Pemphigus vulgaris Pemphigus vulgaris is a rare autoimmune, bullous disease that occasionally occurs during childhood. The disease affects both the skin and mucous membranes and can be life threatening. The typical lesions of pemphigus vulgaris are pictured in Fig. 17-1. Erosions of the lips, gums, tongue, and palate, as pictured here, are a common presenting symptom and may be misdiagnosed early in the course of the disease. The difficulty in chewing and swallowing that may occur can become a significant complication.
Cutaneous lesions consist of flaccid weeping blisters that quickly erode to leave large denuded areas of skin. Nikolsky sign, the extension of blistering by lateral finger pressure, is seen in the presence of widespread disease. Figure 17-2 shows the kind of crusting that develops as the blisters open. Antibodies to desmoglein 1 are associated with skin lesions and antibodies to desmoglein 3 are associated with oral lesions.
Pemphigus vulgaris The blisters of pemphigus vulgaris may arise on an erythematous base, or on normal-appearing skin, as pictured in Fig. 17-3. A variety of modalities have been employed in the treatment of this disease. The patient who is seriously ill requires hospitalization.
Figure 17-4 illustrates both intact blisters and superficial erosions. For most patients, the most rapidly effective treatment remains high-dose systemic steroids. Patients undergoing this form of therapy are at risk for infection and must be followed with extreme care. Rituximab, a monoclonal antibody against the B cell surface protein CD20, is the most promising new treatment for this disease. Immunosuppressive agents such as azathioprine, mycophenolate mofetil, intravenous immunoglobulin, and plasmapheresis are useful therapies.
Pemphigus vegetans When the cutaneous changes of pemphigus take place in intertriginous spaces, clear blistering is not evident. Rather, one sees boggy inflammation. The essential histologic process is again epidermal acantholysis, but blister roofs part almost at once and secondary infection is inevitable. This figure is a good representation of the kind of clinical appearance that develops in pemphigus vegetans. Lesions on other parts of the body take the form of pemphigus vulgaris.
Familial Benign Chronic Pemphigus (Hailey-Hailey Disease)
Familial benign chronic pemphigus (Hailey-Hailey disease) This blistering disease is inherited in autosomal dominant fashion. Onset tends to occur during late adolescence. Patients with this disorder have a pruritic vesicular eruption in intertriginous areas that is worse during the summer months. Intact bullae may be ...
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