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Urticaria pigmentosa - Skin in Child
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Urticaria pigmentosa - Skin in Child

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Contributors: Belinda Tan MD, PhD, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
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Synopsis

In urticaria pigmentosa (a form of cutaneous mastocytosis), mast cells accumulate in the skin, causing characteristic pruritic skin lesions. Unlike adult forms of mastocytosis, there is rarely internal organ involvement. It causes generalized, persistent, pigmented lesions that urticate or wheal with any mechanical stimuli. Severe forms of mastocytosis, particularly diffuse cutaneous mastocytosis, can present with or develop blisters.

Urticaria pigmentosa presents within the first weeks to months of life and can be expected to spontaneously involute. Involution can occur in early childhood or last until puberty. When systemic involvement occurs, flushing, diarrhea, syncope, and bronchospasm are all possible.

Codes

ICD10CM:
Q82.2 – Mastocytosis

SNOMEDCT:
78745000 – Urticaria pigmentosa

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Bullous impetigo
  • Diffuse mastocytosis presents with indurated, thickened skin, and numerous confluent papules are present. Systemic involvement is more common.
  • Juvenile xanthogranuloma has brown papules but will have a negative Darier's sign.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 01/10/2014
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Urticaria pigmentosa - Skin in Child
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Urticaria pigmentosa : Darier's sign positive, Primarily truncal, Tense bullae, Trunk, Widespread, Pruritus, Smooth papules, Hyperpigmented patches
Clinical image of Urticaria pigmentosa
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