Skin disorders affect a large proportion of patients with primary immunodeficiencies (PID). Skin disease in PID can be caused by infections (bacterial, fungal, viral, parasitic), allergy, autoimmunity/autoinflammation (chronic urticaria, granuloma, eczema, alopecia, vitiligo, vasculitis), or malignancy (e.g. cancers due to HPV or EBV infection). Graft versus host disease (GVHD) following hematopoietic stem cell transplantation (STX) in PID can result in severe and chronic skin disease with limited treatment options.
Whereas control of invasive and severe bacterial infections as well as treatment of autoimmunity/ autoinflammation in PID has improved dramatically over the last decades, skin disease still has a major impact on morbidity and life quality of affected patients. Promising novel therapeutic options in various PID-related skin disorders have become available within the last years or are currently evaluated in preclinical and clinical trials.
In this Research Topic, we aim to present new and future therapeutic options in skin disorders related to PID/monogenetic autoinflammatory disorders. These include:
- treatment of allergic manifestations (e.g. eczema in HIES),
- treatment of fungal, viral, and bacterial skin infections (e.g. chronic or relapsing fungal infections in chronic mucocutaneous candidiasis (CMC), warts/condylomata/ mollusca contagiosa as in DOCK8 or GATA2-deficiency, relapsing bacterial infections, skin abscesses)
- therapeutic options for autoimmune/autoinflammatory skin disorders (granuloma, chronic urticaria, vasculitis, vitiligo, alopecia)
- STX as a treatment option for severe skin disorders (e.g. severe condylomata in GATA2-deficiency, rubella-associated granuloma in PID with DNA repair defects)
- skin disorders following STX (e.g. GVHD)
We welcome the submission of Original Research, Reviews, instructive Case Reports, and Clinical Trial articles with a focus on patients with PID/monogenetic autoinflammatory disorders, focusing on, but not limited to the following topics:
1. specific indications for biologicals
2. treatment of eczema (e.g. effects of Dupilumab on and beyond eczema in HIES)
3. novel treatment options for skin infections
4. new therapeutics for autoimmune/autoinflammatory skin manifestations (granuloma, chronic urticaria, vasculitis, vitiligo, alopecia) and PID-related skin cancer
5. STX in patients with severe skin disease (e.g. rubella-associated granuloma in PID with DNA-repair defects)
6. novel therapies for GVHD following STX
7. Future prospects for unsolved issues (e.g. phage therapy in chronic wounds/skin infections with S. aureus/P. aeruginosa)
