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A letter to the editor is presented with regards to the occurrence of segmental capillary haemangioma in a 2-week-old baby girl.

The management of alopecia areata (AA) in pediatric patients poses unique challenges, particularly regarding treatment discussions and decision making involving both patients and their families. This commentary presents findings from unpublished research on treatment-discontinuation discussions between AA patients and their treating providers, shedding light on the hopes, expectations, and disappointments of individuals with severe AA. The study explored patient and guardian satisfaction with these discussions, emphasizing the importance of addressing psychosocial concerns, facilitating contact with support groups, and demonstrating empathy. The role of dermatologists in conversations about treatment, prognosis, and quality of life is examined, emphasizing the need for honesty, empathy, and realistic expectations. The authors propose a patient-centered approach to initiating and guiding discussions, focusing on understanding the impact of AA on patients and their families and collaboratively deciding on treatment options. The mantra: 'I need to understand how this is affecting all of you, so we can decide together what to do next' is central to this proposed approach. Special considerations for different scenarios are discussed, highlighting the importance of individualized care and effective communication. Overall, the commentary emphasizes the significance of actively listening, acknowledging emotions, and prioritizing patient and family goals to optimize care for pediatric AA patients., (© 2024 Wiley Periodicals LLC.)

Aim: To investigate the feasibility and acceptability of the training process, procedures, measures and recruitment strategies necessary for a future investigation to test the reliability and validity of using positivity resonance measures in health care encounters., Background: Although the measurement of positivity resonance is promising, and non-participant observation is considered effective, their approaches to studying nurse-patient relationships have not been fully explored., Design: A mixed-methods observational study., Methods: Video recordings of 30 nurse-patient dyads completing telehealth video visit encounters were edited and coded using behavioural indicators of positivity resonance. A post-visit survey gathered data on the participants' perceptions of positivity resonance and the study procedures. The research team completed memos and procedural logs to provide narrative data on the study's training, coding, recruitment and operational procedures. The study included 33 persons with cancer and 13 oncology nurses engaging in telehealth video visit encounters at an academic oncology ambulatory care center located in the southeastern United States., Results: Study procedures were found to be feasible and acceptable to participants. An adequate sample of participants (N = 46) were enrolled and retained in the study. Interrater reliability, as evidenced by Cohen's weighted kappa, ranged from .575 to .752 and interclass correlation coefficients >.8 were attainable within a reasonable amount of time and with adequate training. Behavioural indicators of positivity resonance were observed in all telehealth visits and reported by the participants in the perceived positivity resonance survey. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist guided reporting., Conclusions: Designing research around the concept of positivity resonance is an innovative and feasible approach to exploring how rapport is cultivated within nurse-patient relationships., Relevance to Professional Practice: Measuring positivity resonance may hold promise for exploring patient and nurse outcomes including trust, responsiveness, health-related behaviours, well-being, resilience and satisfaction., Reporting Method: The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist guided the reporting of results to ensure that adequate details of the study were provided to ensure an accurate and complete report., Patient or Public Contribution: Planning of the research design and study procedures was done in consultation with nurse clinicians with experience with telehealth and managers responsible within the practice setting where the study was conducted. This ensured the study procedures were ethical, safe, secure and did not create unnecessary burden to the study participants. The study included collecting data from nurse and patient participants about the acceptability of the study procedures., (© 2024 John Wiley & Sons Ltd.)

Objectives: Clinician burnout poses risks not just to clinicians but also to patients and the health system. Cardiologists might be especially prone to burnout due to performing high-risk procedures, having to discuss serious news, and treating diseases that incur significant morbidity and mortality. Few have attempted to examine which cardiologists might be at higher risk of burnout. Knowing at-risk cardiologists can help frame resilience interventions., Methods: We enrolled 41 cardiologists across five ambulatory cardiology clinics into a randomized controlled trial where we assessed the Maslach Burnout Inventory at baseline. We used bivariate analyses to assess associations between cardiologist demographics and burnout., Results: Cardiologists reported low burnout for depersonalization and personal accomplishment and moderate levels for emotional exhaustion. Female cardiologists reported emotional exhaustion scores in the "low" range (M = 12.3; SD = 10.06), compared to male cardiologists whose score was in the "moderate" range 19.6 (SD = 9.59; p = 0.113). Cardiologists who had greater than 15 years in practice reported higher mean scores of emotional exhaustion, indicating moderate burnout (M = 20.0, SD = 10.63), compared to those with less than 15 years in practice (M = 16.6, SD = 9.10; p = 0.271)., Conclusions: In this sample, unlike prior studies, male cardiologists reported more burnout. Consistent with prior work, mid-level cardiologists might be at highest risk of emotional exhaustion.

