Skin-Related Quality of Life During Autoimmune Bullous Disease Course.

Importance: Autoimmune bullous diseases (AIBDs) are chronic relapsing-remitting conditions with significant morbidity. Skin-related quality of life (SRQL) may vary by AIBD subtype and disease type. Disease severity and flare severity can be difficult to define; SRQL can offer a key insight. Objectives: To investigate the Skindex-16 score as an SRQL measure in AIBD subtypes during flare and nonflare states and to evaluate Skindex-16 construct validity. Design, Setting, and Participants: This retrospective cross-sectional study was conducted from September 1, 2016, to February 1, 2020, among 192 patients at the University of Utah Health autoimmune dermatology clinic with pemphigoid, pemphigus, dermatitis herpetiformis, and linear immunoglobulin A disease. Patients had an encounter-associated diagnosis, Skindex-16 scores, and self-reported flare status. Statistical analysis was performed from March 2022 to June 2023. Exposure: Autoimmune bullous disease subtype and patient-reported flare status. Main Outcomes and Measures: Skindex-16 domain scores (emotions, symptoms, and functioning; range, 0-100, where 0 indicates no effect on SRQL and 100 maximum effect) and individual item scores were described by disease and flare status. Flare scores were expected to be higher by at least the standard error of measurement (SEm). Convergent validity was assessed using Spearman correlation among Skindex-16 scores, serologic titers, and other patient-reported outcome measures. Floor or ceiling domain scores (<20% of sample scoring either lowest or highest possible domain scores, respectively) were assessed for Skindex-16. Structural validity was assessed using confirmatory factor analysis (CFA). Results: The study included 192 patients with 212 visits (median age, 68 years [IQR, 58-76 years]; 123 of 212 women [58.0%]) with Skindex-16 scores (64 in flare state and 148 in nonflare state). Median Skindex-16 domain scores were higher for all disease categories among patients in the flare state compared with those in the nonflare state (pemphigoid [emotions: flare, 52.4 (IQR, 38.1-69.0); nonflare, 7 (IQR, 0-17); symptoms: flare, 37.5 (IQR, 29.2-58.0); nonflare, 13 (IQR, 0-25); functioning: flare, 26.7 (IQR, 10.0-56.7); nonflare, 0 (IQR, 0-3)]; pemphigus [emotions: flare, 54.8 (IQR, 31.0-81.0; nonflare, 0 (IQR, 0-19); symptoms: flare, 58.3 (IQR, 41.7-70.8); nonflare, 4 (IQR, 0-12.5); functioning: flare, 26.7 (IQR, 13.3-83.3); nonflare, 0 (IQR, 0-3.33)]; dermatitis herpetiformis [emotions: flare, 72.6 (IQR, 34.7-90.5); nonflare, 14.3 (IQR, 2.4-26.2); symptoms: flare, 69 (IQR, 31.3-85.4); nonflare, 12.5 (IQR, 0-29.2); functioning: flare, 38.3 (IQR, 5.0-63.2); nonflare, 0 (IQR, 0-13.3)]. This difference exceeded SEm cut points. Cronbach α was greater than 0.80 for all domains and AIBDs. Moderate or low correlations were seen with desmoglein 1 and bullous pemphigoid 180 titers. Moderate correlation existed between Skindex-16 and Patient-Reported Outcomes Measurement Information System Depression scores (emotions: ρ = 0.40; symptoms: ρ = 0.41; functioning: ρ = 0.48), and strong correlation existed between Skindex-16 and patient-reported disease severity (emotions: ρ = 0.71; symptoms: ρ = 0.73; functioning: ρ = 0.66). Floor domain scores greater than 20% were seen among patients in the nonflare state, but ceiling domain scores were rare (<10% for all domains); CFA model fit was poor. Conclusions and Relevance: In this cross-sectional study, SRQL was highly associated with flare of AIBDs. Skin-related quality of life was worse during periods without flare among patients with pemphigoid and dermatitis herpetiformis compared with pemphigus, highlighting residual SRQL morbidity. Skindex-16 showed good construct validity, but the poor CFA model fit needs further research. Clinical measurement of SRQL in AIBDs can add critical disease-severity information.

