Pemphigus vulgaris is a blistering autoimmune skin lesion mostly seen in elderly patients.The disorder is characterised by a process called Acanthoysis (loss of cohesion between epidermal cells).Lesions are mostly flaccid blisters over the skin.These blisters burst out and the surounding skin gets denuded.In severe cases, substantial portions of the body get denuded.Manual pressue over these blisters can cause separation of epidermis.This is known as Nikolsky's sign.This finding is a characteristic feature of Pemphigus vulgaris but not the specific feature. Nikolsky's sign is also seen in Stevens Johnson Syndrome, Toxic epidermal necrolysis and few other skin conditions.
Clinical Features:
Sometimes the disorder starts with pruritis(itching).Lesions in Pemphigus vulgaris are usually seen over the oral mucosa, scalp, axilla, trunk, face, neck.Blisters are usually seen in the mouth first and then on other mucosa (pharyngeal, laryngeal, oesophageal, conjunctival, vulval or rectal) in severe cases.Severe pain may be seen associated with extensive denudation of body surface area. Lesions usually heal without scarring except at sites complicated by secondary infections or with mechanical dermal wounds.Post inflammatory hyper pigmentation is usaually seen at sites of healed lesions for some time.
Diagnosis:
On biopsy, the lesions show acantholytic epidermal cells and intra epithelial vesicles.Basal keratocytes are attached to the basement membrane, hence blister formation takes place in suprabasal portion of epidermis.Eosinophil predominant leukocytosis is seen in these regions.
Direct immuofluoresence microscopy shows - IgG antibodies both in the lesional and normal skin.But in the lesional skin, there are compliment components which are absent in the normal skin.These IgGs are derived from autoantibodies directed against cell surface antigens on the epidermal cells.These autoantibodies are targeted against desmogleins (Dsgs, these are transmembrane desmosomal glycoprotiens that belong to cadherin supergene family of calcium dependent adhesion molecules).These autoantibodies can be demonstrated by indirect imunofluoresence microscopy or by ELISA.Anti Dsgs titers relate with the activity of the disease.Mostly anti Dsgs3 is seen in these patients but in severe cases, antiDsgs3 and anti Dsgs1 autoantibodies are seen.Anti Dsg antibodies are pathogenic for blister formation and their titers correspond with the disease activity.
Treatment:
Prior to availability of Glucocorticoids, the mortality rate was 60 to 90%. Now it has been reduced to 5%.Prednisolone 60 to 80 mg/day is given. If new blister appear after 1 or 2 weeks of treatment, the dose must be increased. Immunosuppressants are given along with glucocorticoids if needed. Azathioprine (1 to 2 mg/day), Cycliphosphamine (1 to 2 mg/day), Mycophenolate mofetil (20 to 35 mg/day).
Clinical Features:
Sometimes the disorder starts with pruritis(itching).Lesions in Pemphigus vulgaris are usually seen over the oral mucosa, scalp, axilla, trunk, face, neck.Blisters are usually seen in the mouth first and then on other mucosa (pharyngeal, laryngeal, oesophageal, conjunctival, vulval or rectal) in severe cases.Severe pain may be seen associated with extensive denudation of body surface area. Lesions usually heal without scarring except at sites complicated by secondary infections or with mechanical dermal wounds.Post inflammatory hyper pigmentation is usaually seen at sites of healed lesions for some time.
Diagnosis:
On biopsy, the lesions show acantholytic epidermal cells and intra epithelial vesicles.Basal keratocytes are attached to the basement membrane, hence blister formation takes place in suprabasal portion of epidermis.Eosinophil predominant leukocytosis is seen in these regions.
Direct immuofluoresence microscopy shows - IgG antibodies both in the lesional and normal skin.But in the lesional skin, there are compliment components which are absent in the normal skin.These IgGs are derived from autoantibodies directed against cell surface antigens on the epidermal cells.These autoantibodies are targeted against desmogleins (Dsgs, these are transmembrane desmosomal glycoprotiens that belong to cadherin supergene family of calcium dependent adhesion molecules).These autoantibodies can be demonstrated by indirect imunofluoresence microscopy or by ELISA.Anti Dsgs titers relate with the activity of the disease.Mostly anti Dsgs3 is seen in these patients but in severe cases, antiDsgs3 and anti Dsgs1 autoantibodies are seen.Anti Dsg antibodies are pathogenic for blister formation and their titers correspond with the disease activity.
Treatment:
Prior to availability of Glucocorticoids, the mortality rate was 60 to 90%. Now it has been reduced to 5%.Prednisolone 60 to 80 mg/day is given. If new blister appear after 1 or 2 weeks of treatment, the dose must be increased. Immunosuppressants are given along with glucocorticoids if needed. Azathioprine (1 to 2 mg/day), Cycliphosphamine (1 to 2 mg/day), Mycophenolate mofetil (20 to 35 mg/day).
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