Guselkumab Treatment Outcomes and Persistence in a Nationwide Real-world Cohort of Patients with Plaque Psoriasis.

Guselkumab treatment outcomes and persistence were assessed in a real-world cohort of Finnish patients with difficult-to-treat plaque psoriasis over a median follow-up of 1 year. Data on 181 patients who initiated guselkumab at the 15 study centres were collected retrospectively from the patient charts. Prior exposure to biologic therapies was common, with 56% and 35% having used at least 1 and 2 biologics, respectively. Median guselkumab treatment duration was 11 months with 21 patients (12%) discontinuing treatment during follow-up. Of 85 patients with a follow-up duration of at least 1 year, 73 (86%) were still on guselkumab at 1 year. Significant improvements during follow-up were seen in the absolute Psoriasis Area and Severity Index (PASI) scores with 32 patients (80%) having absolute PASI ≤ 2 after a 9-14-month treatment. Guselkumab treatment was effective and treatment persistence was high in the nationwide Finnish real-life setting.

[Hepatic porphyrias with cutaneous symptoms]. / Iho-oireiset hepaattiset porfyriat.

Hepatic porphyrias with cutaneous symptoms Cutaneous symptoms of porphyrias are initiated from a phototoxic reaction caused by sunlight and circulating porphyrins in the vascular walls of the skin. This leads in fragility, blistering and scarring of the skin on light-exposed areas. There are approximately 200 patients having hepatic porphyrias with cutaneous symptoms in Finland. Cutaneous symptoms of variegate porphyria and porphyria cutanea tarda are indistinguishable, but an effective treatment is available only for the latter. Differential diagnosis is important due to acute episodes occurring in variegate porphyria.

[Erythropoietic porphyrias]. / Erytropoieettiset porfyriat.

Erythropoietic, i.e., myelogenous porphyrias include erythropoietic protoporphyria and the very rare congenital protoporphyria and X-linked protoporphyria. Of these, in Finland only erythropoietic protoporphyria has been diagnosed, in which pain and swelling in the skin upon sunlight exposure are the most typical symptoms. A high protoporphyrin level in erythrocytes and a typical peak in the plasma porphyrin spectrum lead to diagnosis.

Histamine, but not leukotriene C4, is an essential mediator in cold urticaria wheals.

In addition to histamine, leukotriene C4 (LTC4) might also play a role in mediating cold urticaria wheals. To study the significance of LTC4 vs. histamine, 6 patients with cold urticaria were challenged with the ice cube test before and after ingestion of 10 mg cetirizine (antihistamine), 10 mg montelukast (leukotriene antagonist) or a combination of both drugs. Cetirizine diminished the cold-induced wheal by 50+/- 42%. Montelukast had no significant effect, and the combination of both drugs diminished the wheal by 37+/- 33%. Furthermore, a skin microdialysis technique detected the release of histamine in the cold-induced wheal, whereas no LTC4 release was detected. In conclusion, the antihistamine is effective and histamine is released, whereas the leukotriene antagonist is not effective and LTC4 is not released in the cold urticaria wheal.