Medicine by Alexandros G. Sfakianakis,Αλέξανδρος Γ. Σφακιανάκης

Xeroderma pigmentosum is a rare disease characterized by an increased propensity for solar skin damage and skin cancer owing to underlying defects in nucleotide excision repair or translesion synthesis.
Patient presentation (e.g. presence of acute sunburn reaction, neurological deficits) depends on both the specific step affected in nucleotide excision repair and the type of mutation.
Management of these patients still relies primarily on limiting sun exposure and attenuating morbidity.

What does this study add?

This review emphasizes novel therapeutic approaches to xeroderma pigmentosum that focus on metabolic defects downstream of nucleotide excision repair.
Specific sulfonylureas promote clearance of DNA damage and increase resistance to ultraviolet radiation in a cellular model of xeroderma pigmentosum.
Nicotinamide attenuates the effects of ultraviolet radiation in cells, and caloric restriction decreases DNA damage burden in animal models of xeroderma pigmentosum.

Linked Comment: Fassihi. Br J Dermatol 2019; 181:236–237.
Plain language summary available online

Evidence‐Based Dermatology
SYSTEMATIC REVIEW

Open Access

Persistence and effectiveness of nonbiologic systemic therapies for moderate‐to‐severe psoriasis in adults: a systematic review

K.J. Mason, S. Williams, Z.Z.N. Yiu, K. McElhone, D.M. Ashcroft, C.E. Kleyn, Z.K. Jabbar‐Lopez, C.M. Owen, N.J. Reynolds, C.H. Smith, N. Wilson, R.B. Warren, C.E.M. Griffiths

Pages: 256-264 | First Published: 10 January 2019

What's already known about this topic?

Research examining acitretin, ciclosporin, fumaric acid esters (FAE) and methotrexate for the treatment of moderate‐to‐severe psoriasis has focused on safety and efficacy in randomized controlled trials.
The persistence and effectiveness of acitretin, ciclosporin, FAE and methotrexate since the introduction of biologic therapies in real‐world clinical practice are poorly understood.

What does this study add?

This systematic review examines the persistence and effectiveness of methotrexate, acitretin, ciclosporin and FAE for moderate‐to‐severe psoriasis.
Data on the persistence and effectiveness of systemic therapies are lacking, particularly for acitretin and ciclosporin.
The definitions of persistence and reporting of effectiveness are inconsistent.
Further good‐quality observational studies are needed to explore the real‐world persistence and effectiveness of systemic treatments used for psoriasis.

Linked Comment: Garcia-Doval and Sbidian. Br J Dermatol 2019; 181:237.
Plain language summary available online

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Medicine by Alexandros G. Sfakianakis,Αλέξανδρος Γ. Σφακιανάκης

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Δευτέρα 5 Αυγούστου 2019

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