Atopic Dermatitis: A Qualitative Study on the Burdens of Living with Itchiness.

Objective: We sought to understand the consequences itchiness has on daily life that may not be immediately obvious in clinical assessments for patients with atopic dermatitis (AD). Methods: Focus groups and interviews involving 21 patients with AD and 12 family members examined aspects of the effects of itchiness on health-related quality of life (HRQL). Investigators conducted a thematic analysis where two researchers independently coded the narratives and arrived at a consensus on major themes. Results: Five themes emerged from our discussions. 1) Miserable experience: Itchiness was difficult to control and cease. 2) Physical damage: Damage to skin and hair occurred from scratching to alleviate the itchiness. 3) Effects on daily activities: Itchiness could affect everything participants did, including how they dressed, used make-up, and slept. 4) Effects on social activities and relationships: The discomfort and embarrassment from scratching in public and others' reactions hindered participants' social lives. 5) Emotional consequences: Various emotional responses to itchiness were reported, including embarrassment, depression, and irritation. Limitations: Though qualitative research provides a level of detail not often found in quantitative analyses, this study design is limited by small sample size and generalizability. Conclusion: Understanding these challenges can help clinicians open deeper conversations with their patients to learn more about what patients need from their dermatologic care. While itchiness from AD is well-known, this study shows that its effects on HRQL are not minimal and that patients may need further care for the consequences of this symptom.

Achieving consensus on patient-reported outcome measures in clinical practice for inflammatory skin disorders.

BACKGROUND: International Dermatology Outcome Measures and the American Academy of Dermatology recently agreed on a physician-reported global severity measure to demonstrate quality of care in inflammatory dermatoses. OBJECTIVE: Because patient-reported outcome measures are also important, we aimed to achieve consensus on 1 of these measures for clinical practice. METHODS: Patients and providers participated in a consensus-building study involving a modified-Delphi technique. Voting was focused on identifying a minimal set of assessments for clinical practice, patient global assessments (PtGAs), Skindex instruments, and final instrument selection for quality improvement. RESULTS: Among 53 stakeholders, >70% agreed that identification of patient goals, assessment of treatment harm, and assessment of the adequacy of treatment response were the minimal assessments needed for clinical practice. The most preferred PtGA was a 5-point PtGA (scale 0-4; 0 = clear, 4 = severe) having an optional check-box for worst ever. A new metric to assess change since treatment initiation, which we called a trajectory measure, was proposed. Stakeholders preferred Skindex instruments over PtGAs and a trajectory measure for clinical practice. LIMITATIONS: A small number of patients and caregivers were involved and a consensus was not reached on all items. CONCLUSION: PtGAs as standalone measures do not adequately capture the patient's assessment of disease severity or the effect of care. The combination of a PtGA with a Skindex instrument or another measure of health-related quality of life might provide a more comprehensive evaluation of patients in clinical practice.

Quality of Life Among Family of Patients with Atopic Dermatitis and Psoriasis.

BACKGROUND: Chronic inflammatory skin diseases like atopic dermatitis (AD) and psoriasis can severely impact patients' quality of life (QOL). However, the effect of these diseases can diminish the QOL of patients' family members as well. The objective of this study was to understand the impact on QOL for family members of patients diagnosed with AD or psoriasis. METHODS: We conducted focus groups and interviews with 23 individuals; 12 had a family member with AD, and 11 had a family member with psoriasis. After investigators independently coded the transcripts, thematic analysis was conducted. RESULTS: Three major themes emerged: (1) lifestyle consequences-many daily activities for family members, including but not limited to leisure activities, sleep, and cleaning, were affected by AD or psoriasis; (2) emotional consequences-family members felt frustrated, worried, or embarrassed, among other concerns, because of their loved ones' AD or psoriasis; (3) relationships-relationships between family members and their loved ones with AD or psoriasis could become strained, and though family members might try to be sympathetic, doing so could be difficult because of their lack of understanding of how these diseases feel and personally affect their loved ones. CONCLUSIONS: This study highlights the impacts of AD and psoriasis on the whole family. Clinicians should be mindful of the effects on QOL not only for patients but also for family members who live with and care about these patients. Especially when family members assist with treatments, it is important to understand family members' experiences when making treatment decisions.

