FIGURE 67.3 A: Infant with mastocytoma on the neck B: Same patient with another mastocytoma demonstrating the Darier sign following vigorous rubbing of the lesion FIGURE 67.4 A patient with junctional epidermolysis bullosa and a large, chronic ulceration of the right leg All subtypes of EB may present at or shortly after birth with variably sized bulla and erosions at sites of friction or adhesion They may affect all areas of the skin and the mucosa, particularly the oral mucosa, making early feedings a challenge due to pain EB may also affect several other organs depending on the subtype Patients may be born with large areas of absent skin known as congenital localized absence of skin (CLAS) Milia are also frequently seen at sites of blisters, and erosions and may be present at birth A hoarse cry may indicate airway involvement and is seen more commonly with junctional EB subtypes Milder types may also present with localized blistering at birth, however some patients may not present until they are adolescents or adults Certain types of EB simplex, for example, may not be diagnosed until the patients enter the army or other occupations where they are required to walk for long distances and subsequently develop blisters on their feet or use their hands in a repetitive motion leading to blistering on the palms Patients may have other associated