ABSTRACT
Vacuoles, E1 enzyme, x-linked, autoinflammatory, and somatic mutation (VEXAS) syndrome is a recently described disease associated with high morbidity and mortality. VEXAS syndrome results from a somatic mutation affecting UBA1, a gene that codes for the E1 ubiquitin activating protein. Loss of UBA1 leads to a broad range of inflammatory conditions and a clinical course often refractive to therapy. We present the cases of two patients who demonstrated a rapid decline in overall health, decreased energy, arthralgias, anemia, fever, increased inflammatory markers, and characteristic bone marrow. Importantly, dermatologic assessment revealed skin biopsy findings of medium-vessel vasculitis and neutrophilic infiltration. Following blood analysis, both patients were diagnosed with VEXAS syndrome resulting from a mutation in the UBA1 gene. Our report highlights the pivotal role dermatologists have in early diagnosis of patients with VEXAS syndrome.
Subject(s)
Dermatology , Ubiquitin-Activating Enzymes , Humans , Ubiquitin-Activating Enzymes/genetics , Ubiquitin-Activating Enzymes/metabolism , Ubiquitin/genetics , Ubiquitin/metabolism , Skin/metabolism , MutationABSTRACT
OBJECTIVES: To evaluate long term effectiveness and safety with the use of a combined radiofrequency and optical home hair removal device in all skin types and over both facial and body areas. METHODS: A combined intense pulsed light (IPL) and radiofrequency (RF) home device was used for hair removal. Two separate study designs were evaluated. In the first design, bilateral body areas were treated with 7 weekly procedures and then unilateral areas received up to 12 monthly maintenance treatments while the corresponding other side received no further therapy. For the second design, facial areas received 4 procedures in 2 to 5 days visits, followed by 2 weekly, and then 6 biweekly procedures. All patients in both study designs had hair count evaluations up to 12 months post their last procedure. The highest energy used was 4 J/cm². RESULTS: In the first design, 58 subjects participated of which 15% were skin types V-VI. A total of 188 anatomical sites were treated. Hair reduction at 6 and 12 months post the last procedure was 56% and 52% respectively for the maintenance side and 47% and 37% for the non-maintenance side. For the second design, 19 subjects had 59 facial sites treated. Fifteen percent were skin types V –VI. The overall hair reduction at the end of receiving their procedures was 45%. At 12 months post the last procedure, the hair reduction was 42%. Aside for transient mild erythema, there were no adverse effects noted. CONCLUSIONS: Long term persistent hair reduction was achieved using an RF/IPL home-use hair reduction device. The combination of RF and optical energies allowed for the effective application of low energy usage. All skin types and both facial and body anatomical regions tolerated the procedures very well. J Drugs Dermatol. 2020;19(5):498-503. doi:10.36849/JDD.2020.4741.
Subject(s)
Erythema/diagnosis , Hair Removal/instrumentation , Hair/radiation effects , Light/adverse effects , Radio Waves/adverse effects , Adolescent , Adult , Aged , Erythema/etiology , Female , Follow-Up Studies , Hair/diagnostic imaging , Hair Removal/adverse effects , Hair Removal/methods , Humans , Male , Middle Aged , Photography , Severity of Illness Index , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: Home hair removal devices are available for skin types I to IV. Side effects may limit hair removal in darker-pigmented individuals. OBJECTIVE: To evaluate a home hair removal device using combined radiofrequency (RF) and intense pulsed light (IPL) energy for effectiveness and safety with all skin types (I-VI). DESIGN: Two study designs: effectiveness (treating 94 bilateral patient areas weekly seven times, with one side then receiving three additional treatments at 4-week intervals) and safety (37 patient areas treated every 2-4 days for three sessions). MEASUREMENTS: Hairs were counted 3 months after treatment for the first design and 2 months after for the second. RESULTS: In the first study design, 55% hair count reduction was achieved1 month after seven treatments to all sites. The side with no further sessions had 43% hair reduction and the side receiving ongoing treatment had 58% reduction after two further treatments. Side effects were transient and minor. In the second study, with 46% of the patients having skin types V/VI, 2 months after the procedure there was 44% hair reduction. There were no adverse effects reported. CONCLUSION: For the first time, a home hair removal device has been shown to be effective and safe in all skin types using a low-energy RF-IPL device.
Subject(s)
Hair Removal/instrumentation , Phototherapy/instrumentation , Radiofrequency Therapy , Self Care , Adult , Female , Humans , Male , Middle Aged , Treatment OutcomeABSTRACT
Wells syndrome, or eosinophilic cellulitis, is a rare disorder characterized by erythematous plaques evolving into dermal or subcutaneous masses. The histopathology shows degenerative collagen, histiocytes, and eosinophils. Although most cases of Wells syndrome occur in adults, we present a child with these clinical and histopathologic findings.
