Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise.

Importance: Scoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent. Objectives: To establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN. Evidence Review: A Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement. Findings: In round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated. Conclusions and Relevance: This consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.

Nivolumab-Induced de novo Discoid Lupus Erythematosus.

An increasing number of checkpoint inhibitor-induced subacute cutaneous lupus erythematosus events have been reported. We present the first case of nivolumab-induced discoid lupus erythematosus in a patient with hepatocellular carcinoma. The patient presents with violaceous hypopigmented plaques on the pinna bilaterally, hypopigmented plaques with central hyperpigmentation on the posterior neck, and other hypopigmented plaques on the face, forearms, and hands. For management, nivolumab was held for 2 months, and Plaquenil and topical steroids were added. Nivolumab was resumed with no further progression of DLE lesions and improvement of the skin. It is important to characterize cutaneous side effects to effectively manage them.

Case Series of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis With Nivolumab and Nivolumab/ Ipilimumab Combination Therapy in Metastatic Melanoma.

Nivolumab (anti PD-1 antibody) and ipilimumab (anti CTLA-4 antibody) are immune checkpoint inhibitors (ICI) that effectively stimulate the native T cell response and lead to an antitumor response. The medications have been approved for the treatment of metastatic melanoma. However, ICIs are associated with higher risk for cutaneous immune-related adverse events (irAEs). Although most of the adverse events present as maculopapular rash, some patients develop Stevens-Johnson Syndrome (SJS) or Toxic Epidermal Necrolysis which are dermatologic emergencies with high mortality. We report a fatal case of SJS associated with nivolumab and a non-fatal case of TEN with nivolumab/ipilimumab combination therapy in patients with metastatic melanoma. It is also not unusual to develop SJS or TEN after weeks or months on checkpoint inhibitor therapy. Given the high rate for mortality, dermatologists and other clinicians should closely follow any rash from these immunotherapies due to the risk for future development of SJS or TEN. J Drugs Dermatol. 2022;21(5):529-530. doi:10.36849/JDD.6559.

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.

Cutaneous Lupus: A Brief Review of Old and New Medical Therapeutic Options.

Systemic lupus erythematosus is a chronic inflammatory condition which affects predominantly women in their 30s. It has several clinical manifestations, including skin lesions that can be classified as acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. A multifaceted approach to treating cutaneous lupus is advocated.

Coexisting Sickle Cell Anemia and Sarcoidosis: A Management Conundrum!

Sickle cell disease and Sarcoidosis are conditions that are more common in the African American population. In this report we share an unfortunate patient who had hepatic sarcoidosis but could not receive steroids since that precipitated acute liver failure. We have discussed potential therapy options but we need more options that improve mortality.