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Dermatosis chronic bullous of childhoodL13.8
Synonym(s)
Blistering disease in children; Bullous disease of childhood; chronic bullous dermatosis of childhood; Chronic bullous dermatosis of childhood; chronic bullous disease of childhood; Chronic non hereditary; Chronic non hereditary blistering disease in children
DefinitionThis section has been translated automatically.
Chronic, vesiculo- bullous, occasionally itchy disease that occurs within the first years of life.
EtiopathogenesisThis section has been translated automatically.
Autoimmunological bubble formation with circulating IgA antibodies against a 97KD protein (LABD97) and a 120KA protein (LAD-1).
ManifestationThis section has been translated automatically.
Early childhood. The average age of the disease is 2.4 years.
LocalizationThis section has been translated automatically.
Groin, inner thighs, rare trunk, head and neck.
Clinical featuresThis section has been translated automatically.
Anular or serpiginous, urticarial erythema (may be absent) with marginal, bulging vesicles or blisters. The vesicles are more frequently strung together like a string of pearls. After rupture of the blisters/bubbles erosions or crusts. Healing under post-inflammatory hyperpigmentation; no scarring.
HistologyThis section has been translated automatically.
Subepithelial blistering, papillary microabscesses of neutrophil but also eosinophilic leukocytes, mixed corial infiltrate in which eosinophilic leukocytes may dominate.
Direct ImmunofluorescenceThis section has been translated automatically.
Linear homogeneous IgA deposits in the dermoepidermal junction zone.
External therapyThis section has been translated automatically.
Drying and disinfecting with 2% Clioquinol-Lotio R050 or antiseptic solutions like polihexanide (Serasept, Prontoderm), potassium permanganate solution(light pink). If necessary, weak to medium glucocorticoids such as 0.5% hydrocortisone cream(e.g. Hydro-Wolff, R121 ), prednicarbate cream(e.g. Dermatop), methylprednisolone cream(e.g. Advantan). Caring with oil baths like e.g. Balneum Hermal.
Internal therapyThis section has been translated automatically.
- Therapy of the 1st choice is Dapson (e.g. Dapson-Fatol) 0.5-2.0 mg/kg bw/day in combination with Vit C (e.g. Cebion Tbl.) 400-1000 mg/day.
- In severe cases, combination with systemic glucocorticoids such as prednisolone (e.g. Decortin H) 0.5-2.0 mg/kg bw/day p.o. Maintenance dose according to clinic, if possible below the Cushing's threshold.
- Alternative: Test with sulfadiazine or dapsone (0.5 mg/kg bw/day) in combination with intravenous immunoglobulins (2 g/kg bw/month).
Progression/forecastThis section has been translated automatically.
Eminently chronic, years of progression, often spontaneous healing before puberty. Individual cases until adulthood.
LiteratureThis section has been translated automatically.
- Fisler RE et al (2003) Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. At J Dermatopathol 25: 183-189
- Gosch S et al. (1993) The therapy of IgA-linear dermatosis in childhood Dermatol Month 179: 330-333
- Hertl M, Schuler G (2002) Bullous autoimmune dermatoses. 1: Classification. dermatologist 53: 207-219
- Hunziker T (1996) Bullous autoimmune dermatoses in childhood. Z Hautkr 71: 169-172
- Lings K et al (2015) Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. Acta Derm Venereol 95: 466-471
- Mintz EM et al (2011) Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin 29:459-462
- Nemeth AJ et al (1991) Childhood bullous pemphigoid. Clinical and immunologic features, treatment, and prognosis. Arch Dermatol 127: 378-386
- Simon JC et al (1995) Chronic bullous dermatosis of childhood. Dermatologist 46: 485-489
- Thappa DM et al (2003) Chronic bullous dermatosis of childhood. Postgrade Med J 79: 437
- Wilk M et al (1993) Chronic bullous dermatosis in children (linear IgA dermatosis) Dermatologist 44: 470-475