Characteristics of Staphylococcus aureus related skin disorders in adults with primary immunodeficiency diseases

Primary immunodeficiency diseases (PIDs) are usually characterized by recurrent
and/or severe infections as well as an increased risk of autoimmunity,
malignancy and allergic disorders. Moreover, both infectious and non-infectious
skin disorders are common in PIDs and are among the presenting clinical
manifestations. Staphylococcus (S.) aureus induced skin infections are the most
common infectious skin disorders reported in PIDs. On the other hand,
dermatitis is one of the most predominant non-infectious skin manifestations in
PIDs, in which S. aureus is suggested to contribute to inflammation. Dermatitis
may be part of the atopic syndrome, in which patients show next to atopic
dermatitis the tendency toward development of food allergies, asthma and
allergic rhinitis. Based on previous narrative reviews and case series, S.
aureus skin infections, dermatitis and other skin disorders as well as atopy
seem to be all fairly common in patients with a PID. However, knowledge on the
prevalence of PID associated skin disorders and atopic manifestations in PIDs
remains limited.

The primary goal is to evaluate the presence and type of skin disorders in
patients with a PID. Secondary objectives are to determine the presence of S.
aureus on the lesional skin and/or nose in patients with a PID and to evaluate
the presence of atopic symptoms (i.e. atopic dermatitis, food allergy, asthma,
rhinoconjunctivitis) in patients with a PID.

An observational clinical study

Patients with a PID and a suspected presence of S. aureus related skin
disorders at the moment of screening are eligible for inclusion in the
observational part of the study. This includes an extensive dermatological and
atopy anamnesis, a total skin inspection and determination of the S. aureus
colonization status by screening the lesional skin and nares. Patients with a
proven S. aureus related skin disorder (skin inspection and positive lesional
skin culture) are eligible for participation in the interventional part of the
study, in which patients are treated with Gladskin gel. The investigational
product will be used twice daily on the lesional part of the skin during 6
weeks. The effect of Staphefekt treatment on the doctor and patient reported
severity of the skin disorder and the S. aureus load on the lesional skin will
be assessed by comparing scores after 6 weeks treatment to scores at baseline
(visit 2).

Risks: There is a possibility of a contact allergic reaction to the swab
solution (Tween).
Benefit: Participation in this study might result in individual benefit for the
patient, as patients could be referred by the researcher (if indicated) for
treatment by the GP or dermatologist.
Burden: The study includes 1 visit. Visits will take approximately 30 minutes.
Screening for S. aureus is performed using swabs, a non-invasive method.