Porokeratosis encompasses a diverse group of dermatoses, both acquired and genetic, marked by a keratinization disorder. Porokeratosis of Mibelli (PKM) presents as solitary plaques or multiple papules/macules with central atrophy and raised hyperkeratotic borders. Here, we present a case of giant porokeratosis (GPK), a rare form often considered a morphological variant of PKM, with unique clinical and histopathological aspects. Our case involves a 29-year-old patient with a 15 × 10 cm irregular plaque on the dorsal aspect of the right hand. The patient was previously prescribed various topical treatments (retinoids, calcineurin inhibitors, and combinations of corticosteroids with vitamin D3 analogs) and systemic retinoids without improvement before presenting to our department. Due to the high risk of neoplastic transformation and the unavailability of imiquimod, the patient was recommended topical 5-fluorouracil treatment. The trajectory of the lesion under treatment revealed a favorable evolution, and the patient was subjected to regular monitoring every three months to assess the ongoing progress. Recognizing GPK as a high-risk variant is crucial for dermatologists, and it requires a personalized approach. Regular monitoring is advised to detect potential malignant transformations promptly. Future research holds promise for diagnostic advancements, refined treatment modalities, and a deeper understanding of the molecular mechanisms underlying malignancy in porokeratosis.

01 Jun 2023·Calcified tissue international

Comparison of the Efficacy of Zoledronate Acid or Denosumab After Switching from Romosozumab in Japanese Postmenopausal Patients.

Article

Author: Shimodan, Shun ; Arita, Kosuke ; Shimizu, Tomohiro ; Iwasaki, Norimasa ; Oue, Tetsuro ; Asano, Tsuyoshi ; Ishizu, Hotaka

We aimed to compare the efficacy of switching from romosozumab (RMAb) to denosumab (DMAb) or zoledronic acid (Zol) with respect to changes in bone mineral density (BMD) and bone metabolism. We also aimed to determine predictors of changes in BMD among patients who received sequential therapy from RMAb. One hundred patients who received RMAb therapy were recruited for this study. A total 49 patients received bisphosphonate (BP) pre-treatment and 51 received active vitamin D3 analog pre-treatment or no treatment. Forty-two patients were switched to Zol (BP-RMAb-Zol; 20 and RMAb-Zol; 22), and 58 patients were switched to DMAb (BP-RMAb-DMAb; 29 and RMAb-DMAb; 29). Longitudinal changes in bone metabolic markers (P1NP and TRACP-5b) and BMD were also evaluated. In the BP-RMAb-Zol group, TRACP-5b increased after administration of Zol, and the mean BMD of the lumbar spine (LS) was significantly lower than those in the BP-RMAb-DMAb, RMAb-Zol and RMAb-DMAb groups at 24 months. The % changes in BMD of the LS after 24 months were associated with TRACP-5b values at baseline and at 12 months in patients who received Zol therapy, and with TRACP-5b value at baseline in patients who received DMAb therapy. The DMAb follow-on regimen could be considered more effective than Zol as a sequential agent for the enhancement of BMD after RMAb in patients with BP pretreatment. TRACP-5b, especially the baseline value, may predict the efficacy of sequential therapy from RMAb, as well as previous treatments.

01 Nov 2022·Journal of the American Academy of DermatologyQ1 · MEDICINE

Real-world experience with ustekinumab for the treatment of plaque psoriasis in pediatric patients: A retrospective, single-center chart review

Q1 · MEDICINE

Article

Author: Stevenson, Rebeka ; Ladha, Malika A ; Prajapati, Vimal H ; Luca, Nadia J ; Rankin, Brian D ; Devani, Alim R ; Adly, Merna

A review.Our understanding of the efficacy and safety of ustekinumab for treatment of pediatric plaque psoriasis is limited to two phase 3 clin. trials.To evaluate the real-world experience of this biol. in children and adolescents, we conducted a retrospective chart review at a community dermatol. practice in Calgary, Canada.Of the 23 patients, 78.3% (18/23) were primary responders and 17.4% (4/23) were secondary responders; 1 primary responder did not sustain IGA 0/1 at week 52.At week 16, the mean Psoriasis Area and Severity Index (PASI) decreased from 13.3 to 2.4(P1/4.0001;Table II); the proportion of patients achieving PASI improvements from baseline to 75%(PASI75), 90% (PASI90), and 100% (PASI100) was 78.3%, 69.9%, and 47.8%, resp.; and the mean body surface area and mean Children's Dermatol. Life Quality Index were reduced from 14.2% to 1.8%(P1/4.0001) and from 9.4 to 2.7 (P1/4.0001), resp.At week 52, the mean PASI was 0.9 (P1/4.0001);PASI75, PASI90, and PASI100 were achieved by 89.5%, 73.7%, and 47.4% of patients, resp.(Table II); the mean body surface area was 0.2%(P1/40001); and the mean Children's Dermatol. Life Quality Index was 2.0 (P1/4.0001).Dose optimization was required in 69.5% of patients; 13.0% had a dose escalation (beyond weight-based adjustments), 26.1% had an interval reduction, and 30.4% required both dose escalation and interval reductionCommon concomitant therapies were topical corticosteroid/vitamin D3 analogs(60.9%), topical corticosteroids (26.1%), and methotrexate (21.7%).Effectiveness outcomes for pediatric patients with plaque psoriasis at weeks 16 and 52 after treatment with ustekinumab.Our findings are congruent with those of previous clin. trials. Landells et al1reported that 80.6%,61.1%, and 38.9% of ustekinumab-treated patients achieved PASI75, PASI 90, and PASI100, resp.,at week 12, with responses maintained at week 52.Our study indicates that ustekinumab is an effective and safe biol. for pediatric plaque psoriasis.The limitations of this study include its retrospective nature and relatively small sample size.

100 Deals associated with Vitamin D3 analogs(University of New South Wales)

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Indication	Highest Phase	Country/Location	Organization	Date
Neoplasms	Phase 1	-	MRC Biotech Pty Ltd.	-
Neoplasms	Phase 1	-	The University of New South Wales	-

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