Type Typical inheritance Epidermolysis Autosomal bullosa simplex dominant (EBS) Autosomal recessive Junctional epidermolysis bullosa (JEB) Autosomal recessive Dystrophic epidermolysis bullosa— Dominant (DDEB) Autosomal dominant Dystrophic epidermolysis bullosa— Recessive (RDEB) Autosomal recessive Clinical features Electron microscope Bullae present at Cleavage through birth, early the basal cell infancy, or later layer or in life; in areas suprabasal layer of trauma; may above basement improve in membrane adolescence; rare mucous membrane involvement; nail involvement Usually at birth; Cleavage at spontaneous junction of bullae and large dermis and areas of erosion epidermis (above basement membrane) Early infancy and Dermal–epidermal later; little or no separation involvement of beneath hair and teeth; basement mucous membrane membrane lesions and nail dystrophy Present at birth; Dermal–epidermal widespread separation scarring and beneath deformity with basement mitten membrane deformity of hands; severe involvement of Kindler syndrome Autosomal recessive mucous membranes and nails Acral blistering; Cleavage at mottled multiple levels pigmentation; photosensitivity: skin atrophy; involvement of mucous membranes FIGURE 67.5 Linear arrangement of lesions (blisters in some cases) in an infant with incontinentia pigmenti Incontinentia Pigmenti Incontinentia pigmenti, a rare condition, occurs almost exclusively in females as it is inherited in an X-linked dominant fashion Inflammatory vesicles and bullae erupt in crops in a linear or curvilinear distribution (especially on the extremities) for the first several weeks to months of life ( Fig 67.5 ) These affected areas then progress to a warty stage Finally, swirl-like pigmentation occurs but not necessarily in the areas previously involved with warty or blistering lesions During the vesiculobullous stage, peripheral eosinophilia occurs (18% to 50% eosinophils) These patients often have extracutaneous manifestations, including dental anomalies, neurologic issues, ophthalmologic disease, and developmental delay Therefore, care must be coordinated with several subspecialists, and genetics referral is highly recommended to assist with family planning AUTOIMMUNE BULLOUS DISORDERS Linear IgA Disease Chronic bullous disease of childhood (CBDC), also known as linear immunoglobulin A (IgA) disease, may be seen in prepubertal children and is the most common acquired autoimmune blistering condition seen in childhood It usually has an acute onset with characteristic vesicles and bulla in an annular distribution often referred to as a “crown of jewels” or “string of pearls” ( Fig 67.6 ) Characteristic areas of involvement include the trunk, extremities, genital region, and face The mucosa may be involved Due to the acute onset and tense bulla, it may be initially misdiagnosed as bullous impetigo, but the recurrent nature and sterile bulla should suggest otherwise Patients will often complain of pruritus The lesions not scar but may leave behind persistent hyperpigmentation at prior sites of blistering The differential diagnosis includes bullous pemphigoid, dermatitis herpetiformis (DH), epidermolysis bullosa acquisita, and erythema multiforme FIGURE 67.6 Chest of a patient with chronic bullous dermatosis of childhood Notice the resemblance to erythema multiforme The diagnosis is confirmed by biopsy of a vesicle demonstrating a subepidermal blister with neutrophils along with direct immunofluorescence (DIF) of perilesional skin highlighting linear IgA staining at the basement