Dystrophic Epidermolysis Bullosa
Mode of inheritance Gene Target protein AD = autosomal dominant AR = autosomal recessive Dominant DEB Dominant DEB, generalized AD COL7A1 Type VII collagen Dominant DEB, acral AD, AR…
Mode of inheritance Gene Target protein AD = autosomal dominant AR = autosomal recessive Dominant DEB Dominant DEB, generalized AD COL7A1 Type VII collagen Dominant DEB, acral AD, AR…
Fig. 24.1 Oral mucosa involvement in pemphigus vulgaris. Erosions on the tongue Fig. 24.2 Oral mucosa involvement in pemphigus vulgaris. Erosions on the lip Fig. 24.3 Oral mucosa involvement in…
Form of EB Lin et al. [6] Tong et al. [7] Simplex EB 3 % 12 % Junctional EB 39 % 40 % Recessive dystrophic EB 51 % 51 %…
Acquired immunobullous diseases (AIBD) Antigen mapping (AgM) 1. Level of split formation Intraepidermal, junctional, dermolytic Autoantibody mapping (AbM) = salt split skin test (SSST) 2. Location of binding of autoantibodies…
Fig. 31.1 Classical bullous pemphigoid with tense bullae on the limb surrounded by erythema with infiltrated confluent urticarial plaques. Vesicles and eroded and crusted lesions are also observed Fig. 31.2…
Vancomycin – most common Captopril Trimethoprim/sulfamethoxazole Phenytoin Diclofenac The most common disease associated with LABD is ulcerative colitis. The relationship is unclear, and abnormal IgA1 production in the gastrointestinal tract…
Fig. 30.1 Palmoplantar keratoderma in EBS Fig. 30.2 Tense blistering, hyperkeratosis and thickened nails in a boy with EBS gen-se Fig. 30.3 Koebnerization in EBS In localised EBS, blistering is…
Fig. 17.1 Molecular constituents of desmosomes Keratinocytes adhere to basement membrane mainly by hemidesmosomes. The core of the hemidesmosome consists of four transmembrane proteins: BP180/type XVII collagen/BPAG2, α[alpha]6 and β[beta]4…
Fig. 1.1 Biochemical composition of dermal-epidermal basement membrane ultrastructural entities. Ultrastructural entities viewed by transmission electron microscopy (above) compared with a schematic (below) revealing their biochemical composition Newer techniques have…
2.5 ml 1 M potassium citrate 5 ml 0.1 M magnesium sulphate 5 ml 0.1 M N-ethylmaleimide to 87.5 ml distilled water Adjust the final mixture to Ph 7 with…