▶Also known as chronic bullous disease of childhood.
▶A rare, acquired autoimmune blistering disorder.
▶Most often occurs in children younger than 5 years.
▶Occasionally preceded by an upper respiratory illness.
▶Usually idiopathic but can be drug-induced. There are also reports of occurrence following vaccinations, although causality is not proven.
▶Vesiculobullous lesions occur on the extremities, face, and trunk.
▶Bullae may form a ring around margins of an older crusted lesion, forming the “cluster of jewels” configuration (Figure 84.1).
▶Pruritus tends to be mild, but pain may be significant.
▶Mucous membranes may be involved.
■Oral erosions most common type of mucous membrane involvement.
■Eye involvement occurs less commonly.
Look-alikes
Disorder | Differentiating Features |
•Does not usually present as a recurrent or chronic condition. •Involvement more focal. •Mucous membranes not involved. •Blisters rupture easily, leaving superficial erosions with peripheral collarettes of scale. | |
Herpes simplex virus infection | •Most often clustered vesicles and erosions on an erythematous base. •Usually more focal. |
•Urticarial plaques present in addition to tense blisters. •Pruritus common with early lesions. •Direct fluorescence and immunoblotting studies will help confirm diagnosis. | |
•Usually presents as tiny vesicles and erosions. •Most often clustered on elbows, knees, shoulders, sacrum, and buttocks. •Pruritus is intense. •May be associated with gluten sensitivity. | |
Stevens-Johnson syndrome (SJS) or Mycoplasma pneumoniae–induced rash and mucositis | •Typical target lesions may be present (primarily with SJS). •Bullae in setting of SJS do not typically present in “cluster of jewels” pattern. •Erosions of 2 or more mucous membranes typically present. •Palms and soles usually involved in SJS. •History of Mycoplasma or herpes simplex virus infection or drug ingestion may be present. |
Epidermolysis bullosa | •Inherited (not acquired) mechanobullous disease. •Usually begins in neonatal period or during early infancy. •Blisters induced by trauma. •Certain subtypes may reveal nail dystrophy, milia, extensive scarring, mitten deformities of the hand. •Mutation analysis or immunomapping of skin biopsy specimens confirms diagnosis. |
•Other features of systemic lupus erythematosus (SLE) usually present. •Concentrated in sun-exposed areas. •Antinuclear antibody (and other serologic studies) will confirm diagnosis of SLE. •Direct immunofluorescence and immunoblotting studies help confirm the diagnosis. | |
•Blisters induced by trauma. •Scarring common. •Direct fluorescence and immunoblotting studies will help confirm diagnosis. | |
•Typically occur in the summer months. •Pruritus present. •Linear groupings of lesions may be present. •Central punctum may be visualized. •Other, more typical urticarial papules may be present. |
▶The diagnosis is suggested clinically and confirmed by skin biopsy.
■Histopathologic analysis reveals a subepidermal blister.
■Direct immunofluorescence examination shows a linear band of IgA along the dermal-epidermal junction.
▶After confirming normal glucose-6-phosphate dehydrogenase levels, dapsone is used initially at a dose of 0.5 to 1 mg/kg/d.
▶Patients on dapsone require monitoring for decreased hemoglobin and leukopenia, as well as hepatotoxicity (rare).
▶Systemic steroids may be useful during acute stage of therapy (often in conjunction with dapsone) but should not be used chronically.
▶For dapsone-resistant disease, other treatment options include sulfapyridine, erythromycin, dicloxacillin, azathioprine, colchicine, mycophenolate mofetil, and intravenous immunoglobulin.
Treating Associated Conditions
▶If conjunctival involvement is present, regular ophthalmologic evaluations are indicated.
▶Most children with linear IgA dermatosis experience spontaneous remission within 5 years of onset, but treatment of the disorder can be challenging.