictate the dosage and frequency of medication. It is important to note that life-threatening HSV infections in immunocompromised patients and HSV encephalitis require high-dose intravenous acyclovir, often started empirically.

    When constitutional effects such as fever occur, symptomatic treatment can be used.

    Appropriate wound care is needed, and treatment for secondary bacterial skin infections may be required.

    Fluids can be given to avoid dehydration.

Oral lichen planus

Oral lichen planus (OLP) is a chronic inflammatory disease that causes bilateral white striations, papules, or plaques on the buccal mucosa, tongue, and gingivae. Erythema, erosions, and blisters may or may not be present. 

Pathophysiology-

Current data suggest that oral lichen planus is a T-cell–mediated autoimmune disease in which autocytotoxic CD8⁺ T cells trigger apoptosis of oral epithelial cells.

Oral lichen planus lesions contain increased levels of the cytokine tumor necrosis factor (TNF)–alpha.Basal keratinocytes and T cells in the subepithelial infiltrate express TNF in situ..

History-

The lichen planus antigen is unknown, although it may be a self-peptide (or altered self-peptide), in which case lichen planus would be a true autoimmune disease. The role of autoimmunity in the pathogenesis is supported by many autoimmune features of oral lichen planus, including its chronicity, onset in adults, predilection for females, association with other autoimmune diseases, occasional tissue-type associations, depressed immune suppressor activity in patients with oral lichen planus, and the presence of autocytotoxic T-cell clones in lichen planus lesions. The expression or unmasking of the lichen planus antigen may be induced by drugs (lichenoid drug reaction), contact allergens in dental restorative materials or toothpastes (contact hypersensitivity reaction), mechanical trauma (Koebner phenomenon), viral infection, or other unidentified agents.


Clinical Features-

In many patients, the onset of oral lichen planus is insidious, and patients are unaware of their oral condition. In such instances, the referring medical or dental practitioner identifies the clinical changes in the oral mucosa.

Some patients report a roughness of the lining of the mouth, sensitivity of the oral mucosa to hot or spicy foods or oral hygiene products, painful oral mucosa, sore gums, red or white patches on the oral mucosa, red gums, or oral ulcerations.

Approximately two thirds of patients with oral lichen planus report oral discomfort, especially in association with atrophic and erosive lesions. Erythematous and erosive lesions are often sensitive or painful. Symptoms vary from mucosal sensitivity to continuous debilitating pain.

Oral mucosal lichenoid lesions may occur after the administration of systemic drugs such as nonsteroidal anti-inflammatory drugs (NSAIDs), sulfonylureas, antimalarials, beta-blockers, and some angiotensin-converting enzyme (ACE) inhibitors. The period between the commencement of the drug therapy and the clinical appearance of oral lichen planus–like disease varies.

In rare cases, oral mucosal lichenoid lesions occur after a dental restoration is performed or after the patient starts using a denture; the lag period varies. Patients with an associated allergy to metals or components of the appliance should be evaluated by means of patch testing.

Up to 44% of patients with oral lichen planus develop coincident skin lesions. Conversely, more that 70% of patients with cutaneous lichen planus develop coincident oral lichen planus.

The genitals are involved in as many as 25% of women with oral lichen planus, compared with only 2-4% of men with oral lichen planus. The features are similar to those of the oral lesions. Patients do not often complain of pain or pruritus, although on questioning, they may admit to such symptoms.

In patients with oral lichen planus, scalp involvement (lichen planopilaris) is rare.

Nail involvement in patients with oral lichen planus is uncommon.

In a small group of patients, lichen planus may involve the esophagus.

Physical-

Pertinent physical findings in oral lichen planus are limited to the oral mucosa. Some patients present with coincident lesions on the skin, scalp, nails, genital mucosa, esophageal mucosa, larynx, and conjunctivae. Complete history taking and physical examination by a dermatologist may be required in patients with extra-oral symptoms or signs associated with oral lichen planus.

Patients with reticular lesions are often asymptomatic, whereas those with atrophic (erythematous) or erosive (ulcerative) disease commonly have significant local morbidity. The oral pain is variable and exacerbated by trauma and foods, particularly those that are hot, spicy, or acidic.

Oral mucosal lesions are variable and present as white striations (Wickham striae), white papules, white plaques, erythema (mucosal atrophy), erosions (shallow ulcers), or blisters. The lesions predominantly affect the buccal mucosa, tongue, and gingivae, although other oral sites are occasionally involved. The lesions are usually bilateral.

The lesions may appear as a mixture of clinical subtypes. For example, white streaks and gray streaks may form a linear or reticular pattern on an erythematous background. Alternatively, a central area of shallow ulceration (erosion) may have a yellowish surface (fibrinous exudate) surrounded by an area of erythema.

In most patients, telltale white striations or papules are evident on the buccal mucosa or on the lateral margin of the tongue, either alone or in combination with other lesions.

Gingival lesions commonly appear with a fiery red erythema that affects the entire width of the attached gingiva, a condition previously called desquamative gingivitis.

In patients predisposed to pigmentation, oral lichen planus lesions may be associated with patchy brown melanin deposits in the oral mucosa (inflammatory melanosis).

