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Dermatological involvement with Cystic Fibrosis

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Manifestations of cystic fibrosis involving the skin 

In addition to the more well-recognised manifestations of cystic fibrosis, a number of findings involving the skin have been reported including aquagenic wrinkling and vasculitis, outlined below1.

Aquagenic wrinkling

Symptoms include rapid, transient formation of oedematous papules and plaques on palms and soles with exposure to water. No investigation or treatment is recommended.

Vasculitis

Vasculitis can affect the skin, and also renal function, gastrointestinal tract and central nervous system. A renal biopsy can show changes similar to Henock Schoenlein Purpura.

Signs and symptoms of vaculitis include:

Investigation:

Treatment:

Cutaneous with no systemic symptoms NSAID, anti-histamines
Recurrent and/or systemic involvement Corticosteroids, chloroquine, azathioprine, methotrexate


Reference

  1. Bernstein ML, McCusker MM, Grant-Kels, JM. Cutaneous manifestations of cystic fibrosis. Padiatric Dermatology 25(2):150-8, 2008 Mar-Apr

Information last reviewed: April 2017

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