Dermatosis chronic bullous of childhood L13.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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

Definition
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Chronic, vesiculo- bullous, occasionally itchy disease that occurs within the first years of life.

Etiopathogenesis
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Autoimmunological bubble formation with circulating IgA antibodies against a 97KD protein (LABD97) and a 120KA protein (LAD-1).

Manifestation
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Early childhood. The average age of the disease is 2.4 years.

Localization
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Groin, inner thighs, rare trunk, head and neck.

Clinical features
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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.

Histology
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Subepithelial blistering, papillary microabscesses of neutrophil but also eosinophilic leukocytes, mixed corial infiltrate in which eosinophilic leukocytes may dominate.

Direct Immunofluorescence
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Linear homogeneous IgA deposits in the dermoepidermal junction zone.

External therapy
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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 therapy
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  • 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/forecast
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Eminently chronic, years of progression, often spontaneous healing before puberty. Individual cases until adulthood.

Literature
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  1. Fisler RE et al (2003) Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. At J Dermatopathol 25: 183-189
  2. Gosch S et al. (1993) The therapy of IgA-linear dermatosis in childhood Dermatol Month 179: 330-333
  3. Hertl M, Schuler G (2002) Bullous autoimmune dermatoses. 1: Classification. dermatologist 53: 207-219
  4. Hunziker T (1996) Bullous autoimmune dermatoses in childhood. Z Hautkr 71: 169-172
  5. Lings K et al (2015) Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. Acta Derm Venereol 95: 466-471
  6. Mintz EM et al (2011) Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin 29:459-462
  7. Nemeth AJ et al (1991) Childhood bullous pemphigoid. Clinical and immunologic features, treatment, and prognosis. Arch Dermatol 127: 378-386
  8. Simon JC et al (1995) Chronic bullous dermatosis of childhood. Dermatologist 46: 485-489
  9. Thappa DM et al (2003) Chronic bullous dermatosis of childhood. Postgrade Med J 79: 437
  10. Wilk M et al (1993) Chronic bullous dermatosis in children (linear IgA dermatosis) Dermatologist 44: 470-475

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020