Clinicians' perspectives on the integration of electronic patient-reported outcomes into dermatology clinics: a qualitative study.

BACKGROUND: Skin conditions can have profound negative symptomatic and psychological effects. Failure to address these effects can lead to poor treatment adherence and/or patient dissatisfaction. Despite patient-reported outcome (PRO) use being highly recommended, real-world adoption has been slow. OBJECTIVES: To assess clinicians' perceived facilitators and barriers to using PROs in daily practice. METHODS: We conducted in-person semi-structured interviews with 19 clinicians and thematic analysis of transcripts. RESULTS: Three main themes emerged: (1) clinicians' attitudes about the value of Skindex-16 in daily practice, (2) patient attitudes influencing clinicians' use of Skindex-16, and (3) clinicians' perceptions of their ability to use PROs successfully for clinical care. Clinicians recognized benefits to using Skindex-16, such as revealing patients' hidden concerns and highlighting discrepancies with the clinician's severity assessments. Conversely, clinicians also identified limitations, such as time constraints and lack of relevance for some skin conditions. Patient complaints about PRO relevance have influenced clinicians' use of Skindex-16 negatively. Finally, some clinicians recognized the need for more training in score interpretation and implementation strategies for optimal clinical flow. CONCLUSIONS: While most clinicians believed PROs like Skindex-16 can be useful for patient care, barriers need to be addressed to make PROs more practical for routine clinical care.

The Disruptiveness of Itchiness from Psoriasis: A Qualitative Study of the Impact of a Single Symptom on Quality of Life.

BACKGROUND: Pruritus is the most common symptom of psoriasis, with a significant impact on patient quality of life. In spite of this, the severity, persistence, and overall impact of itchiness has only been rarely formally assessed during standard psoriasis clinic visits. Objectives: We sought to understand the far-reaching impacts of itchiness on the lives of those with psoriasis and their families. METHODS: We conducted a qualitative study with five focus groups and 10 semi-structured interviews from August 2018 to January 2019. We enrolled 25 individuals with a diagnosis of at least moderate plaque psoriasis and 11 family members (primarily significant others). Views and experiences were analyzed thematically via content analysis. RESULTS: Itchiness considerably impacts those with plaque psoriasis and their families. Our narrative analysis produced three main themes relating to itchiness: the triggers of itchiness, including climate, emotions, and behaviors; the physical consequences of itchiness, including disruption of emotional well-being, sleep disturbance, and daily activities; and the prevention and treatment strategies used to alleviate itchiness. CONCLUSION: Itchiness impacts the quality of life in those with psoriasis and their family members. We strongly urge clinicians to inquire about and monitor the severity and impact of itchiness in psoriasis patients.

Effects of Pain From Atopic Dermatitis: Interview and Focus Group Study With Patients and Their Families.

