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