Afamelanotide for Treatment of the Protoporphyrias: Impact on Quality of Life and Laboratory Parameters in a US Cohort.

BACKGROUND: Erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) are rare disorders of heme biosynthesis characterized by severe cutaneous phototoxicity. Afamelanotide, an α-melanocyte-stimulating hormone analogue, is the only approved treatment for protoporphyria and leads to increased light tolerance and improved quality of life (QoL). However, published experience with afamelanotide in the US is limited. METHODS: Here, we report on all adults who received at least one dose of afamelanotide at the Massachusetts General Hospital Porphyria Center from 2021 to 2022. Changes in the time to phototoxic symptom onset, QoL, and laboratory parameters were assessed before and during treatment with afamelanotide. RESULTS: A total of 29 patients with protoporphyria were included, 26 of whom (72.2%) received ≥2 afamelanotide implants. Among the patients who received ≥2 implants, the median time to symptom onset following sunlight exposure was 12.5 min (IQR, 5-20) prior to the initiation of afamelanotide and 120 min (IQR, 60-240) after treatment (p < 0.001). Improvements in QoL during afamelanotide treatment were measured using two QoL tools, with good correlation observed between these two instruments. Finally, we found no improvements in the median levels of metal-free erythrocyte protoporphyrin, plasma protoporphyrin, or liver biochemistries during versus prior to the initiation of afamelanotide treatment. CONCLUSIONS: This study highlights a dramatic clinical benefit of afamelanotide in relation to light tolerance and QoL in protoporphyria, albeit without improvement in protoporphyrin levels or measures of liver function.

Poppers Dermatitis: A Systematic Review on a Unique Form of Contact Dermatitis in the MSM Community.

There are certain demographic characteristics that may serve as risk factors for exposure to a contact allergen. Volatile alkyl nitrites, colloquially known as "poppers," are commonly inhaled by men who have sex with men (MSM) for their psychoactive and muscle-relaxing effects. They have been reported to cause either allergic contact dermatitis (ACD) or irritant contact dermatitis (ICD), termed "poppers dermatitis." We searched PubMed, Embase, and Web of Science to understand the patient population, clinical presentation, diagnosis, and clinical course of poppers dermatitis. Our search returned 13 articles detailing 18 patients total. Most patient cases were male (n = 13/15; 87%), of whom 5 were MSM. The reports on the remaining 8 men did not disclose their sexual behaviors. The chemicals deemed responsible for adverse cutaneous side effects included amyl nitrite (6/18; 33%), butyl nitrite (3/18; 167%), isobutyl nitrite (2/18; 11%), and an unspecified alkyl nitrite (7/18, 39%). The most common diagnosis was ICD (10/18; 56%). Only 3 patients underwent alkyl nitrite patch testing, with 2 testing positive for ACD. The most commonly involved anatomic sites were perinasal (13/18; 72%) and perioral regions (10/18; 56%). Rash morphology has been described as yellow crusting, impetigo-like, eczematous, vesicular, edematous, and erythematous. Ulceration can occur with direct skin contact to concentrated nitrite solution. Poppers dermatitis was often self-limited with complete resolution. Dermatologists should consider poppers dermatitis in those presenting with a characteristic midfacial rash, especially in MSM.

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.

Perceptions of telehealth among inpatient consultative dermatology providers and practice patterns during COVID-19.

Use of inpatient teledermatology increased during the COVID-19 pandemic. We surveyed the Society for Dermatology Hospitalists to better characterize the impact of COVID-19 on teledermatology use by inpatient dermatology providers, particularly on provider perceptions of teledermatology. Prior to the COVID-19 pandemic, 40% (8/20) of surveyed providers had used telehealth at their institution to help perform inpatient consults, while 90% (18/20) adapted use of teledermatology during the pandemic. 80% (16/20) reported that their opinion of teledermatology changed as a result of the COVID-19 pandemic, with the vast majority (87.5%, 14/16) reporting having a more positive opinion. Benefits of teledermatology included efficiency, ability to increase access safely, and ability for clinicians to focus on complex cases. Some providers expressed concerns over the potential implications regarding the perception of dermatology within medicine, limitations of inadequate photos, and breakdowns in communication with consulting teams and patients. Robust algorithms and or utilization criteria of teledermatology may help to mitigate risk, while increasing access to inpatient dermatologic evaluation.

A description of Kaposi sarcoma risk factors and outcomes in HIV-positive and HIV-negative patients at a tertiary care medical center from 2005 to 2020.

