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.
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 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:
- petechial or maculo-papular rash
- distribution - lower limbs, ankles and dorsum of feet, upper limbs, trunk and face.
- non-blancing, occasionally palpable, painful and itchy
- constitutional symptoms including fever, malaise, myalgia, arthralgia, arthritis
- occasional haematuria, proteinuria
- gastrointestinal bleeding and anaemia have been reported
- there has been one report of central nervous system involvement
- full blood count, ESR, urea and electrolytes, liver function tests
- anti-nuclear factor, cANCA (PR3), pANCA (MPO)
- urine: microscopy, glomerular filtration rate estimates
|Cutaneous with no systemic symptoms||NSAID, anti-histamines|
|Recurrent and/or systemic involvement||Corticosteroids, chloroquine, azathioprine, methotrexate|
- 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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