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Vesiculobullous Disorders

»What is the difference between a vesicle and a bulla?
»How are the bullous diseases defined?
»What things cause vesicles and bullae?
»How do you approach a patient who presents with an acute onset of a vesiculobullous eruption?
»Which skin findings are helpful in evaluating a patient with blisters?
»Do particular vesiculobullous diseases occur in characteristic distributions?
»Which tests are most useful in evaluating vesiculobullous diseases?
»How should a skin biopsy of a vesiculobullous eruption be performed?
»When are special tests necessary to diagnose blistering diseases of the skin?
»How are specimens obtained for direct immunofluorescence?
»For which vesiculobullous diseases are indirect immunofluorescence helpful?
»List the most common blistering diseases due to external agents.
»Name examples of drugs that can cause vesiculobullous eruptions.
»What is epidermolysis bullosa?
»Describe the other genetic blistering diseases.
»List the vesiculobullous diseases caused by metabolic disorders.
»Describe the clinical findings in bullous diabeticorum.
»What is the cause of pellagra?
»What is the difference between porphyria cutanea tarda and pseudoporphyria?
»What are the necrolytic erythemas?
»What is the difference between bullous pemphigoid and cicatricial pemphigoid?
»How do pemphigus vulgaris and pemphigus foliaceus differ?
»Linear IgA bullous dermatosis occurs in two different clinical situations. What are they?
»Describe the clinical findings in dermatitis herpetiformis.
»Does herpes gestationis have anything to do with herpes viruses?
»What is bullous systemic lupus erythematosus?
»What is epidermolysis bullosa acquisita?

 
 
 

What is the difference between bullous pemphigoid and cicatricial pemphigoid?


Bullous pemphigoid. Erythematous, urticarial plaques with multiple vesicles and bullae are seen. Many of the blisters are tense.
Fig. 10.4 Bullous pemphigoid. Erythematous, urticarial plaques with multiple vesicles and bullae are seen. Many of the blisters are tense.
Bullous pemphigoid (IgG directed against BP180 and BP230) is a chronic autoimmune bullous disease that most commonly affects older adults. The primary lesions may be urticarial plaques or tense bullae (Fig. 10-4). Lesions occur particularly on the flexural surfaces but may be widespread. Blisters form crusts and may heal with pigmentary changes, but not scarring. The oral mucosa is sometimes affected, but lesions in this area are usually minor. Cicatricial pemphigoid (IgG or IgA directed against multiple antigens at the basement membrane zone, including BP180) is a chronic autoimmune blistering disorder associated with scarring of mucosal surfaces. It primarily affects the elderly, with some patients having blisters on the cutaneous surface. In both diseases, the diagnosis is established by correlating the clinical findings with routine histologic examination of lesional skin, direct immunofluorescence of perilesional skin, and indirect immunofluorescence or ELISA testing of serum.

Zenzo G, Marazza G, Borradori L: Bullous pemphigoid: physiopathology, clinical features and management, Adv Dermatol 23:257–288, 2007.