Kaposi sarcoma (KS) is a low-grade vascular malignancy caused by human herpesvirus-8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). There are four established subtypes of KS, which are described by unique risk factors, presentation, and disease course. A "non-epidemic" variant to describe HIV-negative men who have sex with men (MSM) is emerging as a fifth subtype. We retrospectively examined patients with KS at an academic medical center in central Ohio, USA. To our knowledge, this is the first US-based report to describe KS risk factors and outcomes in the context of HIV status. Data were extracted from patient charts including demographic information, history at time of KS diagnosis, and information about KS disease course. HIV-positive and HIV-negative patients were grouped into established categories. HIV-negative patients who did not fit an existing subtype were described as "Unclassified-KS-Type." Demographic characteristics for AIDS-KS patients in our cohort match established trends in this subtype, such as male, MSM, and younger age at diagnosis compared to HIV-negative patients. Most Unclassified-KS-Type patients fit well into the emerging "non-epidemic KS" subtype. These patients are described as healthy, middle-aged, HIV-negative MSM with lower extremity lesions. This descriptive report provides an updated view of KS risk factors and outcomes to improve detection and treatment in dermatology.

Hospital triage and skin disease: hospital outcomes are differentially associated with cutaneous morphology.

BACKGROUND: Determining the exact dermatologic diagnosis is difficult in the inpatient setting. OBJECTIVE: Determine whether morphologic classification rather than specific diagnosis is associated with hospital outcomes. METHODS: Retrospective single-center study. Information from 1798 inpatient dermatology consults at The Ohio State University Wexner Medical Center from 2012 to 2014 was queried. Dermatologic diseases were categorized into 16 groups based on appearance. Logistic regression was performed comparing mortality rate vs morphology. Linear regression was performed comparing the length of stay (LOS) vs morphology. RESULTS: Morphology was associated with a mortality rate (P = 0.038). The morphologic subgroups acneiform/follicular/occlusion (P = 0.011), blistering disorders (P = 0.009), retiform purpura (P = 0.011), and vasculitis/vascular (P = 0.007) were associated with increased mortality. Morphology was associated with LOS (P = 0.004), and the morbilliform subgroup was associated with increased LOS (P < 0.001). CONCLUSION: This study demonstrated the importance of morphologic diagnosis and its association with mortality rate and LOS. This information may help triage cutaneous disorders in the inpatient setting and determine the relative risk of dermatologic conditions when assessing the need for hospital transfers and more aggressive therapies.

Characterizing risk factors for hospitalization for psoriasis patients.

Psoriasis is a chronic autoimmune disease with a large economic impact. Inpatient care is a significant expense, and about one-third of patients admitted for psoriasis are readmitted. Reducing hospitalizations and readmissions is an important goal for improving outcomes for psoriasis patients. The objective of this study is to characterize patients who are hospitalized for psoriasis, and differentiate features for patients with a single hospitalization from those who were hospitalized multiple times during the study period. Hospitalized psoriasis patients were identified from an in-patient database at a single academic institution. Differences between psoriasis patients with one hospitalization and those with multiple hospitalizations were characterized, as were differences between patients who were hospitalized primarily for psoriasis and those who were admitted primarily for other reasons. Patients who were primarily hospitalized for psoriasis had fewer comorbidities, shorter hospitalizations, and a lower death rate than those hospitalized for other reasons. Patients with multiple hospitalizations had more comorbidities and worse outcomes than patients with a single hospitalization. Patients who are hospitalized primarily for psoriasis are more likely to be pustular, and tend to have fewer comorbidities and better outcomes than patients with psoriasis who are hospitalized with psoriasis as a secondary diagnosis. One limitation of this study is the lack of data available to consistently quantify disease severity, such as percent of body surface area affected by psoriasis or Physician's Global Assessment score.

Mpox in Young Woman with No Epidemiologic Risk Factors, Massachusetts, USA.

We describe a case of mpox characterized by a circularly distributed facial rash but no identified risk factors. Fomite transmission of monkeypox virus from contaminated linen at a massage spa was suspected. Clinicians should consider mpox in patients with consistent clinical syndromes, even in the absence of epidemiologic risk factors.

Factitial dermatitis in the hospital setting.

Factitial Dermatitis (FD) is a notoriously difficult disease to diagnose, as patients produce self-induced cutaneous lesions and provide an inadequate or inaccurate history. We performed a cross-sectional study, querying an inpatient consultation database of all patients admitted to the Ohio State University Wexner Medical Center from 2012 to 2017 with a dermatologic ICD as a discharge diagnosis. Our exhaustive keyword search produced 189 candidates. Consult notes were thoroughly examined, and 32 patients were found to meet case definition of FD. Our analysis of this cohort revealed a significantly greater proportion of cases in the female population. Lesions were more often found to involve the skin on the upper extremities. Isolated secondary skin changes such as erosions, ulcers and excoriations in the absence primary morphologies were also significant in our cohort. As FD is difficult to identify, further understanding of its presentation pattern will decrease time to diagnosis and improve both hospital resource allocation and patient care.