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Vesiculobullous diseases

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  • 1. CLASSIFICATION OF VESICULOBULLOUSCLASSIFICATION OF VESICULOBULLOUS DISEASESDISEASES VESICLE & BULLAVESICLE & BULLA A clear fluid lesion just below theA clear fluid lesion just below the epithelium which ruptures to form an ulcer,epithelium which ruptures to form an ulcer, if this is smaller than 5mm then it is aif this is smaller than 5mm then it is a vesicle ,if larger than 5mm than it is a bullavesicle ,if larger than 5mm than it is a bulla
  • 2. CLASSIFICATION OF VESICULOBULLOUSCLASSIFICATION OF VESICULOBULLOUS DISEASESDISEASES CLASSIFICATIONCLASSIFICATION INTRA EPITHELIAL VESICLESINTRA EPITHELIAL VESICLES: The lesion is formed: The lesion is formed within the epitheliumwithin the epithelium Acantholytic vesicles :Acantholytic vesicles : This is because of the breakThis is because of the break down of specialized attachments called thedown of specialized attachments called the desmosomesdesmosomes Nonacantholytic vesiclesNonacantholytic vesicles: It is usually in the viral: It is usually in the viral infections because of the death or the rupture of theinfections because of the death or the rupture of the group of cells.group of cells. SUB EPITHELIAL VESICLESSUB EPITHELIAL VESICLES: Lesions formed between the: Lesions formed between the epithelium and the lamina propria eg:epithelium and the lamina propria eg: Erthyma multifomeErthyma multifome PhempegoidPhempegoid Dermatitis herpetiformisDermatitis herpetiformis Epidermolysis bullosaEpidermolysis bullosa
  • 3. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS Autoimmune disease.Autoimmune disease. Common in Ashkenazi and Mediterranean jews .Common in Ashkenazi and Mediterranean jews . Middle aged females.Middle aged females. Other variants are:Other variants are: Pemphius vegetansPemphius vegetans Paraneoplastic pemphgusParaneoplastic pemphgus
  • 4. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS CLINICAL FEATURES:CLINICAL FEATURES: Painful ulcers or bulla are formed which are fluidPainful ulcers or bulla are formed which are fluid filled.filled. They can be formed any where in the oral cavity .They can be formed any where in the oral cavity . The bulla is rapidly ruptured leaving a collapsed roofThe bulla is rapidly ruptured leaving a collapsed roof of grayish membrane with a red ulcerated base.Theof grayish membrane with a red ulcerated base.The ulcer may look like an apthous ulcer or may be largeulcer may look like an apthous ulcer or may be large map shaped.map shaped. Nikolsky sign is positive.Nikolsky sign is positive.
  • 5. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS Sometimes the ulcers are joined together to make aSometimes the ulcers are joined together to make a confluence. this condition is very painful.confluence. this condition is very painful. It has a variable course might involve skin,It has a variable course might involve skin, oesophagus, cervix.oesophagus, cervix. Protein/fluid,electrolyte and weight loss /secondaryProtein/fluid,electrolyte and weight loss /secondary infections.infections. Fatal if untreated.Fatal if untreated.
  • 6. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
  • 7. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS PATHOGENESIS:PATHOGENESIS: It is an autoimmune diseaseIt is an autoimmune disease There are circulating antibodies of type IgG.There are circulating antibodies of type IgG. These antibodies are reactive against theThese antibodies are reactive against the desmosomes or the tonofilament complex.desmosomes or the tonofilament complex. There destruction or disruption of theseThere destruction or disruption of these tonofilament complex ,resulting in the loss oftonofilament complex ,resulting in the loss of attachment from cell to cellattachment from cell to cell path.cont…dpath.cont…d
  • 8. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS HISTOPATHOLOGY:HISTOPATHOLOGY: Intra epithelial vesicles or bulla and cleft like spacesIntra epithelial vesicles or bulla and cleft like spaces are produced by acantolysis .are produced by acantolysis . These changes are in the stratum spinosum or theThese changes are in the stratum spinosum or the prickle cell layerprickle cell layer Inflammatory cells are very scanty howeverInflammatory cells are very scanty however eosinophils may be seen.eosinophils may be seen. Acantholytic statum spinosum cells occur singly orAcantholytic statum spinosum cells occur singly or are in the forms of clumps lying freely within theare in the forms of clumps lying freely within the blister fluid. These cell loose there polyhedralblister fluid. These cell loose there polyhedral morphology rather they are small rounded andmorphology rather they are small rounded and contain hyper chromatic nuclei called the TZANKcontain hyper chromatic nuclei called the TZANK CELLS.CELLS.
