Tofacitinib citrate is a Janus kinase 1/3 inhibitor approved for the treatment of rheumatoid arthritis, but it has recently been used to treat alopecia areata (AA). 1-3 In this study, investigators searched the medical records of the Cleveland Clinic for any patients with confirmed AA who were treated with oral tofacitinib (Xeljanz; Pfizer) using a standardized, systematic treatment regimen. Methods | A standard departmental treatment protocol was developed prior to this review. Treatment with tofacitinib citrate was initiated at 5 mg twice daily, and all other AA therapies were ceased. The daily dosage was increased, as allowable by insurance coverage, by 5 mg per month until the treating physician noted first signs of hair regrowth and then held the medication at that dose. The Cleveland Clinic Foundation Institutional Review Board approved this retrospective medical record review and waived the patient informed consent requirement. The review took place from May 1, 2016, to June 1, 2016. Scalp hair loss was calculated from visit to visit by the same treating physician (M.P. or W.B.) using the validated Severity of Alopecia Tool (SALT) 4 score, which can range from 0% to 100%; the higher the score, the greater the amount of scalp hair loss. In all, patients were treated by 2 different physicians (M.P. and W.B.
Our study supports previous findings that atenolol is at least as effective as propranolol for treatment of IHs and poses less risk of bronchospasm. Our SCAMP proposes guidelines for dosing and monitoring parameters.
IMPORTANCEA comprehensive, user-friendly system to assess global ichthyosis disease burden is imperative to improving the care of patients with ichthyosis, identifying appropriate participants for clinical trials, and quantifying treatment outcomes. To our knowledge, there is currently no validated scale to objectively and systematically measure ichthyosis severity across the entire body.OBJECTIVE To create and evaluate a comprehensive and user-friendly instrument to measure total body ichthyosis severity in adults and children.
DESIGN, SETTING, PARTICIPANTSIn this qualitative study, ichthyosis experts participated in the content development of the Ichthyosis Scoring System (ISS). The body was divided into 10 regions, and Likert scales (0-4) were created to quantify scale and erythema, with extensive descriptors and photographic standards. An 83-image teaching set was created from photographs of participants with ichthyosis. Two cohorts of dermatologists (11 total) independently scored all test photographs twice to evaluate interrater and intrarater reliabilities. Participants were enrolled worldwide from referral centers and patient advocacy groups. Participants of all ages, races, and ethnicities were included in the creation of ISS, and dermatologists with varying experience and areas of expertise participated as raters to evaluate the ISS. The study was conducted from 2019 to 2021, and the data were analyzed in 2021.
MAIN OUTCOMES AND MEASURES Intraclass correlation coefficients determined overall reliabilities.RESULTS Across both cohorts of 11 dermatologists in total, the intraclass correlation coefficients for total, scale and erythema scores were greater than 0.90 (95% CI, 0.77-0.97), greater than 0.91 (95% CI, 0.79-0.98), and greater than 0.88 (95% CI, 0.72-0.97), respectively. Most body sites exhibited moderate to good interrater reliabilities for scale and erythema. Intrarater reliabilities were good to excellent.
CONCLUSIONS AND RELEVANCEThe results of this qualitative study demonstrate reproducibility and suggest that the ISS is a reliable system to measure global ichthyosis severity in adults and children.
Combigan (Allergan, Irvine, CA) is an ophthalmic solution that combines 0.2% brimonidine, a selective α-2 adrenergic agonist, with 0.5% timolol, a nonselective β-adrenergic antagonist. It is approved for the reduction of intraocular pressure in patients with glaucoma or ocular hypertension. There have been recent reports of successful treatment of superficial infantile hemangiomas (IHs) using Combigan topically. We report the case of a 2-month-old girl who developed life-threatening brimonidine toxicity requiring intubation and mechanical ventilation secondary to central nervous system depression and apnea after topical application to an ulcerated IH.
Over 200,000 children have been adopted into United States (US) families from abroad since the year 2000. Health care providers who care for children adopted internationally should be aware of the spectrum of illnesses seen in this population, and should be prepared to encounter potentially unusual situations. An appreciation for the unique pre-adoption exposures and vulnerabilities inherent in international adoption is critical for proper diagnosis and treatment of this heterogeneous group of children. It is important to consider the impact of potential early childhood stressors such as nutritional, sensory, and emotional deprivation, trauma and abuse, as well as prenatal exposures to drugs, alcohol, and infectious diseases. Providers must also take into account international variation in health care practices, including immunization, treatment, surgical, and hygiene standards. The differential diagnosis for cutaneous eruptions in children adopted internationally is broad and must encompass endemic systemic illnesses with skin manifestations, such as measles, tuberculosis, leprosy, and congenital syphilis, and primary dermatologic diseases such as scabies and bacterial and fungal infections. The importance of maintaining a broad differential and open mind when addressing the dermatologic needs of these children cannot be overemphasized.
Propranolol has revolutionized the treatment of infantile hemangiomas, and other beta-blockers provide promising alternatives. Unanswered questions remain about the optimal choice of agent, delivery mechanism, dosage, need for pretreatment evaluation or ongoing monitoring, and duration of therapy. The role of beta-blockers in treating other types of vascular tumors requires further study.
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