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Chapter 44: Linear IgA Dermatosis and Chronic Bullous Disease of Childhood

Sarah Blanchard; Russell P. Hall III; Caroline H. Rao

LINEAR IGA DERMATOSIS AND CHRONIC BULLOUS DISEASE OF CHILDHOOD

Therapeutic Approach

Linear IgA dermatosis (LAD) is a group of neutrophilic autoimmune conditions characterized by linear deposition of IgA along the basement membrane zone. LAD clinical subtypes include adult onset, childhood variant, and drug-induced. LAD classically presents with tense bullae forming the “string of pearls” or “cluster of jewels” sign although significant variation in presentation exists. The adult form is rare and follows a chronic relapsing course. Chronic bullous disease of childhood (CBDC) is the most common blistering disease of childhood and follows a waxing/waning course with resolution typically by puberty. Drug-induced LAD has variable clinical presentations and cessation of the culprit drug typically results in resolution. Dapsone is considered first line treatment for LAD unresponsive to topical steroids. Case reports and small case series offer alternatives, sometimes used in combination with low dose of systemic corticosteroids (<0.1 mg/kg/d), for recalcitrant disease. See Table 44-1.

Table 44-1Linear IgA Dermatosis and Chronic Bullous Disease of Childhood Treatment Table
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Table 44-1 Linear IgA Dermatosis and Chronic Bullous Disease of Childhood Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Mid- to high-potency topical steroids+ LABD, CBDC Anti-inflammatory BID Skin atrophy, striae, hypopigmentation, acneiform eruption HPA-axis suppression None III1-4
Tacrolimus 0.03% ointment LABD, CBDC Calcineurin inhibition BID Skin irritation, black box warning, avoid <2 y of age None III5,6
Systemic Therapy
Dapsone LABD, CBDC Inhibition of neutrophil chemotaxis and myeloperoxidase activity Pediatric: 0.5-2 mg/kg/d Adult: 25-150 mg/d Hemolytic anemia, agranulocytosis, methemoglobinema, GI upset, peripheral neuropathy, LFT abnormalities, hypersensitivity reaction G6PD level prior to initiation. CBC and LFTs weekly × 1 mo, monthly × 2-4 mo, then q6mo and with dosage changes III7-9
Sulfasalazine LABD, CBDC Anti-inflammatory 40-60 mg/kg/d 500 mg once daily or 1 g/d in 2 divided doses; increase weekly to maintenance dose: 2 g/d in 2 divided doses; maximum Hypersensitivity reaction, hemolytic abnormalities, anemia, agranulocytosis, GI upset, headache, LFT abnormalities, pneumonitis, reversible oligospermia G6PD level prior to initiation. CBC, LFTs, Cr at baseline, every 2-4 wk × 3 mo, every 8-12 wk × 3 mo, then every 12 mo. CBC 1 wk after any dose increase. III10-12
Systemic Corticosteroids LABD, CBDC Anti-inflammatory Pediatric: prednisolone or prednisone 0.5-2 mg/kg/d Adult: prednisone 0.5 mg/kg-1 mg/kg daily Hyperglycemia, infection, hypertriglyceridemia, cataracts, peptic ulcer disease, electrolyte disturbances, acne, bone changes, glaucoma, growth suppression, adrenal suppression, cushingoid states Fasting glucose or HbA1c, triglycerides, and potassium monthly and then every 3-4 mo. Blood pressure and weight monthly and then every 3 mo. TB screening and immunization history review. Growth in children, Ophthalmology exam annually III13-15
Mycophenolate mofetil LABD, CBDC Noncompetitive inhibition of purine synthesis

Pediatric: 300-600 mg/m2 maximum 2000 mg

Adult: 1000-3000 mg/d

 Carcinogenicity, gastrointestinal (nausea, diarrhea) dysuria, infections (including Progressive multifocal leukoencephalopathy) ...

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