  • 9. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS histologyhistology
  • 10. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS tzank cellstzank cells
  • 11. DIAGNOSISDIAGNOSIS Skin biopsySkin biopsy Electron microscopy has shown thatElectron microscopy has shown that widening of the intercellular space iswidening of the intercellular space is followed by splitting of the desmosomefollowed by splitting of the desmosome junctions.junctions. Direct & indirect immunofluorescenceDirect & indirect immunofluorescence ELISAELISA
  • 12. Direct immunofluorescenceDirect immunofluorescence
  • 13. Indirect immunofluorescenceIndirect immunofluorescence
  • 14. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS DIFFRENTIAL DIAGNOSIS:DIFFRENTIAL DIAGNOSIS: PemphegoidPemphegoid Erthema multiformeErthema multiforme Bullous lichen plannusBullous lichen plannus
  • 15. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS TREATMENT:TREATMENT: High mortality rates previouslyHigh mortality rates previously Introduction of systemic corticosteroidsIntroduction of systemic corticosteroids like prednisolone in stable cases.like prednisolone in stable cases. Prednisolone plus azathioprinePrednisolone plus azathioprine methotrexate and cyclophosphamide inmethotrexate and cyclophosphamide in progressed or advanced cases.progressed or advanced cases.
  • 16. PemphigusPemphigus foliaceusfoliaceus
  • 17. DefinitionDefinition: Blistering in this group of: Blistering in this group of autoimmune diseases is high in theautoimmune diseases is high in the epidermis, either in the granular layer orepidermis, either in the granular layer or just beneath the stratum corneum.just beneath the stratum corneum. Antibody binding may have a direct effectAntibody binding may have a direct effect on the function of the desmosomalon the function of the desmosomal cadherins in the upper epidermis, causingcadherins in the upper epidermis, causing detachment of keratinocytes.detachment of keratinocytes. Desmoglein-l is present but only weaklyDesmoglein-l is present but only weakly expressed in mucosae accounting for theexpressed in mucosae accounting for the lack of mucosal involvement inlack of mucosal involvement in pemphigus foliaceus.pemphigus foliaceus.
  • 18. Clinical featureClinical feature The onset is usually insidious with scattered, scalyThe onset is usually insidious with scattered, scaly lesions involving the 'seborrhoeic' areas: scalp,lesions involving the 'seborrhoeic' areas: scalp, face, chest and upper back. Blistering may not beface, chest and upper back. Blistering may not be obvious because the cleavage is superficial and theobvious because the cleavage is superficial and the small flaccid blisters rupture easily.small flaccid blisters rupture easily. Oral lesions are uncommon.Oral lesions are uncommon. Pemphigus foliaceus is generally regarded as aPemphigus foliaceus is generally regarded as a benign disease which responds well to treatmentbenign disease which responds well to treatment and may remit.and may remit.
  • 19. Pemphigus foliaceusPemphigus foliaceus
  • 20. Pemphigus foliaceusPemphigus foliaceus histologyhistology
  • 21. TREATMENTTREATMENT Potent topical or intralesional steroids or, ifPotent topical or intralesional steroids or, if control is inadequate, prednisolone 20-40control is inadequate, prednisolone 20-40 mg/ day.mg/ day. Azathioprine or cyclophosphamide areAzathioprine or cyclophosphamide are effective adjuncts to oral steroids in severeeffective adjuncts to oral steroids in severe cases. Hydroxychloroquine 200/mg twicecases. Hydroxychloroquine 200/mg twice per day has also been recommended asper day has also been recommended as adjuvant therapy. Intravenous Ig has beenadjuvant therapy. Intravenous Ig has been reported as effective in resistant cases.reported as effective in resistant cases.