BACKGROUND: Pain is an underappreciated symptom of atopic dermatitis that can affect the health-related quality of life (HRQL) of patients. OBJECTIVE: The aim of this study is to understand the effect of pain on patients with atopic dermatitis and their family members and to recognize how this symptom affects HRQL. METHODS: We conducted focus groups and interviews with patients with atopic dermatitis and their family members. Researchers independently coded the transcripts and reached a consensus on the major themes. RESULTS: A total of 33 adult participants, consisting of 21 patients with atopic dermatitis (median age 47 years, range 22-77) and 12 family members (median age 50, range 22-72), attended either focus groups (23/33, 70%) or interviews (10/33, 30%), where we assessed their experiences of pain. Four themes emerged in our study. Itchiness and pain can be intertwined: pain was often caused by or otherwise associated with itchiness and could result from open sores and excoriated skin. Characteristics of pain: pain was most often described as burning. Other descriptors included mild, persistent discomfort; stinging; and stabbing. Effects of pain: pain negatively affected various aspects of daily life, including choice of clothing, sleep, social activities, and relationships. The location of painful areas could also limit physical activity, including sex. Pain management: pain from atopic dermatitis could be managed to varying degrees with different over-the-counter and prescription treatments. Systemic agents that cleared the skin also resolved the pain associated with atopic dermatitis. CONCLUSIONS: Pain can be a significant factor in the HRQL of patients with atopic dermatitis and should be considered by clinicians when caring for patients with atopic dermatitis.

Quality of Life Assessed Using Skindex-16 Scores Among Patients With Acne Receiving Isotretinoin Treatment.

Importance: Acne is a common dermatologic condition and significantly affects psychosocial health and quality of life. An international task force recommended routine use of quality-of-life measures for clinic visits associated with acne management, but this has yet to translate into clinical practice. Objective: To assess mean Skindex-16 scores over time among patients with moderate to severe acne receiving isotretinoin treatment. Design, Setting, and Participants: A longitudinal, retrospective case series study of Skindex-16 data collected at monthly visits from 57 consecutive patients with acne receiving isotretinoin; data were collected and evaluated between November 23, 2016, and January 22, 2019. Continuous variables were compared using quantile regression. Multivariable linear mixed models evaluated mean (95% CI) score trajectory over time. Main Outcomes and Measure: Skindex-16 scores, including normalized scores for the emotional, symptomatic, and functional aspects of having skin disease as well as an overall score. Results: Fifty-seven patients (31 [54.4 %] males, with median [interquartile range] age of 17.2 [15.9-18.1] years) in this case series study completed the Skindex-16 at baseline and at least once during follow-up. Baseline Skindex-16 scores were similar by sex but worse with increasing age. Emotional impact was more bothersome to patients with acne requiring isotretinoin treatment than either symptoms or functioning. Improvements of greater than 50% in overall and Emotional domain scores were seen by month 2 of receiving isotretinoin treatment (eg, overall scores decreased from 39.4 to 17.5 by month 2; a decrease of 22.0; P < .001). Qualitatively, Skindex-16 scores reached their nadir between months 3 and 5; at month 4, overall Skindex-16 scores showed a 4.4-fold improvement (from 39.4 at baseline to 8.9; P < .001) and Emotional domain scores showed a 4.8-fold improvement (from 57.7 at baseline to 11.9; P < .001). Conclusions and Relevance: The findings of this case series suggest that patients receiving isotretinoin treatment achieve greater than a 50% improvement in quality of life by month 2 and can expect approximately 4-fold to 5-fold improvements from baseline with a full course of isotretinoin. This study shows the potential of routine administration of quality of life measures to assess patient care in dermatology.

Information needs of physicians, care coordinators, and families to support care coordination of children and youth with special health care needs (CYSHCN).

OBJECTIVES: Identify and describe information needs and associated goals of physicians, care coordinators, and families related to coordinating care for medically complex children and youth with special health care needs (CYSHCN). MATERIALS AND METHODS: We conducted 19 in-depth interviews with physicians, care coordinators, and parents of CYSHCN following the Critical Decision Method technique. We analyzed the interviews for information needs posed as questions using a systematic content analysis approach and categorized the questions into information need goal types and subtypes. RESULTS: The Critical Decision Method interviews resulted in an average of 80 information needs per interview. We categorized them into 6 information need goal types: (1) situation understanding, (2) care networking, (3) planning, (4) tracking/monitoring, (5) navigating the health care system, and (6) learning, and 32 subtypes. DISCUSSION AND CONCLUSION: Caring for CYSHCN generates a large amount of information needs that require significant effort from physicians, care coordinators, parents, and various other individuals. CYSHCN are often chronically ill and face developmental challenges that translate into intense demands on time, effort, and resources. Care coordination for CYCHSN involves multiple information systems, specialized resources, and complex decision-making. Solutions currently offered by health information technology fall short in providing support to meet the information needs to perform the complex care coordination tasks. Our findings present significant opportunities to improve coordination of care through multifaceted and fully integrated informatics solutions.

