Introduction:
Vesiculobullous lesions are a group of oral disorders that present with the formation of vesicles or bullae. These eruptions are rarely observed intraorally in an intact state as they rupture, turning into erosions or ulcers. These lesions might precipitate as a result of viruses, autoimmunity, or genetic etiology.
What Are the Types of Vesiculobullous Disorders?
Based on the separation at the intraepithelial level:
Granular Layer
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Pemphigus foliaceous.
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Pemphigus erythematosus.
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Frictional blisters.
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Bullous impetigo.
Spinous Layer
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Familial benign pemphigus.
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Herpes simplex virus infection.
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Herpes zoster and varicella zoster.
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Eczematous dermatitis.
Suprabasal Layer
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Pemphigus vulgaris.
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Pemphigus vegetans
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Darier's disease.
Basal Layer
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Toxic epidermal necrolysis(TEN).
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Lichen planus.
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Lupus erythematous.
Based on separation at the dermo-epidermal junction:
Lamina Lucida
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Bullous pemphigoid.
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Cicatricial pemphigoid.
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Epidermolysis bullosa junctional.
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Dermatitis herpetiformis.
Sublamina Densa
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Epidermolysis bullosa acquisita.
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Epidermolysis bullosa dystrophic.
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Linear IgA dermatosis.
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Bullous systemic lupus erythematosus (SLE).
What Is Pemphigus Vulgaris?
Pemphigus vulgaris, the most common form of pemphigus, is an autoimmune disorder resulting in mucocutaneous lesions. The condition shows an annual incidence between 0.1 to 0.5 per 100,000 individuals. The condition shows a high incidence in Ashkenazi Jews between the ages of 40 and 80. In a few populations, women to men ratio stand at four to one.
Pemphigus vulgaris is caused due to the production of autoantigens against desmoglein 1 and 3 proteins. These are keratinocyte proteins present in the skin that helps in intracellular adhesion. Immunoglobulins attack these proteins leading to characteristic mucocutaneous lesions. The condition is diagnosed with direct and indirect immunofluorescence and ELISA (enzyme-linked immunosorbent assay), which can detect the circulating antibodies. A positive Nikolsky sign (blister formation even with minor pressure or trauma) is evident.
Pemphigus is treated by systemic corticosteroids, Azithropine, Rituximab, Ofatumumab, Intravenous immunoglobulin (IVIG), Cyclophosphamide, Dapsone, Methotrexate, and immunoadsorption.
What Is Pemphigoid?
Pemphigoid is a rare autoimmune disorder that causes blisters over the mucous membranes and cutaneous surfaces. The most common types of pemphigoid are bullous pemphigoid and cicatricial pemphigoid. The annual incidence of the condition was reported to be six to 13 for bullous and 1.6 for cicatricial pemphigoid per million individuals. The disease was usually in ages above 60. The bullous type shows no racial or gender predominance, whereas the cicatricial type showed a female predilection by a factor of 1.5 (in Greece) to 7 (in Germany).
Bullous type is caused by autoantibodies generated against keratinocyte hemidesmosomes (BP 180 and 230). When autoantibodies bind to these antigen proteins, it activates an inflammatory cascade that progressively damages the dermal-epidermal junction. The autoantibodies in cicatricial pemphigoid attack transmembrane components (BP 180 and laminin V), which forms mucocutaneous scars that are more superficial than the bullous type.
Pemphigoid is diagnosed with DIF (direct immunofluorescence), IIF (indirect immunofluorescence), and ELISA tests and treated with topical steroids, calcineurin inhibitors (Tacrolimus), systemic steroid therapy with Dapsone, artificial tears, immunosuppressants (Prednisone) and TNF_alpha inhibitors (Rituximab). Additionally, oral hygiene maintenance and prevention therapy for opportunistic infections are essential for a comprehensive treatment regimen.
What Is Epidermolysis Bullosae?