  • 22. BULLOUS PEMPHIGOIDBULLOUS PEMPHIGOID Bullous pemphigoid is an affliction of elderlyBullous pemphigoid is an affliction of elderly people,with onset usually after 60 years ofpeople,with onset usually after 60 years of age.age. The blister in bullous pemphigoid isThe blister in bullous pemphigoid is subepidermal with an intact and often viablesubepidermal with an intact and often viable epidermis forming the roof.epidermis forming the roof. Bullous pemphigoid commonly starts withBullous pemphigoid commonly starts with itching and a non-specific rash on the limbsitching and a non-specific rash on the limbs that may be either urticaria-like orthat may be either urticaria-like or occasionally eczematous and rarely mayoccasionally eczematous and rarely may simulate vesicular eczema.simulate vesicular eczema.
  • 23. PEMPHGOIDPEMPHGOID Blisters may arise on erythematous and onBlisters may arise on erythematous and on normal skin and may be associated with dermalnormal skin and may be associated with dermal edema. The blisters are tense and domeedema. The blisters are tense and dome shaped, obtaining a diameter of manyshaped, obtaining a diameter of many centimeteres.centimeteres. The blisters are tough and may remain intact forThe blisters are tough and may remain intact for several days, the contents often becoming jelly-several days, the contents often becoming jelly- like with coagulated fibrin.like with coagulated fibrin. Mucosal lesions occur less frequently and areMucosal lesions occur less frequently and are less severe than in pemphigus vulgaris and areless severe than in pemphigus vulgaris and are usually confined to the mouth.usually confined to the mouth.
  • 24. BULLOUS PEMPHGOIDBULLOUS PEMPHGOID
  • 25. BULLOUS PEMPHIGOIDBULLOUS PEMPHIGOID HISTOLOGYHISTOLOGY
  • 26. PEMPHGOIDPEMPHGOID Untreated bullous pemphigoid runs aUntreated bullous pemphigoid runs a chronic, self limiting course over a numberchronic, self limiting course over a number of months or years.of months or years. The disease duration is usually 3-6 years,The disease duration is usually 3-6 years, with most patients achieving completewith most patients achieving complete remission off treatment.remission off treatment.
  • 27. TREATMENTTREATMENT Topical and systemic steroids are theTopical and systemic steroids are the mainstay of treatment. For localized BP,mainstay of treatment. For localized BP, very potent topical steroids are oftenvery potent topical steroids are often sufficient.sufficient. Corticosteroid therapy has lowered theCorticosteroid therapy has lowered the morbidity from the disease considerablymorbidity from the disease considerably and most patients achieve remission off alland most patients achieve remission off all therapy, but significant mortality of bulloustherapy, but significant mortality of bullous pemphigoid still remains at 15-40%, and ispemphigoid still remains at 15-40%, and is nearly always treatment related or relatednearly always treatment related or related to the general condition and age of theto the general condition and age of the patients.patients.
  • 28. DERMATITIS HERPETIFORMIS Definition. Dermatitis herpetiformis (DH) is a rare, intensely pruritic,chronic, recurrent, papulovesicular disease.There is an underlying gluten-sensitive enteropathy that may be asymptomatic. The mechanism by which ingestion of gluten induces granular IgA deposition in the skin and blistering is still obscure. There is a family history of dermatitis herpetiformis or coeliac disease in 10.5% of patients and it has been reported to be both concordant and discordant in monozygotic twins.
