Dermatitis Herpetiformis
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…
IgA Pemphigus
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…
Junctional Epidermolysis Bullosa, Generalized Intermediate Type
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…
Clinical Features and Pathogenesis of Chronic Bullous Disease of Childhood
(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…
Extent Measures in Autoimmune Bullous Disease
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…
Ocular Involvement in Autoimmune Blistering Skin Diseases
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…
Generalized Severe Junctional Epidermolysis Bullosa
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…
Prevention and Treatment of Infections in Patients with Autoimmune Blistering Disease
Fig. 65.1 Patient with pemphigus vulgaris treated with rituximab and intravenous corticosteroids. Superficial swabs revealed the presence of Proteus mirabilis, Pseudomonas aeruginosa, and Candida parapsilosis Fig. 65.2 Patient with pemphigus…
Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis
Category Consensus description Proposed classification of lesions Bullous EM Detachment <10 % body surface area (BSA) Raised typical targets + Typical targets or raised atypical targets Raised atypical targets SJS…
Monitoring to Prevent Complications: Anemia, Infections, Osteopenia, Failure to Thrive, Renal Disease, Squamous Cell Carcinoma, Cardiomyopathy
Investigations Subtype Frequency Urine dipstick All types Each clinic visit Urine culture All types If dipstick (+) Urea, creatinine, electrolytes (Na, K), liver function tests (total bilirubin, albumin, alkaline phosphatase,…
