Vesiculobullous diseases of the skin encompass a wide range of entities, including autoimmune bullous diseases and inherited blistering disorders (see 28, 29, 30, 31, 32 ). This chapter focuses on the remaining non-infectious disorders in which cutaneous bullae develop and…
Synonyms ▪ All forms of EB: EB hereditaria ▪ EB simplex: epidermolytic EB ▪ Junctional EB: EB atrophicans, EB letalis ▪ Dystrophic EB: EB dystrophica Key features ▪ Epidermolysis bullosa (EB) encompasses multiple clinically distinctive disorders that share three major…
Key features ▪ Dermatitis herpetiformis (DH) is differentiated from linear IgA bullous dermatosis (LABD) on the basis of granular IgA versus linear IgA deposition at the basement membrane zone, as observed by direct immunofluorescence ▪ DH is a cutaneous manifestation…
Bullous Pemphigoid Synonym ▪ Pemphigoid Key features ▪ Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease, and its onset is often after 60 years of age ▪ It is usually a chronic disease, with spontaneous exacerbations and…
Synonyms ▪ Fogo selvagem: endemic pemphigus foliaceus; Brazilian pemphigus ▪ Pemphigus erythematosus: Senear–Usher syndrome ▪ IgA pemphigus: intercellular IgA dermatosis; intraepidermal neutrophilic IgA dermatosis; intercellular IgA vesiculopustular dermatosis ▪ Paraneoplastic pemphigus: paraneoplastic autoimmune multi-organ syndrome Key features ▪ Pemphigus is…
Synonyms ▪ Bullous pemphigoid antigen 1: BP230 ▪ Bullous pemphigoid antigen 2: BP180; type XVII collagen ▪ Laminin 311: laminin 6 ▪ Laminin 332: laminin 5; epiligrin; kalinin; nicein; GB3 antigen; BM600 ▪ Laminin 511: laminin 10 ▪ Type VII…
For decades, the specific dermatoses of pregnancy represented a confusing group of overlapping entities, understood largely through anecdotes and case reports. However, more recent reviews have simplified and condensed the list of pregnancy-specific dermatoses ( Table 27.1 ) . For…
The neutrophilic dermatoses constitute a heterogeneous but linked spectrum of diseases , with significant overlapping histopathologic findings and similar pathogenic mechanisms and therapeutic approaches. They are often associated with underlying internal diseases, which may have significant morbidity and mortality. Histologically,…
Key features ▪ Eosinophil infiltration of the skin is commonly observed and therefore clinicopathologic correlation is required to determine a correct diagnosis ▪ Eosinophil-associated dermatoses include arthropod bite reactions and drug eruptions (“bugs and drugs”), as well as parasitic infections,…
Key features ■ Cutaneous signs of vasculitis are a reflection of the size of the vessels involved ■ Vasculitis can be limited to the small vessels of the skin or it can be a sign of life-threatening internal organ involvement…