Background: Although videoconferencing between oncology patients and nurses became routine during the pandemic, little is known about the development of clinician-patient rapport in this care environment. Evidence that virtual visits may challenge nurses' ability to form connections with patients, demonstrate empathy, and provide support suggests that videoconferencing may not ensure optimal care for persons with cancer. Establishing rapport during videoconferencing visits (VCVs) is important in oncology nursing, as rapport enables the nurse to provide emotional support and assistance to patients as they navigate their cancer journey., Objective: This study investigated the nature of nurse-patient rapport in ambulatory cancer care videoconferencing telehealth visits. Objectives included exploring (1) how patients with cancer and nurses describe experiences of and strategies for cultivating rapport and (2) similarities and differences between rapport in videoconferencing and in-person visits (IPVs)., Methods: In this qualitative descriptive study, interviews were conducted from October 2021 to March 2022 with 22 participants, including patients with cancer (n=10, 45%) and oncology nurses (n=12, 55%), about their experiences of rapport building during VCVs. All interviews were analyzed using conventional content analysis. Data from nurses and patients were analyzed separately using identical procedures, with a comparative analysis of patient and nurse results performed in the final analysis., Results: Most patients in the study had experienced 3-5 video visits within the past 12 months (n=7, 70%). Half of the nurse participants (n=6, 50%) reported having participated in over 100 VCVs, and all had experiences with videoconferencing (ranging from 3 to 960 visits) over the past 12 months. In total, 3 themes and 6 categories were derived from the patient data, and 4 themes and 13 categories were derived from the nurse data. Comparisons of themes derived from participant interviews identified similarities in how nurses and patients described experiences of rapport during VCVs. Three themes fit the collective data: (1) person-centered and relationship-based care is valued and foundational to nurse-patient rapport in oncology ambulatory care regardless of how care is delivered, (2) adapting a bedside manner to facilitate rapport during VCVs is feasible, and (3) nurses and patients can work together to create person-centered options across the care trajectory to ensure quality care outcomes. Barriers to relationship building in VCVs included unexpected interruptions from others, breaks in the internet connection, concerns about privacy, and limitations associated with not being physically present., Conclusions: Person-centered and relationship-based approaches can be adapted to support nurse-patient rapport in VCVs, including forming a personal connection with the patient and using active listening techniques. Balancing the challenges and limitations with the benefits of videoconferencing is an essential competency requiring additional research and guidelines., International Registered Report Identifier (irrid): RR2-10.2196/27940., (©Paula D Koppel, Jennie C De Gagne, Sharron Docherty, Sophia Smith, Neil S Prose, Terri Jabaley. Originally published in the Journal of Medical Internet Research (https://www.jmir.org), 08.09.2022.)

Background: Our documentary, Keepers of the House, highlights ways that hospital housekeepers, typically unnoticed care team members, provide emotional support for patients and their families. This film addresses a gap in education by emphasizing the importance of valuing and reflecting on the unique lived experiences of others., Approach: We created this documentary to expose students to the experiences and perceptions of hospital housekeepers. A focus group with six hospital housekeepers informed an interview script for the film's creation. Nine additional housekeepers were then interviewed, which developed into a 15-min documentary. Healthcare students and educators from five disciplines viewed the documentary during their institution's Medical Education Day., Evaluation: To expose students and educators to housekeepers' experiences, we designed our post-viewing survey to address whether the housekeepers' stories impacted their understanding of the role and value of these workers. Viewers were surprised by the depth and breadth of patient-housekeeper interactions, the trauma housekeepers experienced from patient loss and the pride housekeepers take in their work. The stories that touched the viewers varied but centred on connections between housekeepers and patients. Lessons learned focused on recognizing the contributions of unseen team members., Implications: This innovative documentary amplifies the perspectives of voices rarely heard in healthcare. We aim to use this film, alongside its associated learning session, in education and grand round settings to foster discussion around empathy, valuing underrecognised team members and applying these insights in practice. This work can be disseminated to other institutions, further amplifying underrepresented narratives in healthcare., (© 2021 The Authors. The Clinical Teacher published by Association for the Study of Medical Education and John Wiley & Sons Ltd.)

