Into the Light: Afamelanotide and the Treatment of Erythropoietic Protoporphyria in the United States.

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare disease that causes disabling cutaneous photosensitivity with pain and burning sensations. In 2019, afamelanotide, an α-melanocyte-stimulating hormone analogue, was approved in the United States for treatment of EPP. In this study, patients receiving afamelanotide filled out questionnaires assessing the benefit of treatment. Outcomes measured included: return to normal activities, experience of phototoxic reactions, effect on patient confidence, and more. Patients ranked their experience on a descriptive scale ranging from "very much" to "never". RESULTS: Prior to treatment, 75% of patients indicated that EPP affected their lives "very much" or "a lot". This number fell to 11% after the 1st implant and to 0% after each subsequent implant. The number of patients that willingly ventured outside increased with each subsequent implant. CONCLUSION: The results of this study clearly show that afamelanotide treatment can dramatically and positively impact the lives of EPP patients.  Citation: Resnik SR, Targett D, Resnik BI. Into the light: afamelanotide and the treatment of erythropoietic protoporphyria in the United States. J Drugs Dermatol. 2023;22(9):941-949. doi:10.36849/JDD.7126R1.

A case of Stevens-Johnson syndrome treated with oral cyclosporine.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) can occur at any age and are commonly caused by adverse drug events. Rapid diagnosis of SJS/TEN is imperative, followed by immediate cessation of offending agent and induction of appropriate treatment. Cyclosporine, a calcineurin inhibitor, has been reported to have a promising therapeutic effect in SJS/TEN patients with few side effects. Diagnosis of SJS/TEN in children is especially challenging as many of the symptoms mimic that of an upper respiratory infection, or other viral entities such as cocksackie A, roseola, or herpes simplex virus. We recommend initiating cyclosporine modified treatment, especially in children, upon any suspicion of SJS/TEN in a patient in order to halt the disease progression as early as possible.

An unusual case of pyogenic granuloma-like Kaposi sarcoma.

Kaposi sarcoma (KS) is not typically included in the differential diagnosis of lesions with clinical characteristics of pyogenic granuloma. However, cases of pyogenic granuloma-like Kaposi sarcoma have been reported in the literature. This variant is extremely rare and possesses clinical and histological findings consistent with both conditions. We report an elderly, immunocompetent man with pyogenic granuloma-like Kaposi sarcoma, which was clinically consistent with a pyogenic granuloma and possessed histological findings consistent with Kaposi sarcoma and pyogenic granuloma.

Examining the Safety and Efficacy of Low-Level Laser Therapy for Male and Female Pattern Hair Loss: A Review of the Literature.

PURPOSE: Pattern hair loss is the most common type of alopecia. Standard of care involves long-term use of topical medications with limited effectiveness. Low-level laser therapy (LLLT) has become a popular alternative treatment. Here, we examine published clinical trials to establish whether the breadth of evidence supports LLLT for pattern hair loss. METHODS: A literature search was conducted within the PubMed, Embase, Scopus, and Cochrane Trials databases to identify original articles evaluating hair regrowth following LLLT. Articles were selected based on use of 600-1,100 nm wavelengths, treatment time ≥16 weeks, and objective evaluation for hair regrowth. RESULTS: Ten randomized controlled trials were included, of which 8 compared LLLT to sham device and 1 to no treatment. The study populations varied, with 3 studies evaluating only women. All sham-device controlled studies demonstrated statistically significant increase in hair diameter or density (p < 0.01) following LLLT. DISCUSSION: Based on our review of the literature, LLLT appears to be effective for treating pattern hair loss in both men and women. These laser devices have good safety profiles, with only minor adverse effects reported. However, physicians should be cautious when drawing conclusions as some studies included have a relationship with industry.

The importance of caveolins and caveolae to dermatology: Lessons from the caves and beyond.

Caveolae are flask-shaped invaginations of the cell membrane rich in cholesterol and sphingomyelin, with caveolin proteins acting as their primary structural components that allow compartmentalization and orchestration of various signalling molecules. In this review, we discuss how pleiotropic functions of caveolin-1 (Cav1) and its intricate roles in numerous cellular functions including lipid trafficking, signalling, cell migration and proliferation, as well as cellular senescence, infection and inflammation, are integral for normal development and functioning of skin and its appendages. We then examine how disruption of the homeostatic levels of Cav1 can lead to development of various cutaneous pathophysiologies including skin cancers, cutaneous fibroses, psoriasis, alopecia, age-related changes in skin and aberrant wound healing and propose how levels of Cav1 may have theragnostic value in skin physiology/pathophysiology.

Prevalence and Risk Factors for Anemia in a Population With Hidradenitis Suppurativa.

BACKGROUND:  Hidradenitis suppurativa (HS) is an inflammatory disease presenting as nodules evolving into scarred plaques. HS is associated with many co-morbidities, including anemia of chronic disease, though few studies report on this association. OBJECTIVES: This study evaluated the prevalence of anemia among the HS patient population and potential associations between risk factors for HS and anemia development. METHODS: Records diagnosed HS patients in one private practice (BR) were reviewed by investigators. The 92-patient cohort was evaluated for multiple data-points and responses grouped based on age, gender, ethnicity, body mass index (BMI), smoking status, and comorbidities. Data were analyzed using STATA to perform descriptive analysis and bivariate analysis. RESULTS: The prevalence of anemia in this cohort was 41.3%. Of anemic patients, the majority were men (65.2%), African American (60.6%), and never/former smokers (48.6%). There was a significant increase in the odds of developing anemia in HS patients that are men (odds ratio (OR) 3.8) and African American (OR 3.5). CONCLUSIONS: We show that the prevalence of anemia in an HS patient population greatly surpasses that of the U.S. population (~5%). It is clear that anemia is a significant complication for HS patients. We hope that physicians can recognize the importance of screening patients with HS for anemia to medically optimize treatment for their patients.

dsRNA Released by Tissue Damage Activates TLR3 to Drive Skin Regeneration.

Regeneration of skin and hair follicles after wounding--a process known as wound-induced hair neogenesis (WIHN)--is a rare example of adult organogenesis in mammals. As such, WIHN provides a unique model system for deciphering mechanisms underlying mammalian regeneration. Here, we show that dsRNA, which is released from damaged skin, activates Toll-Like Receptor 3 (TLR3) and its downstream effectors IL-6 and STAT3 to promote hair follicle regeneration. Conversely, TLR3-deficient animals fail to initiate WIHN. TLR3 activation promotes expression of hair follicle stem cell markers and induces elements of the core hair morphogenetic program, including ectodysplasin A receptor (EDAR) and the Wnt and Shh pathways. Our results therefore show that dsRNA and TLR3 link the earliest events of mammalian skin wounding to regeneration and suggest potential therapeutic approaches for promoting hair neogenesis.