Nevus comedonicus: a case series.

Twelve previously unreported cases of nevus comedonicus are presented. Characteristic closely grouped dilated follicular openings with horny plugs that mimic comedones led to the diagnosis. One patient had nevus comedonicus syndrome and there were cases with atypical locations and unusual complications of this condition. We also highlight clinical associations and therapeutic options.

Kaposi's varicelliform eruption: A case series.

Kaposi's varicelliform eruption is a rare and potentially fatal viral infection caused mainly by reactivation of herpes simplex virus. It concomitantly occurs with pre-existing skin conditions, mostly atopic dermatitis, so it is predominately found in children. We present a case series that includes four adults, familial cases, and previously healthy patients. We also highlight clinical features, associations and therapeutic options.

The expectations of nurses about the implementation of a Barcoded Medication Administration System: a qualitative study.

The implementation of a health system like the Barcode Administration System (BCMA) often disrupts the workflow of nursing staff. Taking into account that system acceptance by nurses is one of the most important factors for successful implementation, this paper describes nurses' expectations regarding the introduction of BCMA. For this purpose, interviews were conducted with 18 nurses in different units of an academic medical center in Argentina. Analysis of the interviews produced six main themes. Among these themes two factors were identified as determinants for system acceptance: ease of use perceptions and usefulness.

Integrating personalized health information from MedlinePlus in a patient portal.

The objective of this paper is to describe the implementation and use of context aware information in Spanish from MedlinePlus embedded in a Patient Portal. Personalized information can help patients solve problems, make treatment decisions, gain confidence in their ability to care for themselves and communicate with providers. To integrate MedlinePlus information in our institutional PHR we used the HL7 Context-Aware Knowledge Retrieval Standard, also known as the Infobutton Standard. After analysing one year of use, patients accessed MedlinePlus information in Spanish in a similar rate to other personalized information generated locally. Infobuttons associated to laboratory test results were used in approximately 10% of patients portal sessions when reviewing lab results.

Infantile perianal protrusion.

Infantile perianal protrusion is characterized by a skin fold located in the perianal area. It is a relatively recent reported condition and affects both infants and prepubertal children with a clear female predominance. Three types are recognized: constitutional/congenital, acquired, and associated with lichen sclerosus et atrophicus. We report eleven new cases, three of whom have the defect in locations that have been reported only once before. We would like to increase the awareness of this condition to avoid erroneous diagnostic and therapeutic procedures.

Onicomadesis asociada a la enfermedad de boca, mano y pie / Onychomadesis associated with mouth, hand and foot disease

La onicomadesis es el desprendimiento completo y espontáneo de la uña desde su extremo proximal, sin dolor o inflamación, consecutivo a la detención de la función de la matriz ungular. Es rara en los niños y puede afectar las uñas de las manos o de los pies. Puede ser secundaria a enfermedades sistémicas, enfermedad de Kawasaki, dermatosis ampollares, fármacos, paroniquia, estrés y radioterapia. Desde el año 2000, la enfermedad de boca, mano y pie (EBMP) se ha descrito como causa de onicomadesis y ha ocasionado brotes epidémicos en distintas regiones del mundo. La EBMP es una infección caracterizada por una estomatitis vesicular y erosiva asociada a una erupción vesicular palmoplantar. Afecta a los niños pequeños en verano y otoño, y es causada por el virus coxsackie. Presentamos un estudio que muestra la situación actual de la onicomadesis en niños de la Argentina, con una fuerte asociación entre esta alteración y EBMP previa, siendo, probablemente, una nueva manifestación de una enfermedad ya conocida.(AU)

