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…
Fig. 44.1 Direct immunofluorescence showing granular IgA deposits in the papillary dermis 44.2 Skin Symptoms The extremely pruritic, rather polymorphic chronic skin rash in DH is more easily recognized by…
Fig. 26.1 (a) Clinical appearance of SPD-type IgA pemphigus and (b) IEN-type IgA pemphigus 26.4 Histopathology Histopathologically, most IgA pemphigus skin lesions show minimal acantholysis [12]. In the SPD-type IgA…
Subtype Onset Clinical features Gene Inheritance IF TEM JEB, generalized severe Birth Lethal in childhood; extensive generalized blistering; dyspnea; anemia; failure to thrive; nail loss and abnormalities; enamel hypoplasia LAMB3/LAMC2/LAMA3…
(1) Department of Dermatology, Columbia University, New York, NY, USA (2) Department of Dermatology, Morgan Stanley Children’s Hospital of New York-Presbyterian/Columbia University, New York, NY, USA 34.1 Introduction 34.2…
Fig. 68.1 Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) (Adapted with permission from Nature Publishing Group: Rosenbach et al. [8]) 68.3 Pemphigus Disease Area Index (PDAI) The Pemphigus Disease Area…
Fig. 45.1 Symblepharon in a patient with OcMMP Fig. 45.2 Blepharitis in the lower eye lid margin The course of OcMMP is variable among patients. Some patients have limited disease…
Fig. 36.1 Generalized severe JEB presenting with generalized blistering and extensive areas of detachment of the epidermis Secondary lesions following chronic, repeated (even intrauterine) tissue traumatization include atrophic scarring, webbing…
