Blowing Bubbles: Dermoscopy of Bubble Hair.

Exposing wet hair to high temperatures can create gas bubbles within the hair shaft, leading to brittle, dry hairs in a disorder known as bubble hair abnormality. We present a case of a 61-year-old woman who presented for hair breakage over her crown. She regularly dried her damp hair with a blow dryer. Dermoscopy revealed multiple bubbles within the hair shaft, and diagnosis of bubble hair abnormality was confirmed by light microscopy. Our unusual case highlights the ease of acquisition of this abnormality by means of a common hair dryer, and the utility of dermoscopy to make a fast and accurate diagnosis within the office.

Developing biomarkers for predicting clinical relapse in pemphigus patients treated with rituximab.

BACKGROUND: Rituximab is an effective therapy for pemphigus, although relapses are common. OBJECTIVE: To identify biomarkers to predict relapse of pemphigus following rituximab treatment. METHODS: In this retrospective cohort study, 62 patients with pemphigus treated with 99 rituximab cycles provided longitudinal clinical scoring and biomarker data, including levels of CD19+ B cells, CD4+ T cells, and desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3) autoantibodies. An extended time-variant Kaplan-Meier estimator and extended Cox model were applied. RESULTS: Relapse was rare before B-cell repopulation. Univariate analysis revealed low CD4 count (<400 cells/µL) to predict relapse (P < .001). A positive result of testing for Dsg1 (>20 IU) was predictive of relapse among patients with mucocutaneous disease (hazard ratio, 6.40; P = .019); a positive result of testing for Dsg3 (>20 IU) was predictive in patients with mucocutaneous and mucosal disease (hazard ratio, 32.92; P < .001). Multivariable analysis revealed that every CD4 value increase of 200 decreases the hazard ratio for relapse by 35% (P = .029). A positive result of testing for Dsg1 increases the risk for relapse by a factor of 12.32 in patients with mucocutaneous disease (P = .001); positive result of testing for Dsg3 increases risk for relapse by 28.38 in patients with mucosal and mucocutaneous disease (P = .006). LIMITATIONS: Limitations include the retrospective design and inconsistent follow-up. CONCLUSION: Relapse is associated with B-cell repopulation, low CD4+ T -cell count, and positive result of testing for Dsg1 and Dsg3.

Treatment of Hailey-Hailey Disease With Low-Dose Naltrexone.

Importance: Hailey-Hailey disease is a severe genetic blistering disease of intertriginous skin locations that can lead to poor quality of life and increased morbidities. Multiple therapies are available with inconsistent outcomes and potentially severe adverse effects. Objective: To determine whether low-dose naltrexone is an effective treatment for Hailey-Hailey disease. Design, Setting, and Participants: This study was a case series performed at a dermatology outpatient clinic of 3 patients with severe Hailey-Hailey disease recalcitrant to at least 4 therapies. Interventions: Low-dose naltrexone, 3 mg nightly, titrated to 4.5 mg nightly in 2 patients. Main Outcomes and Measures: Reduction in size of lesions as well as subjective improvement of symptoms. Results: All 3 patients noted significant healing of erosions and plaques starting from the peripheral aspect within 1 to 2 weeks of treatment, and clinical resolution of lesions within 2 months. Discontinuation of low-dose naltrexone resulted in flaring of symptoms, which cleared within 2 to 3 days on rechallenge with low-dose naltrexone. Conclusions and Relevance: We present herein 3 cases of patients with severe Hailey-Hailey disease treated with low-dose naltrexone who achieved clinical resolution of symptoms. The success of these cases suggests low-dose naltrexone as a novel therapy for Hailey-Hailey disease. The possible mechanism may involve low-dose naltrexone influencing opioid or toll-like receptor signaling to improve calcium mobilization and improve keratinocyte differentiation and wound healing. Future studies are needed to clarify the mechanism and to define the role of low-dose naltrexone for treatment of Hailey-Hailey disease.

Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis.

Importance: Dermatitis herpetiformis (DH) is an autoimmune blistering condition seen in the context of celiac disease. While typically managed by gluten-free diet and dapsone, treatment of DH refractory to standard treatments is not well defined. Observations: A man in his 80s with DH not controlled by gluten-free diet (with poor adherence), dapsone, and conventional immune-suppressing agents responded to treatment with rituximab according to the lymphoma protocol (4 weekly infusions of 375 mg/m2). Thirteen months after treatment, the patient had achieved complete resolution of pruritus and clinical manifestations of the disease, as well as normalization of antibodies against epidermal and tissue transglutaminases. He achieved complete clinical and serological remission and has remained symptom-free up to 18 months following treatment. Conclusions and Relevance: We present here the first case of a patient with DH treated with rituximab who achieved complete clinical and serological remission. We suggest rituximab as a viable treatment option for recalcitrant DH.