Urticaria pigmentosa in children vs adults

Children

In children the trunk is most commonly affected with lesions

Clinical features

  •  Mastocytosis with increased mast cells, usually in the skin.
  •  Usually presents within the first few months of life and resolves spontaneously by teenage years.
  • Widespread itchy, brown or reddish macules, papules and plaques that may blister.
  • Lesions urticate and weal develops when rubbed (Darrier's sign).
  • Trunk most commonly affected but may involve extremities (not face, scalp or palms).
  • Can, rarely, lead to life-threatening episodes of shock or systemic involvement.
  • Skin biopsy if necessary, but be careful not to inject local anaesthetic into the lesion.

Management

  • Does not require treatment unless symptomatic.
  • Antihistamines and calamine lotion if necessary.
  • Avoid aggravating factors: exercise or heat; medications such as aspirin, opioids, anticholinergics and alcohol.

Adults

Uriticaria pigmentosa lesions in adults are brown to reddish in colour

Clinical features

  •  Onset in adult life with peak at 30-40 years.
  • More likely to have persistent disease.
  • Greater risk of systemic involvement.
  • Widespread itchy, macules, papules and plaques.
  • Lesions are brown to reddish.
  • Lesions urticate and weal develops when rubbed (Darrier's sign).
  • Systemic involvement may lead to bone pain or fracture, malaise or psychiatric symptoms, weight loss, diarrhoea and vomiting, liver or splenic enlargement, hypotension, tachycardia, shock or angina.

Management

  • Bone scan to identify osteoporosis or lytic bone lesions.
  • If GI symptoms, endoscopy, CT scan and small bowel X-ray.
  • Avoid aggravating factors.
  • If necessary treat with: oral antihistamines; mast cell stabilisers (sodium cromoglicate); topical steroids may help itching; PUVA - may improve appearance and itching; interferon if severe.

Dr Jean Watkins is a sessional GP in Hampshire.

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