Transient Bullous Dermolysis Of The Newborn

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Junctional Epidermolysis Bullosa

Figure 1.212. Epidermolysis bullosa in which the lesions were present at birth. The feet are one of the most commonly affected sites because babies kick their feet. If the lesions are extensive, the infant is at risk for fluid and electrolyte loss as well as infection.

Figure 1.213. Epidermolysis bullosa letalis (junctional epidermolysis bullosa, Herlitz's disease) in an infant with generalized involvement of the skin. Note the massive involvement of the lower extremities showing the denudation, scarring, and contractures. In this form of epidermolysis bullosa, the skin separates in the lamina lucida of the dermal-epidermal junction and blistering leads to mild atrophic changes. Junctional epidermolysis bullosa is the most severe form of epidermolysis bullosa. It is characterized by blistering and large erosions, mainly on the buttocks, trunk, and scalp without scarring unless complicated by secondary infection. Approximately 50% of these infants die within the first 2 years of life; some survive into adulthood. Therefore, recently the term "letalis" has not been used.

Figure 1.214. In transient bullous der-molysis of the newborn, the lesions are present at birth or shortly thereafter. With the bullous appearance of the lesions, epidermolysis bullosa should be considered in the differential diagnosis, but the family history and rapid regression of the lesions can confirm die diagnosis of transient bullous dermolysis of the newborn. The sibling of this infant had similar lesions at birth. In the figure on the left note the large bullous lesions on the 5 th finger of the right hand. There were similar lesions on the left hand at birth. In the figure on the right note the healing of the lesions 5 days after birth.

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