'Peeling paint' dermatitis as a presenting sign of cystic fibrosis

Presentation of cystic fibrosis with a rash is rare, with only 19 previously reported cases. This unusual presentation is associated with false negative sweat tests, delays in diagnosis and considerable mortality. Multiple nutritional deficiencies, the aberrant production of prostaglandins and free-radical mediated damage have been implicated in the pathogenesis of this kwashiorkor-like dermatitis. In spite of the rarity of this presentation, recognition of the rash is important, not only to expedite the diagnosis, but also to gain insight into the disease. We present a further case to highlight this unusual presentation and discuss potential pathophysiological mechanisms.