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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 here. Erosions of the lips, gums, tongue, and palate, as pictured in Fig. 14-1, 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's sign, the extension of blistering by lateral finger pressure, is seen in the presence of widespread disease. Figure 14-2 shows the kind of crusting that develops as the roofs of blisters of pemphigus vulgaris disintegrate. Antibodies to desmoglein 1 are associated with skin lesions and antibodies to desmoglein 3 are associated with oral lesions.

The blisters of pemphigus vulgaris may arise on an erythematous base, or on normal-appearing skin, as pictured here. A variety of modalities have been employed in the treatment of this disease. The patient who is seriously ill requires hospitalization. For most patients, the most rapid and effective treatment remains high-dose systemic steroids. Patients undergoing this form of therapy are at high risk for infection and must be followed with extreme care. Immunosuppressive agents such as azathioprine, mycophenolate mofetil, and plasmapheresis are other useful therapies.

When the cutaneous changes of pemphigus take place in intertriginous spaces, clear blistering is not evident. Rather, one sees boggy inflammation and tumid granulation. 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.

This figure illustrates the type of scaling that accompanies pemphigus foliaceus. What one sees is largely exfoliating stratum corneum, not blisters. On biopsy analysis, one finds acantholysis occurring high in the epidermis, usually in the granular layer. There may be a subcorneal cleft, but the rest of the epidermis remains attached. Antibodies to desmoglein-1 are associated with pemphigus foliaceous.

This form of pemphigus is less severe than pemphigus vulgaris because blister formation occurs higher in the epidermis. As a result, there is less compromise of vital cutaneous functions. In most areas of the world, pemphigus foliaceus is extremely unusual in ...

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