Oral lichen planus lesions usually persist for many years with periods of exacerbation and quiescence. During periods of exacerbation, the area of erythema or erosion increases, with increased pain and sensitivity. During periods of quiescence, the area of erythema or erosion decreases, with decreased pain and sensitivity. Patients are often unaware of quiescent oral lichen planus, which may manifest as faint white striations, papules, or plaques. Exacerbations of oral lichen planus have been linked to periods of psychological stress and anxiety.

Lichenoid drug reactions have the same clinical features as those of idiopathic oral lichen planus. Lichenoid disease may be unilateral and associated with circulating epithelial antinuclear antibodies, but few data support this possibility. Rarely, lichenoid reactions of the oral mucosa occur on the oral mucosa in contact with (or close to) an amalgam or composite resin dental restoration, or a denture component. Mechanical trauma (the Koebner phenomenon) may exacerbate lichenoid lesions, especially when it affects the midline of the buccal mucosa or the lateral margin of the tongue.

Up to 44% of patients with oral lichen planus develop coincident skin lesions. These typically appear as pruritic, flat-topped, violaceous papules and plaques that predominantly affect the flexor aspects of the wrists or ankles, the extensor aspects of the lower legs, the skin of the lower central part of the back, and the natal cleft.

Causes-

Current data suggest that oral lichen planus is a T-cell–mediated autoimmune disease in which autocytotoxic CD8⁺ T cells trigger the apoptosis of oral epithelial cells. However, the precise cause of oral lichen planus is unknown.

In many patients, a cause for the oral lichenoid lesions cannot be identified; in these patients, the disease is called idiopathic oral lichen planus.

Oral lichenoid drug reactions may be triggered by systemic drugs including NSAIDs, beta-blockers, sulfonylureas, some ACE inhibitors, and some antimalarials. In patients with oral lichenoid lesions, be alert for any systemic drug as a cause.

Oral lichenoid contact-sensitivity reactions may be triggered by contact allergens including dental amalgam composite resin, and toothpaste flavorings, especially cinnamates. Skin patch testing may help in identifying contact allergens (see Other Tests). If an allergy is detected, lesions may heal when the offending material is removed.

Oral lichenoid lesions may be triggered by mechanical trauma (Koebner phenomenon) due to calculus deposits, sharp teeth, rough surfaces of dental restorations or prostheses, cheek or tongue biting, and oral surgical procedures. Scale any teeth associated with oral lichen planus lesions to remove calculus deposits and reduce sharp edges. Dental restorations and prostheses that are associated with oral lichen planus lesions should be mirror-polished.

Some studies have revealed viral infections in oral lichen planus, including those due to human papillomavirus (HPV-6, 11, 16, or 18) and human herpesvirus 6. A causal role for viral infection in oral lichen planus has not been identified.

Some studies show an increased incidence of C albicans infection in oral lichen planus. A causal role for C albicans infection in oral lichen planus has not been identified.

Some study findings suggest an association between oral lichen planus and chronic hepatic diseases such as hepatitis C virus (HCV) infection, autoimmune chronic active hepatitis, and primary biliary cirrhosis. 

Oral lichenoid lesions may arise in people who habitually chew betel quid. A causal role for betel quid in oral lichen planus has not been identified.

Oral lichenoid lesions are part of the spectrum of chronic graft-versus-host disease that occurs after allogeneic hemopoietic stem cell transplantation.

No consistent association with human leukocyte antigen (HLA) is reported in oral lichen planus. This finding suggests that the patient's genetic background does not play a critical role in oral lichen planus pathogenesis.

Little evidence supports a connection between diabetes mellitus and oral lichen planus. The oral lichenoid lesion in Grinspan syndrome (triad of oral lichen planus, diabetes mellitus, and hypertension) is probably an adverse effect of the drug therapy for diabetes mellitus and hypertension.

Hitopathology-

Histopathologic examination of lesional tissue is the most relevant investigation in cases of oral lichen planus.Consistent findings include a bandlike subepithelial mononuclear infiltrate consisting of T cells and histiocytes, increased numbers of intraepithelial T cells, and degenerating basal keratinocytes that form colloid (Civatte, hyaline, cytoid) bodies, which appear as homogenous eosinophilic globules. Variable findings include parakeratosis, acanthosis, and sawtooth rete pegs.

Degeneration of the basal keratinocytes and disruption of the anchoring elements of the epithelial basement membrane and basal keratinocytes (eg, hemidesmosomes, filaments, fibrils) weakens the epithelial-connective tissue interface. As a result, histologic clefts (ie, Max-Joseph spaces) may form, and blisters on the oral mucosa (bullous lichen planus) may be seen at clinical examination. B cells and plasma cells are uncommon findings.

Treatment-

Medical treatment of oral lichen planus (OLP) is essential for the management of painful, erythematous, erosive, or bullous lesions. The principal aims of current oral lichen planus therapy are the resolution of painful symptoms, the resolution of oral mucosal lesions, the reduction of the risk of oral cancer, and the maintenance of good oral hygiene. In patients with recurrent painful disease, another goal is the prolongation of their symptom-free intervals.

