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6-year-old girl, with a 3-month history of a pruritic eruption, which began on the trunk and then gradually extended to the face and extremities. examination showed vesicules and bullae on annular, polycyclic, and erythematous plaques, leaving residual hyperpigmentation No oral mucosal involvement was present.The remaining physical examination was otherwise normal Case 7.
Direct immunofluorescence showed positive intercellular IgG and C3 staining throughout the epidermis and on the dermo-epidermal junction The child was diagnosed as/ ??
Pemphigusherpetiformis • clinical features of dermatitis herpetiformis with the immunopathologic features of pemphigus • IgG ,+_ IgA , intercelluar without mucous membrane involvement and a negative nikolsky sign • that respond good to Dapsone Or azathioprine + corticosteroids..
Managementof Pemphigus .Herpetif./ • dapsone at an initial dose of 2 mg/kg/day. continued to be maintained (up to 24 months after the initial diagnosis). plus prednisone that was reduced gradually then stopped after 3 months of treatment.
Case :no 8.An elderly female (72 year old)with recent onset of eye disease and blurred vision and oral erosion and poor feeding ,,pain in mucosae
Pathologically: Conjunctival biopsy for the separation of the epithelium from the inflammedsubstantiapropria.
Ocular form LABD resembles C.P/ but immunodeposits differ from point of view of DIF. C.P LABD
Case 9:A pregnant lady(second trimester) DIF ,,linear deposits along BMZ of/IgG
Herpes G./It begins usually in second or third trimester and resembles clinically and histologically to BP.
In herpes G./autoantibodies against two hemidesmosomal antigens: BPAG1 and BPAG2. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence tests. The most discriminant examination for other pruritic dermatoses of pregnancy is the enzyme-linked immunosorbent assay (Elisa) NC16A BP 180.First-line treatment is local corticosteroid therapy; if local treatment fails, general corticosteroid therapy should be adminstered.
herpes gestationis:interaction of IgG1 auto-antibodies and the 180 kD BPA2 remains paramount in understanding the immunologic mechanism of disease.
Case 10:6 month history of pruritic urticarial plaques and vesicles that started on her trunk and became more generalized. She developed occasional vesicles in her mouth, but examination failed to reveal any mucous membrane lesions. except for intermittent pruritus, her activity was normal. A skin biopsy showed a subepidermal blister with a mixed dermal inflammatory infiltrate and linear deposition IgG at the dermal-epidermal junction. Salt split skin showed staining on the bottom of the blister
EBA :autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis.
EBA:A Form of epidermolysisbullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. Direct immunofluorescence shows IgG deposited at the dermo-epidermal junction.
a middle age adult female with recent Hx: of/ Seborrheic like lesions unresponsive to antifungal creams and washes Case no.11
P.ErythematousSeborrheic areas are often involved DIF both reminding of LE and P.Foliaceus andANA often+P.erythematous is more localized than P.foliaceus./
A case of Pemphigus.FOliaceus. pruritic, burning, or painful dermatitis, most often in a seborrheic distribution, small flaccid intraepidermal bullae and erosions. Exacerbation by sun exposure.
Intensely itchy papulovesicles exacerbated with iodides Quizz: no.1
Quizz,no.3malar rash with erythema and scaling,DIF and ANA +
Quizz.no.4PAINFUL EROSIONS and painnful paronychia,this disease in common in middle age and Jews
Quizz No.5A 54 -yr -old female with recalcitrant cheilitis and stomatitis?
A rare variant of immunobullous dis. predominance of facial and intertriginous lesions, heal with verrucoid granulations. Two types exist Quizz. No 6
quizz,no.7A middle age lady with 9 month history of vesicular , eroded papular lesions& milia of hands and feet , no other areas involved
quizz.no.8a 76 yr –old- lady with recent bulla and itching over exterimities and trunk her feet are red and edematous with studded bullae
quizz no.9Bp The mechanism involves autoantibodies directed against antigens in the lamina lucida and hemidesmosomes of basal keratinocytes, ….. and ……. ……belongs to the plakin family and is restricted to the intracellular hemidesmosomal plaque, whereas the ……. is a transmembrane glycoprotein with intracellular and extracellular interactions with hemidesmosomes. The most common immunoglobulin overexpression is of …, …., ….. and less frequently of …..types.
Quizz.no.10Bullous Pemphigoid:alternative treatment options such as ….. and ………., this combination anti-inflammatory regime is only useful in those with limited or localized disease e.g. acralbullouspemphigoid
Intensely itchy papulovesicles Quizz: no.1
Quizz,no.3malar rash with erythema and scaling,DIF and ANA +
Quizz.no.4PAINFUL EROSIONS and painnful paronychia,this disease in common in middle age and Jews
Quizz No.5A 54 -yr -old female with recalcitrant cheilitis and stomatitis?
A rare variant of immunobullous dis. predominance of facial and intertriginous lesions, heal with verrucoid granulations. Two types exist Quizz. No 6
quizz,no.7A middle age lady with 9 month history of vesicular , eroded papular lesions& milia of hands and feet , no other areas involved
quizz.no.8a 76 yr –old- lady with recent bulla and itching over exterimities and trunk her feet are red and edematous with studded bullae
quizz no.9Bp The mechanism involves autoantibodies directed against antigens in the lamina lucida and hemidesmosomes of basal keratinocytes, ….. and ……. ……belongs to the plakin family and is restricted to the intracellular hemidesmosomal plaque, whereas the ……. is a transmembrane glycoprotein with intracellular and extracellular interactions with hemidesmosomes. The most common immunoglobulin overexpression is of …, …., ….. and less frequently of …..types.
