main treatment oral prednisolone main treatment oral prednisolone high-dose regimen a rapid initial control of disease, but this regimen did not have any long- term benefit over the moderate-dose with respect to the relapse or the complications
Rituximab, a monoclonal anti-CD20 an tibody. It is hypothesized,, agent depletes B-lymphocytes and removes desmoglein antibodies from the circulation. a promising therapeutic option for refractory immunobullous diseases A new therap. / agent Rituximab
Pemphigus and Biologicals … Weekly iv infusion Rituximab 375mg/m2 is very promising in PV recalcitrant recalcitrant cases of PV show also a good response to etanercept 50mg/twice weekly s.c. up to 6 weeks..
IV Ig in pemphigus 2g/kg/ cycle (given 3-5 days),,for lowering side effects pre - hydration and slow rate of infusion required Side effects: aseptic meningitis, thromboemboli,
D+C+P therapy in PV : 136mg/ day Dexamethasone in 500 cc Dextrose 5% slow IV drip in 2 hours for 3 consecutive days and, on second day patient receives 500 mg Cyclophosphamide in same drip and from the first day counted 28 days for next session. Between pulses patient receives 50 mg/day cyclphos.. When patient is lesion –free,, he receives 6 more pulses.
It is advisable patients who are on cyclophosphamide / prophylaxis against pneumocystitis carinii pneumonia
A 34 yr old female from 3 months ago complains of intense pruritic papulovesicular lesions especially in exterimities and lower trunk Case 2.
DH: Rare, chronic, papulo- vesicular disease characterized by an intensely pruritic eruption consisting of various combinations of symmetrical, erythematous, papular, vesicular, or bullous lesions. The disease is strongly associated with the presence of HLA-DQ2 antigens. Buttocks are a common site of lesions in DH
pruritic excoriated papulovesicular rash on those parts from several months ago,
in DH anti-endomysial antibodies(IgA) auto Ag : epidermal transglutaminase
Herpetiform grouping of lesions in Dermatitis herpetiformis(DH)
In Dermatitis Herpetiformis an increased risk for autoimmune diseases and lymphoma,
Gluten free diet and dapsone effective for skin eruption DH In DH : many patients can achieve total resolution of skin disease by diet alone,
DH treatment Dapsone (initial dosage of 0.5 -2 mg/kg/day) can provide dramatic relief of pruritic symptoms, typically within 24 to 48 hours after beginning therapy. Baseline CBC (G6PD) level should be checked prior to this medication, weekly CBC till first month, and. monthly checks continued for the next 5 months.
Case.no.3 A 4 yr old boy with recent, generalised eruption somewhat but, not severely itchy
IgA B. D. of Childhood Linear IgA B. D. of Childhood
LABD Both in adults and children annular and circinate plaques can seen
Case No.4 A 74-year-old man with Alzheimer was referred for a pruriginous and bullous eruption that had developed four weeks after a boiling water burn on his abdomen The lesions consisted of tense bullae developing at the site of the burn initially, Subsequently, the lesions spread to the arms and legs and there were no mucosal lesions.
Bullous pemphigoid Bullous pemphigoid The condition is not as serious as Pemphigus V. ansd there is no racial predisposition. oral lesion may occur only in minority of patients other mucosae are not involved
Painful erosions in this middle age lady from a few months no mucosal lesions,otherwise healthy,drug responsible for LABD,Captopril
a 55-year-old man who, after a 6-month history of enlargement of cervical lymph nodes, presented with multiple painful ulcerations of the oral mucosa and lips and multiple skin erosions on the trunk, extremities,. A lymph node biopsy was revealed B-cell non- Hodgkin lymphoma. Case no.6
shows the blister within the epidermis, with the basal layer on the floor of the blister and the rest of the epidermis making up the roof of the blister. There are some inflammatory cells in the blister, mainly eosinophils with a few lymphocytes.
IIF of rodent bladder as a convenient and cost- effective method of screening for this syndrome, since bladder epithelium has numerous desmosomes, but the antigens of pemphigus vulgaris and pemphigus foliaceus are not expressed in this tissue but the antigens of pemphigus vulgaris and pemphigus foliaceus are not expressed in this tissue
A case with bronchiolitis oblitrans and conjunctival and oral erosions with PNP she was died due to Bronchiol/oblit/ A case with bronchiolitis oblitrans and conjunctival and oral erosions with PNP she was died due to Bronchiol/oblit/.
Another severly ill patient with SJS like symptoms and pasinful paronychia with Lymphoma/Assoc./ PNP
Some times targetoid or lichenoid lesions in in Paraneoplastic pemphigus