Onychomadesis is the spontaneous, complete shedding of the nail from its proximal side, without pain or inflammation, following nail matrix arrest. This disorder is uncommon in children and it can occur in fingernails, toenails or both. It may be secondary to systemic disorders, Kawasaki disease, bullous dermatoses, drugs, paronychia, stress and radiotherapy. Since 2000, Hand, Foot, and Mouth Disease (HFMD) has been described as a cause of onychomadesis, and has been associated with outbreaks of this condition in different regions of the world. HFMD is an infection characterized by vesicular and erosive stomatitis in combination with a vesicular eruption in palms and soles. It occurs in small children during summer and autumn months, and it is caused by coxsackie virus. We present a study that reflects the current situation of onychomadesis in Argentinian children and shows a strong association between this disorder and HFMD, suggesting that onychomadesis is a new manifestation of a previously known disease.(AU)

Onicomadesis asociada a la enfermedad de boca, mano y pie / Onychomadesis associated with mouth, hand and foot disease

La onicomadesis es el desprendimiento completo y espontáneo de la uña desde su extremo proximal, sin dolor o inflamación, consecutivo a la detención de la función de la matriz ungular. Es rara en los niños y puede afectar las uñas de las manos o de los pies. Puede ser secundaria a enfermedades sistémicas, enfermedad de Kawasaki, dermatosis ampollares, fármacos, paroniquia, estrés y radioterapia. Desde el año 2000, la enfermedad de boca, mano y pie (EBMP) se ha descrito como causa de onicomadesis y ha ocasionado brotes epidémicos en distintas regiones del mundo. La EBMP es una infección caracterizada por una estomatitis vesicular y erosiva asociada a una erupción vesicular palmoplantar. Afecta a los niños pequeños en verano y otoño, y es causada por el virus coxsackie. Presentamos un estudio que muestra la situación actual de la onicomadesis en niños de la Argentina, con una fuerte asociación entre esta alteración y EBMP previa, siendo, probablemente, una nueva manifestación de una enfermedad ya conocida.

Onychomadesis is the spontaneous, complete shedding of the nail from its proximal side, without pain or inflammation, following nail matrix arrest. This disorder is uncommon in children and it can occur in fingernails, toenails or both. It may be secondary to systemic disorders, Kawasaki disease, bullous dermatoses, drugs, paronychia, stress and radiotherapy. Since 2000, Hand, Foot, and Mouth Disease (HFMD) has been described as a cause of onychomadesis, and has been associated with outbreaks of this condition in different regions of the world. HFMD is an infection characterized by vesicular and erosive stomatitis in combination with a vesicular eruption in palms and soles. It occurs in small children during summer and autumn months, and it is caused by coxsackie virus. We present a study that reflects the current situation of onychomadesis in Argentinian children and shows a strong association between this disorder and HFMD, suggesting that onychomadesis is a new manifestation of a previously known disease.

Fascitis necrotizante como complicación de la varicela en niños / Necrotizing fasciitis as a complication of chickenpox in children

La fascitis necrotizantees una enfermedad grave y poco frecuente. Se la ha descripto como una complicación rara dela varicela, en niños previamente sanos. Se caracteriza por una extensa necrosis de la piel y el tejido celular subcutáneo, que se extiendea las fascias y músculos subyacentes. Está vinculada a alta toxicidad sistémica y es rápidamente fatal, sino se diagnostica con celeridad. La presente revisión tiene como objetivo repasar los elementos que permiten reconocer y diagnosticar esta patología para llevar a cabo un tratamiento adecuado lo antes posible...

Necrotizing fasciitis is an uncommon but serious disease. It has been described as a rare complication of chickenpox in healthy children.It is characterized by extensive necrosis of skin and subcutaneous tissue with subsequent compromise of the fascial planes and muscles. It presents with severe systemic toxicity and is usually rapidly fatal unless diagnosed promptly and treated aggressively. The present review emphasizes the elements that guide the recognition and diagnose of the disease, in order to carry out immediate and proper treatment...