Epidermolysis bullosae is a rare, inherited disorder that manifests as epithelial and cutaneous blisters or erosions. It has several variants namely acquisita, simplex, junctional, and dystrophic, based on the level of the epidermal split. The condition is known as Kindler syndrome when a multiple-level split is seen. The prevalence was estimated at eight per million, and the incidence was 19 per million live births.
Epidermolysis bullosae is an autoimmune disorder with antibodies targeted against various antigens, namely type VII collagen, keratin 5 and 14, laminin-5 and XVII collagen, and type VII collagen, respectively, in various variants. The disease causes failure of keratinization and blistering with friction. The condition can be diagnosed by a punch biopsy, IF (immunofluorescence) mapping, and genetic testing.
What Is Erythema Multiforme?
Erythema multiforme is an autoimmune hypersensitivity reaction that precipitates mucocutaneous papular, bullous, and necrotic lesions. The condition has been reported in all ethnicities, mostly in young adults, out of which 20 percent of the cases occur in children with a male predilection by a factor of five. Erythema multiforme is caused by HSV (herpes simplex virus) 1 and 2, mycoplasma, and other viral, bacterial, and fungal infections, and reactions from penicillins, cephalosporins, macrolides, sulfonamides, anti tuberculoid agents, antipyretics, heavy metals, herbal agents, topical therapies and poison ivy that may trigger autoantibodies against desmoplakin proteins in intercellular desmosomes.
The disease is treated by antiseptics (both as mouthwash and topical), vitamin A ointment on the eyes, systemic corticosteroids, and intravenous immunoglobulins, Azithromycin, Aciclovir or Valaciclovir. Recurrences can be prevented by Hydroxychloroquine, Dapsone, and systemic corticosteroids.
What Is Linear IgA Disease?
Linear IgA disease is a rare autoimmune disorder characterized by linear deposits of IgA in the basement. It has two variants-childhood bullous type and adults linear type. The condition is caused due to autoantibodies targeted against several antigens within the dermal-epidermal junction. The autoimmunity may be triggered by infections, Penicillin, Vancomycin, Diclofenac, NSAIDs (non-steroidal anti-inflammatory drugs), Captopril, and lithium.
The characteristic vesicular or bullous lesions are seen in a clustered arrangement all over the body, giving an itchy and burning sensation. The disease is seen in around 0.8 to 1.3 cases per million individuals with no gender or racial predilection. It is diagnosed with a skin biopsy and DIF. The condition is treated by discontinuing the trigger element and applying topical corticosteroids, Erythromycin, Dapsone, and sulphonamides. Spontaneous remission occurs within three to six years.
What Is Dermatitis Herpetiformis?
Dermatitis herpetiformis is a chronic, autoimmune disease that causes itchy blisters primarily over the extensor sites. The lesions are grouped and rupture spontaneously and may heal without scarring.
Enamel pits and other dental abnormalities have also been reported. This is most commonly seen in individuals of European descent, with a prevalence between 1.2 to 39.2 and incidence between 0.4 to 2.6 per 100,000 annually, typically in the fourth decade of life with a gender ratio of 1.5 to 2: 1 (male predomination).
The condition is caused due to gluten and gliadin. Tissue transglutaminase is required for gliadin modification in the gut. Autoantibodies are directed against the tissue transglutaminase. The condition may even be triggered by iodine and tobacco. The condition is treated by a gluten-free diet, Dapsone, topical steroids, Sulphamethoxypyridazine, Sulphapyridine, and Sulphasalazine.
What Is Herpes Simplex?
Herpes simplex is an infectious vesiculobullous disease caused by herpes simplex virus types 1 and 2. Herpes is spread through direct contact with an active lesion or body fluid exchange. HSV-1 primarily causes oral, pharyngeal, facial, ocular, and neural infections. In contrast, HSV-2 causes anogenital infections. HSV can be diagnosed by viral culture, direct fluorescent antibody (DFA) test, biopsy, polymerase chain reaction (PCR), and serological test. The transmission can be prevented using barrier contraceptives and treated with antivirals (Acyclovir, Valaciclovir, Famciclovir, and Penciclovir) and topical antiviral ointments.