The main concerns with the current therapies are the local and systemic adverse effects and lesion recurrence after treatment is withdrawn. No treatment of oral lichen planus is curative.

Eliminate local exacerbating factors. Treat any sharp teeth or broken restorations or prostheses that are likely to cause physical trauma to areas of erythema or erosion by using conventional dental means. Scale the teeth to remove calculous deposits and reduce sharp edges. If the patient has an isolated plaquelike or erosive oral lichen planus lesion on the buccal or labial mucosa adjacent to a dental restoration, and if an allergy is detected by means of skin patch testing, the lesion may heal if the offending material is removed or replaced. (However, most lichenoid lesions adjacent to dental restorations are asymptomatic.)

If systemic drug therapy (eg, treatment with NSAIDs, antimalarials, or beta-blockers) is suspected as the cause of oral lichenoid lesions, changing to another drug may be worthwhile. This change must be undertaken only by the patient's attending physician. However, the switch rarely resolves the erosions, and almost never resolves the white patches of oral lichen planus.

Inform all patients with oral lichen planus about their slightly increased risk of oral SCC (the most common of all oral malignancies). As with all patients, advise those with oral lichen planus that this risk may be reduced by eliminating tobacco and alcohol consumption and by consuming a diet rich in fresh fruits and vegetables, among other measures. Erosive and atrophic lesions can be converted into reticular lesions by using topical steroids. Therefore, the elimination of mucosal erythema and ulceration, with a residual asymptomatic reticular or papular lesions, may be considered an end point of current oral lichen planus therapy. With respect to plaque lesions, the effect of treatment on the risk of oral cancer is unclear.

Topical corticosteroids are the mainstay of medical treatment of oral lichen planus, although rarely, corticosteroids may be administered intralesionally or systemically. Some topical corticosteroid therapies may predispose the patient to oral pseudomembranous candidosis. However, this condition is rarely if ever symptomatic, and it generally does not complicate healing of the erosions related to oral lichen planus. Topical antimycotics (eg, nystatin, amphotericin) may be prescribed when an infection is present.

Erosive oral lichen planus that is recalcitrant to topical corticosteroids may respond to topical tacrolimus. Other potential therapies for recalcitrant oral lichen planus include hydroxychloroquine, azathioprine, mycophenolate, dapsone, systemic corticosteroids, and topical and systemic retinoids.

Tonsillitis

Tonsillitis is inflammation (swelling) of the tonsils.

Causes, incidence, and risk factors

The tonsils are lymph nodes in the back of the mouth and top of the throat. They normally help to filter out bacteria and other germs to prevent infection in the body.

Strep throat is one cause of tonsilitis.

The tonsils may become so overwhelmed by a bacterial or viral infection that they swell and become inflamed, causing tonsillitis. The infection may also be present in the throat and areas around it, causing inflammation of the pharynx. The pharynx is in the back of the throat, between the tonsils and voicebox (larynx). 

Tonsillitis is very common, especially in children.

Symptoms

• Difficulty swallowing
• Ear pain
• Fever, chills
• Headache
• Sore throat - lasts longer than 48 hours and may be severe
• Tenderness of the jaw and throat
• Voice changes, loss of voice

Signs and tests

The health care provider will look in the mouth and throat for swollen tonsils. The tonsils are usually red and may have white spots on them. The lymph nodes in the jaw and neck may be swollen and tender to the touch.

Tests that may be done include:

• Blood count
• Mononucleosis test
• Rapid strep test
• Throat swab culture

Treatment

If bacteria such as strep are causing the tonsillitis, antibiotics are given to cure the infection. The antibiotics may be given once as a shot, or taken for 10 days by mouth.

If antibiotic pills are used, they must be taken for the entire amount of time prescribed by the doctor. DO NOT stop taking them just because the discomfort stops, or the infection may not be cured.

Other treatments include:

• Drink cold liquids or suck on popsicles
• Drink fluids, especially warm (not hot), bland fluids
• Gargle with warm salt water
• Suck on lozenges (containing benzocaine or similar ingredients) to reduce pain (these should not be used in young children because of the choking risk)
• Take over-the-counter medications, such as acetaminophen (Tylenol) or ibuprofen to reduce pain and fever. Do NOT give a child aspirin. Aspirin has been linked to Reye syndrome.

Some people who have repeated infections may need surgery to remove the tonsils (tonsillectomy).

Expectations (prognosis)

Tonsillitis symptoms usually improve 2 or 3 days after treatment starts. The infection usually is cured after treatment is completed, but some people may need more than one course of antibiotics.

Complications of untreated strep tonsillitis may be severe. Children with tonsillitis related to strep throat or pharyngitis should generally be kept home from school or day care until they have been on antibiotics for 24 hours. This helps reduce the spread of illness.

Complications

• Blocked airway from swollen tonsils
• Dehydration from difficulty swallowing fluids
• Kidney failure
• Peritonsillar abscess or abscess in other parts of the throat
• Pharyngitis - bacterial
• Post-streptococcal glomerulonephritis
• Rheumatic fever and related cardiovascular disorders