Background: Mistreatment of women during facility-based childbirth is a major violation of human rights and often deters women from attending skilled birth. In Ethiopia, mistreatment occurs in up to 49.4% of mothers giving birth in health facilities. This study describes the development, implementation and results of interventions to improve respectful maternity care. As part of a national initiative to reduce maternal and perinatal mortality in Ethiopia, we developed respectful maternity care training module with three core components: testimonial videos developed from key themes identified by staff as experiences of mothers, skills-building sessions on communication and onsite coaching. Respectful maternity care training was conducted in February 2017 in three districts within three regions., Methods: Facility level solutions applied to enhance the experience of care were documented. Safe Childbirth Checklist data measuring privacy and birth companion offered during labor and childbirth were collected over 27 months from 17 health centers and three hospitals. Interrupted time series and regression analysis were conducted to assess significance of improvement using secondary routinely collected programmatic data., Results: Significant improvement in the percentage of births with two elements of respectful maternal care-privacy and birth companionship offered- was noted in one district (with short and long-term regression coefficient of 18 and 27% respectively), while in the other two districts, results were mixed. The short-term regression coefficient in one of the districts was 26% which was not sustained in the long-term while in the other district the long-term coefficient was 77%. Testimonial videos helped providers to see their care from their clients' perspectives, while quality improvement training and coaching helped them reflect on potential root causes for this type of treatment and develop effective solutions. This includes organizing tour to the birthing ward and allowing cultural celebrations., Conclusion: This study demonstrated effective way of improving respectful maternity care. Use of a multipronged approach, where the respectful maternity care intervention was embedded in quality improvement approach helped in enhancing respectful maternity care in a comprehensive manner., (© 2021. The Author(s).)

Given the role of effective communication in improving patient adherence and satisfaction, high quality patient-clinician communication is critical. Building on previous communication interventions in oncology and pediatrics, we developed a tailored communication coaching intervention to improve empathic communication quality and patient-centered care. In this randomized controlled trial, cardiologists record their patient encounters for review by a communication coach who provides tailored feedback. We are recruiting 40 cardiologists and 400 patients, or 4 patients per cardiologist in the Pre-intervention phase and 6 patients per cardiologists in the Post-intervention phase, from outpatient cardiology clinics within the Duke Health System. The primary goal of the trial is to determine the efficacy of the clinician communication coaching versus usual care in the post-intervention phase (240 patient encounters). In this paper, we describe the development of the communication coaching intervention. We also describe the details of the methods and outcomes of the ongoing trial. Finally, we discuss the challenges, solutions, and lessons learned during the start-up phase of the study., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Inflammatory linear verrucous epidermal nevus (ILVEN) is a chronic, a linear, or whorled array of inflammatory, following the lines of Blaschko. Treatment of ILVEN is challenging with numerous therapies of varying degrees of success reported. We present a case of ILVEN in a 5-year-old-boy, treated successfully with crisaborole 2% ointment. This brief report suggests that there may be additional cellular immunologic pathways responsible for the presentation of ILVEN that may be explained by management with crisaborole use., (© 2019 Wiley Periodicals, Inc.)

There are few reports of chemotherapy-induced eccrine squamous syringometaplasia in children. We report the first case of an infant developing this condition after treatment with busulfan, fludarabine, and antithymocyte globulin in preparation for bone marrow transplantation. Twenty-eight days after transplantation, the infant developed faintly erythematous papules and plaques on the bilateral axillae, inguinal folds, and sites of adhesives. Punch biopsy revealed eccrine glands with dyskeratotic cells and focal squamous metaplasia consistent with chemotherapy-induced eccrine squamous syringometaplasia., (© 2017 Wiley Periodicals, Inc.)

Eccrine chromhidrosis can occur secondary to hyperbilirubinemia. We report an adolescent with sickle cell disease who presented with eccrine chromhidrosis on his palmar and plantar surfaces. He had extremely high levels of conjugated bilirubin but no fever. This is the youngest known reported patient with eccrine chromhidrosis and the first in a patient with sickle cell disease., (© 2017 Wiley Periodicals, Inc.)

The needs of South African men with HIV may often be overlooked in the provision of HIV services, leading to care programs that do not adequately serve the unique needs of male patients. Additionally, norms of masculinity guide men's behaviors as they navigate health decision-making and the healthcare systems. The aim of this study is to examine how masculinity influences healthcare access and utilization in South Africa, and to identify opportunities for interventions. The qualitative study took place at one primary health care clinic in a peri-urban township in KwaZulu-Natal, South Africa. In-depth individual interviews were conducted with 21 HIV-infected men recruited from the study clinic. Direct observations of the clinic waiting area were conducted to provide context. Data were analyzed using a grounded theory-informed approach involving memo writing and thematic exploration with data coding. On average, participants were 42 years old and had been on ART for 3.6 years. Participants expressed a range of ways in which masculine ideals and identity both promoted and inhibited their willingness and ability to engage in HIV care. Notions of masculinity and social identity were often directly tied to behaviors influencing care engagement. Such engagement fostered the reshaping of identity around a novel sense of clinic advocacy in the face of HIV. Our findings suggested that masculinities are complex, and are subject to changes and reprioritization in the context of HIV. Interventions focusing on reframing hegemonic masculinities and initiating treatment early may have success in bringing more men to the clinic.