What Is Varicella Zoster ?
Varicella zoster is a virus known as human alphaherpesvirus-3 that causes chickenpox (in children and young adults) and shingles (in adults). Varicella zoster is a highly contagious disease found worldwide and can spread by inhaling infected aerosolized droplets. It is responsible for close to 7000 deaths annually worldwide and about 9000 hospitalizations in the US alone. The infection causes muscle pain, nausea, appetite loss, headache, rashes, oral sores, malaise, and fever. The rashes cover the entire body, progressing to blisters or pustules and, eventually, umbilication and scab formation. The treatment is symptomatic. Patients are advised not to scratch and apply calamine lotion over the body. Daily cleaning, antiviral medicines (Acyclovir or Valacyclovir) in adults, and intramuscular varicella-zoster immune globulin injection (in severe infections) can be used to treat varicella.
What Is Hand, Foot, and Mouth Disease?
Hand, foot, and mouth disease is a common viral disorder usually seen in children and can also affect adults. The causative organism for the infection has been identified as coxsackievirus stains. More than 90 percent of recorded cases were children below five years old, with a mortality rate of 0.03 percent. The infection causes low-grade fever, appetite loss, malaise, and throat pain secondary to enanthem (a rash on the mucous membrane). The extraoral rashes can be macular, papular, or vesicular, lasting for about ten days and then rupturing to form shallow, painless ulcers that heal without scarring.
The treatment is primarily supportive with NSAIDs and Acetaminophen prescribed against pain and fever, along with sufficient hydration, liquid Ibuprofen, and liquid Diphenhydramine. Vaccines are also available that have sown an average efficiency of 94.7 percent.
What Is Herpangina?
Herpangina is a viral infection caused by multiple enteroviruses and coxsackie viruses, mostly in children younger than ten. The viruses typically spread via the fecal-oral route or from ingestion of infected saliva, respiratory droplets, or direct contact with fluid from vesicles. The incubation lasts about five days, and viral shedding can last for three weeks via the respiratory route and eight weeks via stool. Patients are highly contagious in the first two weeks of infections.
The patients present with painful enanthem over the soft palate, tonsils, and posterior pharynx that eventually develop into vesicles and ulcerate. Other findings include pharyngitis (pharynx inflammation) and cervical lymphadenopathy (lymph node swelling). The patients should be isolated in clean and well-ventilated rooms to avoid cross-infection, and oral care must be taken. The patients should consume sufficient calories. Pharmacotherapy includes Ibuprofen, Acetaminophen (for fever), and interferon-alpha sprays for local drug administration. Children might suffer seizures for which intravenous Midazolam can be given.
What Is Measles?
Measles, also known as rubella, is a preventable, highly contagious viral infection caused due to the measles virus, which is an ss-RNA virus. The virus is responsible for over 100,000 annual global death despite the availability of an effective vaccine, down from 2.6 million deaths before the vaccine. Unvaccinated children and pregnant mothers are at high risk of infection.
The diagnosis is based on clinical suspicion when the children present with febrile illness and a maculopapular rash, and laboratory studies show leukopenia, thrombocytopenia, and electrolyte abnormalities. PCR results from samples collectorate from the throat, nasal, nasopharyngeal, and urine. There is no specific therapy for measles, and the management involves symptomatic treatment along with vitamin supplementation.
Conclusion:
Vesciculobullous lesions are autoimmune, inherited, or infectious mucocutaneous lesions that may be highly fatal in some cases. Almost all of the autoimmune conditions are detected by IDF, DIF, and ELISA, while the viral ones are detected with culture, serological studies, and PCR. Nikolsky test, Biopsy, Tzancky test, and LE cell (lupus erythematosus cells) are the essential microscopic studies. It is essential to visit the physician if symptoms develop and be up-to-date with the vaccines.
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