Urticaria pigmentosa bullosa Q82.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.11.2021

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Synonym(s)

Urticaria pigmentosa pemphigoides

Definition
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Rare form of cutaneous mastocytosis with vesicular reaction in the area of pigmented efflorescences to physical stimuli. Internal manifestations are possible(mastocytosis).

Manifestation
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Especially infants and small children are affected.

Clinical features
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Itching, rubbing, urticarial reaction with blistering, long-standing hyperpigmentation.

Differential diagnosis
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General therapy
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According to Urticaria pigmentosa, see there.

External therapy
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Dry brushing with disinfecting solutions such as potassium permanganate (light pink) or quinolinol (e.g. Chinosol 1:1000 or R042 ). In case of severe itching, creams or lotions containing glucocorticoids with 0.5-1.0% hydrocortisone R119, R123 or 0.05-0.1% betamethasone R030 for a short time.

Internal therapy
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Literature
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  1. Fenske NA et al (1985) Congenital bullous urticaria pigmentosa. Arch Dermatol 121: 115-118
  2. Has C et al (2002) Recurring staphylococcal scalded skin syndrome-like bullous mastocytosis: the utility of cytodiagnosis and the rapid regression with steroids. Pediatric Dermatol 19: 220-223
  3. Haustein UF et al (1997) Bullous mastocytosis in a child. dermatologist 48: 127-129
  4. Stanka F et al (1984) Urticaria pigmentosa bullosa. Nude Dermatol 10: 166-168
  5. Valent P et al (2003) Mast cell proliferative disorders: current view on variants recognized by the World Health Organization. Hematol Oncol Clin North Am 17: 1227-1241

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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: 19.11.2021