Clinical manifestation

Characteristic coarse facies; early non-specific papular or pustular eruption, favoring the scalp, proximal flexures, and buttocks; eczematous eruption; recurrent staphyloco-ccal abscesses, often described as cold abscesses because of lack of pain, heat, or redness; cellulitis; recurrent bronchitis, caused by S. aureus or Haemophilus influ-enzae; other systemic bacterial infections; chronic mucocutaneous candidiasis and onychomycosis; skeletal abnormalities: frequent painless bone fractures; scoliosis; hyperextensible joints

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