Long-term Physical and Psychological Outcomes of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

IMPORTANCE: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is known to cause multiple end-organ complications in its acute phase, but less is known about the long-term association with patients' mental health and quality of life. OBJECTIVE: To examine the chronic physical and psychological sequelae affecting patients with SJS/TEN. DESIGN, SETTING, AND PARTICIPANTS: A survey study conducted at 11 academic health centers in the US evaluated 121 adults diagnosed with SJS/TEN by inpatient consultive dermatologists between January 1, 2009, and September 30, 2019. INTERVENTIONS: Patients completed a survey that included the following validated questionnaires: Patient Health Questionnaire-9 (PHQ-9), Generalized Anxiety Disorder-7 (GAD-7), Primary Care Post-Traumatic Stress Disorder Screen (PC-PTSD), and the 12-item Short Form Health Survey (SF-12). The survey also included questions created by the study team regarding fear, patient education, and long-term sequelae relevant to SJS/TEN. MAIN OUTCOMES AND MEASURES: Primary outcome measures were the percentage of patients reporting long-term physical sequelae; the percentage of patients with positive results on PHQ-9, GAD-7, and PC-PTSD screening; and the numeric score on the SF-12 (score of 50 defined as average physical and mental well-being). RESULTS: A total of 121 individuals (73 women [60.3%]; mean [SD] age, 52.5 [17.1] years) completed the survey (response rate, 29.2%). The most common long-term physical sequelae reported were cutaneous problems (102 of 121 [84.3%]), ocular problems (72 of 121 [59.5%]), and oral mucosal problems (61 of 120 [50.8%]). A total of 53.3% (64 of 120) of the respondents had results indicating depression on the PHQ-9, 43.3% (52 of 120) showed signs of anxiety on the GAD-7, and 19.5% had results indicating PTSD on the PC-PTSD. The mean (SD) SF-12 Physical Component Summary score was 42.4 (22.8), and the mean Mental Component Summary score was 46.1 (20.9). A total of 28.2% (33 of 117) of the respondents were unable to work, 68.1% (81 of 119) were fearful of taking new medications, and 30.0% (36 of 120) avoided taking prescribed medications for a diagnosed medical condition. CONCLUSIONS AND RELEVANCE: This survey study found that long-term physical sequelae, depression, and anxiety appear to be common in patients with SJS/TEN, with implications for health and well-being. Improved awareness of these complications may assist health professionals in offering medical care, counseling, and support to patients with SJS/TEN.

Teledermatology Consultation Can Optimize Treatment of Cutaneous Disease by Nondermatologists in Under-Resourced Clinics.

Background: Access to dermatologists is limited for disadvantaged patients, who may receive suboptimal dermatologic care from nonspecialists. We assessed if teledermatology could improve primary care provider (PCP)-delivered care for cutaneous disease at a clinic serving uninsured patients. Materials and Methods: Utilizing the American Academy of Dermatology's free AccessDerm program, we offered store-and-forward teledermatology to PCPs, who initiated consultations at will during clinical care independent of the study. We retrospectively analyzed all consultations from 2013 to 2017 and collected patient age/sex, teledermatologist diagnosis, time to teledermatologist reply, time to next dermatology appointment, as well as PCP- and teledermatologist-proposed care plans. Results: Retrospective analysis of 131 consults revealed a 37-h mean teledermatology response-time versus a 14-day appointment wait (p < 0.00001). Teledermatologists provided a definitive care plan without in-person evaluation for 82 (65%) of completed consults and recommended interim treatments while awaiting appointments in 15 cases, thus accelerating care plan delivery in 97 cases (76%). The triage decision rate differed among diagnostic categories; deferral to in-person evaluation was more frequent for neoplasms (p < 0.0001). When PCPs specified preconsult treatment plans, 82% differed from teledermatologist-advised management. Following teledermatologist recommendations would have changed the clinical course in 70% of cases, potentially avoiding suboptimal care, including inappropriate corticosteroids, antimicrobials, and emergency room referrals. Conclusions: We found teledermatology can effectively guide PCPs in resource-limited settings by accelerating delivery of dermatologist-recommended care plans for uninsured patients. Expanding teledermatology for PCPs in under-resourced clinics has the potential to improve treatment of cutaneous disease by nonspecialists and to mitigate suboptimal care for disadvantaged patients.

Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index.

BACKGROUND: Limited studies describe the longitudinal course of cutaneous dermatomyositis (DM). OBJECTIVE: To characterize disease course in treated cutaneous DM by using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a validated outcome instrument. METHODS: A retrospective cohort included patients with DM who had their CDASI activity subscore recorded for at least 2 years. Disease progression was classified as improved, worsened, or stable, and disease course was classified as monophasic, polyphasic, or chronic. Subjects were divided into groups with disease of mild (n = 16) and moderate-to-severe (n = 24) baseline severity. RESULTS: A total of 40 patients with DM met inclusion criteria. The majority demonstrated improvement in disease activity (n = 21 [52.5%]) rather than worsening (n = 7 [17.5%]) and stable (n = 12 [30.0%]) disease. Most patients with mild disease remained stable (n = 10 [62.5%]), whereas most with moderate-to-severe disease improved (n = 19 [79.2%]). A polyphasic course (n = 33 [82.5%]) predominated over monophasic (n = 5 [12.5%]) and chronic (n = 2 [5%]) courses. The average number of flares per year per number of years of follow-up was independent of baseline disease activity. LIMITATIONS: The retrospective design, potential referral bias, and cutoff values in classification criteria are limitations. CONCLUSION: Baseline CDASI activity score is associated with particular patterns of disease course and progression in cutaneous DM.

Accidental hydroxychloroquine overdose resulting in neurotoxic vestibulopathy.

Hydroxychloroquine is an oral antimalarial medication commonly used off-label for a variety of rheumatological conditions, including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome and dermatomyositis. We present a case of a 64-year-old woman who presented with acute onset headache, bilateral tinnitus, and left-sided facial numbness and tingling in the setting of accidentally overdosing on hydroxychloroquine. By the next morning, the patient began to experience worsening in the tingling sensation and it eventually spread to her left arm, thigh and distal extremities. The patient also complained of new onset blurring of her peripheral vision and feeling 'off balance.' Despite a complete neurological and ophthalmological work-up with unremarkable imaging and blood work, the patient has had no improvement in her tinnitus, left-sided paresthesias, visual disturbance or ataxia. This is a unique case of hydroxychloroquine overdose resulting in permanent neurotoxic vestibulopathy.