  • 29. PATHOGENESIS The IgA deposits are gluten dependent, and are slowly cleared from the skin once gluten is removed from the diet. The Ag within normal human skin to which IgA antibodies from DH sera bind is still unknown. One of the most exciting developments of recent years has been the recognition that autoantibodies and T-cell reactions to tissue transglutaminases, and in particular transglutaminase 2, are relevant to the pathogenesis of coeliac disease .These antibodies have been demonstrated in dermatitis herpetiformis.In addition, it is now clear that the previously recognized antireticulin and endomysial antibodies, in coeliac disease and dermatitis herpetiformis, are associated with these antibodies and require transglutaminase 2 to bind to tissues.
  • 30. PATHOLOGY Diagnostic histological changes are best seen in the vicinity of early blisters or in lesions that have not yet blistered.Neutrophils and eosinophils accumulate within the dermal papillae and form microabscesses. The surrounding collagen is degraded, resulting in detachment of the epidermis and a subepidermal vesicle. Multilocular vesicles may coalesce to form blisters; Direct immunofluorescence is always positive. There are granular deposits of IgA in the dermal papillae There may also be C3 and IgG.
  • 31. DERMATITIS HERPETIFIRMIS HISTHOLOGY
  • 32. CLINICAL FEATURES Dermatitis herpetiformis presents mainly between the ages of 20 and 55 years, but can present both in childhood and old age. The onset may be acute or gradual, and pruritus is usually the first and predominant symptom. Early lesions on the skin are erythematous papules, urticarial weals or groups of small vesicles often excoriated so rapidly that it may be impossible to find one intact. The vesicles are usually grouped together on plaques of erythema, and rarely blisters 1-2 cm in diameter occur. The distribution of the lesions is characteristic. The extensor aspects of the limbs, especially the knees, just below the point of the elbows, buttocks and the natal cleft, are affected in the majority of patients The axillary folds, shoulders, trunk, face and scalp are all frequently involved.
  • 33. There may be a feeling of malaise with the acutely active disease. In addition, constitutional symptoms due to the glutensensitive enteropathy can be present. The patient may experience bouts of abdominal pain, constipation and diarrhoea, and be undernourished. Associated diseases. There are often associated autoimmune diseases, particularly thyroid disease, pernicious anaemia and diabetes.There is an association with thyroid disease in up to 30% of patients. Lymphoma is a well-recognized complication of dermatitis herpetiformis, as are other malignancies although a recent study contradicts this . Moreover, the protective role of a gluten-free diet for the lymphomas has been established.
  • 34. Differential diagnosis The diagnosis should be suspected when any persistent, pruritic, symmetrical eruption resists topical treatment. In view of the pruritus and involvement of the axillary folds and buttocks, many patients are thought to have scabies, but the absence of burrows or of contact cases should help with the diagnosis. The most difficult diagnostic problem is the group of patients with chronic exudative eczema, papular urticaria and chronic prurigo, some of whom may be dapsone responsive. The histology and the lack of IgA deposition should help establish the correct diagnosis.
  • 35. TREATMENT Dapsone is the most widely used treatment for dermatitis herpetiformis. The dose needed for the average case is 100-200 mg/ day but a few may require 400 mg/ day. Patients at risk of glucose-6-phosphate dehydrogenase deficiency should be screened prior to treatment. Methaemoglobinaemia is common, reaching a steady state after about 2 weeks, and may cause cyanosis, breathlessness and angina. Hepatitis, the dapsone syndrome (lymphadenopathy and hepatitis) and agranulocytosis are serious, usually early complications. Motor neuropathy may occur. Although systemic corticosteroids are in the main ineffective and not indicated, topical steroids may be helpful in lessening symptoms.
  • 36. A gluten-free diet is the treatment of choice in the long term. It has been shown not only to improve the enteropathy, but also to allow discontinuation of drug therapy. It is usually many months and sometimes years before patients are able to reduce their dapsone requirements. Often dapsone can be discontinued altogether after 2-3 years on a strict gluten-free diet, but some patients take much longer . Reintroduction of gluten in selected patients produced a relapse in skin lesions .The gluten-free diet after 5-10 years protects patients from lymphoma, and this is an additional reason to recommend a gluten-free diet.