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Hall, John C. Sauer's Manual of Skin Diseases, 9th Edition. 2006. Lippincott Williams & Wilkins

SECTION II - Inflammatory Skin Diseases > 18 - Bullous Dermatoses

Bullous Dermatoses

In almost all cases of bullous diseases, it is necessary to examine a fresh tissue biopsy specimen for deposits of

immune reactants, immunoglobulins, and complement components, at or near the basement membrane zone.

Routine histologic examination of a formalin-fixed biopsy specimen is, of course, also usually indicated (seeChap. 10).

Three bullous diseases are discussed in this chapter: pemphigus vulgaris, dermatitis herpetiformis, and erythemamultiforme bullosum. However, other bullous skin diseases do occur, and in this introduction they are

differentiated from these three:

Staphylococcal scalded skin syndrome: Clinically, this disorder is similar to TEN but has been separated fromthis disease because of the finding that phage group 2 S. aureus is the usual cause. In newborns, this formerly

was known as Ritter von Ritterschein's disease. It also occurs in children and rarely adults. The prognosis is

very favorable. If suspected, anti-staphylococcus drugs should be started intravenously immediately. The break

in the skin is higher in the epidermis than in drug-induced TEN and this can be rapidly ascertained with a skinbiopsy.

Stevens-Johnson syndrome is a variant of TEN with more mucous membrane disease. Drugs are usually thecausative factor especially in adults. Most commonly implicated are sulfonamides, anticonvulsants, and

NSAIDs. There may be a genetic predisposition to this bullous drug reaction. Therapy is supportive, and an

appreciable number of cases are fatal. Intravenous immunoglobulins (IVIG) may be life saving. Cyclosporine isa preferred mode of therapy by some authors. High-dose systemic corticosteroids are controversial withopinions ranging from contraindicated to life saving if given in very high doses very early in the course of the

disease. Wound care is essential. Debridement should be avoided. Silver nitrate irrigation and soft gauze

dressings (Sof Sorb Gauze, which may be fitted on as a garment) are used in wound care. This is usually done

in an intensive care unit because electrolyte and fluid balance is crucial. A central venous line helps greatly inmanaging these patients. The most crucial factor in survival is stopping any potential offending drug as quickly

as possible. Acute graft-versus-host disease can be an identical disease process and should be treated in asimilar manner.