Pemphigus groups

Classification Pemphigus vulgaris : Pemphigus vulgaris : اتوایمون (IgG) اتوایمون (IgG) مصرف دارو (captopril-penycillin) مصرف دارو (captopril-penycillin) میاستنی گراو میاستنی گراو فعال شدن سیستم ایمنی علیه اپیتلیال پوست فعال شدن سیستم ایمنی علیه اپیتلیال پوست

Age Age years years Rare in children Rare in children Gender Gender Occurs in equal numbers in men and women. Occurs in equal numbers in men and women. Race Race More common in Ashkenazi Jews and in people of Mediterranean decent. More common in Ashkenazi Jews and in people of Mediterranean decent. Genetics Genetics Associated with HLA DR4, DR6, and A10. Associated with HLA DR4, DR6, and A10.

Symptom تاول دردناک در دهان شروع میشود + کاهش وزن تاول دردناک در دهان شروع میشود + کاهش وزن تاول در پوست و مخاط ها ایجاد میشود بخصوص جاییکه تحت تروما یا فشارباشد تاول در پوست و مخاط ها ایجاد میشود بخصوص جاییکه تحت تروما یا فشارباشد تب تب عدم خارش عدم خارش بوی بد ترشحات و تاولها بوی بد ترشحات و تاولها

Signs Skin or mucosae can just shear off leaving widespread painful erosions Skin or mucosae can just shear off leaving widespread painful erosions Nikolsky's sign – جداشدن لایه های پوست ازهم ( در اثر فشار دادن ) Nikolsky's sign – جداشدن لایه های پوست ازهم ( در اثر فشار دادن )

Flaccid bullae of various sizes Flaccid bullae of various sizes Bullae arise from 'healthy' skin, rupture, and leave crusting and denuded skin Bullae arise from 'healthy' skin, rupture, and leave crusting and denuded skin Any area of stratified epithelium can be affected Any area of stratified epithelium can be affected

Signs Lesions often appear first in the mouth prior to skin involvement Lesions often appear first in the mouth prior to skin involvement Extent of skin and mucosal involvement varies Extent of skin and mucosal involvement varies Eyes, nasal mucosa, and genitalia may be affected Eyes, nasal mucosa, and genitalia may be affected Lesions may occur in oropharynx and upper esophagus Lesions may occur in oropharynx and upper esophagus Pyrexia and systemic features common Pyrexia and systemic features common Secondary infection is common Secondary infection is common

Skin biopsy Acantholysis and intraepidermal separation of keratinocytes, is likely to have pemphigus vulgaris. Acantholysis and intraepidermal separation of keratinocytes, is likely to have pemphigus vulgaris. Diagnosis is confirmed by direct or indirect immunofluorescence. Diagnosis is confirmed by direct or indirect immunofluorescence.

Don't miss! Pemphigus vulgaris should be suspected in any bullous or chronic mucosal ulceration Pemphigus vulgaris should be suspected in any bullous or chronic mucosal ulceration Widespread skin involvement with erosions and bullae requires urgent hospitalization and systemic treatment as morbidity and mortality are high Widespread skin involvement with erosions and bullae requires urgent hospitalization and systemic treatment as morbidity and mortality are high

Don't miss! Severe oral ulceration spreading past the vermilion border should raise suspicion of paraneoplastic pemphigus Severe oral ulceration spreading past the vermilion border should raise suspicion of paraneoplastic pemphigus

Tzanck test Acantholytic cells Acantholytic cells Cause of abnormal result Cause of abnormal result Pemphigus vulgaris Pemphigus vulgaris Other bullous skin diseases Other bullous skin diseases

عوارض جانبی عفونت باکتریال ثانویه عفونت باکتریال ثانویه عدم تعادل اب والکترولت و پروتئین عدم تعادل اب والکترولت و پروتئین درگیری سطح وسیع پوست Alb درگیری سطح وسیع پوست Alb

اهداف درمان Prevent new lesions forming Prevent new lesions forming Prevent scarring Prevent scarring Prevent secondary infections Prevent secondary infections Minimize effects of disease on patient's lifestyle Minimize effects of disease on patient's lifestyle

درمان کنترل بیمار ی : جلوگیری از تاول : کورتیکواستروئید کنترل بیمار ی : جلوگیری از تاول : کورتیکواستروئید سرکوب کننده ایمنی ( ازاتیوپرین - سیکلوفسفاماید - طلا ) سرکوب کننده ایمنی ( ازاتیوپرین - سیکلوفسفاماید - طلا ) پلاسمافرز موقت سطح Ab پلاسمافرز موقت سطح Ab

Treatment Topical steroid Topical steroid Systemic steroid Systemic steroid Methotrexate Methotrexate Azathioprine Azathioprine Cyclophosphamide Cyclophosphamide Dapsone Dapsone IVIG IVIG Plasmapheresis Plasmapheresis