Deployed Airbag Causes Bullous Reaction Following a Motor Vehicle Accident.

Airbags can be lifesaving during a motor vehicle accident (MVA), but airbag deployment has been the cause of dermatologic injuries including irritant dermatitis, as well as thermal, friction, and chemical burns. A highly corrosive alkaline aerosol composed of sodium hydroxide, sodium bicarbonate, and metallic oxides is released during airbag deployment. We present the case of a 35-year-old man who developed a bullous reaction to released by-products from airbag deployment during an MVA.

A Panoply of Rheumatological Manifestations in Patients with GATA2 Deficiency.

PURPOSE: To characterize rheumatological manifestations of GATA2 deficiency. METHODS: Single-center, retrospective review of 157 patients with GATA2 deficiency. Disease course, laboratory results, and imaging findings were extracted. In-person rheumatological assessments were performed on selected, available patients. A literature search of four databases was conducted to identify additional cases. RESULTS: Rheumatological findings were identified in 28 patients, out of 157 cases reviewed (17.8%). Twenty-two of those patients (78.6%) reported symptom onset prior to or in conjunction with the molecular diagnosis of GATA2 deficiency. Notable rheumatological manifestations included: piezogenic pedal papules (PPP), joint hyperextensibility, early onset osteoarthritis, ankylosing spondylitis, and seronegative erosive rheumatoid arthritis. In peripheral blood of patients with rheumatological manifestations and GATA2 deficiency, CD4+ CD3+ helper T cells and naïve CD3+ CD4+ CD62L+ CD45RA+ helper T cell subpopulation fractions were significantly lower, while CD8+ cytotoxic T cell fractions were significantly higher, compared to those without rheumatological manifestations and with GATA2 deficiency. No changes in CD19, CD3, or NK populations were observed. CONCLUSION: GATA2 deficiency is associated with a broad spectrum of rheumatological disease manifestations. Low total helper T lymphocyte proportions and low naïve helper T cell proportions are associated with those most at risk of overt rheumatological manifestations. Further, PPP and joint hyperextensibility may explain some of the nonimmunologically-mediated joint problems encountered in patients with GATA2 deficiency. This catalogue suggests that rheumatological manifestations and immune dysregulation are relatively common in GATA2 deficiency.

Atopic dermatitis in adolescents with skin of color.

Atopic dermatitis (AD) is a common chronic inflammatory dermatosis of childhood that typically improves during adolescence but can be lifelong. Black children are at 6-times greater risk for severe AD than white children, and AD may lead to a greater negative impact on quality of life (QOL) in patients with skin of color. For these reasons, it is important for dermatologists to consider the various issues associated with AD that adolescents with skin of color may face. We present management strategies for AD in this patient population.

Gorlin syndrome in a patient with skin type VI.

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant disorder that is characterized by multiple basal cell carcinomas developing at a young age, keratocystic odontogenic tumors of the jaw, palmar or plantar pits, calcification of the falx cerebri, and skeletal abnormalities. Nevoid basal cell carcinoma syndrome is caused by mutations in the PTCH1 or SUFU genes. Our patient with Fitzpatrick skin type VI was diagnosed with Gorlin syndrome based on the presentation of multiple major diagnostic characteristics. Although he is 33 years old, he has not developed any multiple basal cell carcinomas to date.