Objective: To describe the design, content, implementation, and evaluation of a national curriculum for teaching practical skills in empathic communication to residents in neurosurgery., Design: Based on needs assessed through a national survey of neurosurgery program directors, videotaped scenarios using standardized patients illustrating good and bad communication skills were developed. Presurveys and postsurveys were conducted querying participants on their level of competence and the specific behaviors they would attempt to change following participation. A subgroup of residents was evaluated before and after the training based on videotaped role-play exercises., Setting: A pilot study was conducted at the authors' institution and later implemented at National Neurosurgery Boot Camps., Participants: A total of 14 Duke graduate medical education neurosurgery residents agreed to participate in the pilot study. From across the country, 93 residents (representing 59 institutions) participated in the communication training as part of the Neurosurgery Boot camps, 11 of whom volunteered to participate in a role-playing session before and after the formal teaching session., Results: Most of the neurosurgery program directors responding to the survey indicated that an interactive online communication-training module would be of value (77%). A total of 93 residents participated in communication training as part of the Neurosurgery Boot Camps. Approximately half of the residents reported having no formal physician-patient communication training. Presurvey and postsurvey results showed significant improvement in several of the communication scenarios. Those who participated in role-play showed significant improvement in "asking open-ended questions," "listening," "fire warning shot," "allowing patient to absorb," and "explaining in clear language.", Conclusions: Neurosurgeons frequently participate in difficult conversations. Both residents and faculty note that exposure to this content is suboptimal. A hybrid approach to teaching communication skills is well received and enhances graduate medical education training of surgical subspecialists., (Copyright © 2015 Association of Program Directors in Surgery. Published by Elsevier Inc. All rights reserved.)

Cutaneous toxoplasmosis is a rare and diagnostically challenging entity. Today, the acquired form occurs predominantly in immunocompromised patients with human immunodeficiency virus or after hematopoietic stem cell transplantation. We report a case of cutaneous toxoplasmosis in a 6-year-old girl after allogeneic stem cell transplantation for immune-mediated encephalopathy, first manifesting at 16 months of age. In the post-transplant setting, she developed a rash consisting of approximately 8 scattered 3–4-mm round, erythematous macules and papules on her back, abdomen, and right shoulder. Sections from a biopsy of a lesion on the back revealed numerous spherules tightly packed within small cystic structures in the epidermis. The diagnosis of cutaneous toxoplasmosis was confirmed by an immunohistochemical stain for Toxoplasma gondii and polymerase chain reaction on the peripheral blood for the T. gondii genome. This case should raise awareness that acquired toxoplasmosis with cutaneous involvement can occur in the pediatric population, particularly in immunocompromised patients after stem cell transplantation. Early diagnosis and treatment of this life-threatening opportunistic infection may improve patient outcomes.

The motivation for developing patient-centered communication stems from a desire to enhance the quality of patient care, fulfill professional competency requirements, reduce medical errors, and improve health outcomes and patient satisfaction. Patient-centered communication skills can optimize the physician-patient relationship without significantly prolonging office visits. We propose a series of practical and generally effective techniques for verbal and nonverbal communication. We also suggest a targeted approach for specific difficult conversations that may occur frequently in the practice of dermatology., (Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Patient education is a fundamental part of caring for patients. A practice gap exists, where patients want more information, while health care providers are limited by time constraints or difficulty helping patients understand or remember. To provide patient-centered care, it is important to assess the needs and goals, health beliefs, and health literacy of each patient. This allows health care providers to individualize education for patients. The use of techniques, such as gaining attention, providing clear and memorable explanations, and assessing understanding through "teach-back," can improve patient education. Verbal education during the office visit is considered the criterion standard. However, handouts, visual aids, audiovisual media, and Internet websites are examples of teaching aids that can be used as an adjunct to verbal instruction. Part II of this 2-part series on patient-physician interaction reviews the importance and need for patient education along with specific guidelines and techniques that can be used., (Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Paediatric scalp naevi may represent a source of anxiety for practitioners and parents, as the clinical and dermoscopic features of typical naevi have yet to be defined. Prompted by concern about the large size, irregular borders and colour variation of scalp naevi, clinicians and parents may request unnecessary excision of these naevi., Objectives: To establish the typical clinical and dermoscopic patterns of scalp naevi in children younger than 18 years old to help optimize clinical care and management., Methods: Scalp naevi were imaged with a camera (Canon Rebel, XSi; Canon, Tokyo, Japan) and dermoscopic attachment (Dermlite Foto, 30 mm lens; 3Gen, San Juan Capistrano, CA, U.S.A.) to the camera. The clinical and dermoscopic images were reviewed and analysed. Both acquired and congenital scalp naevi were included but were not further differentiated from each other., Results: We obtained clinical and dermoscopic images of 88 scalp naevi in 39 white children. Two subjects had received chronic immunosuppressive medication. Nineteen children had a family history of melanoma. Boys (18/39 subjects, 46%) possessed 68% (60 naevi) of scalp naevi imaged. Younger (< 10 years old) subjects (24/39 subjects, 62%) possessed 42% (37 naevi) of scalp naevi. The main clinical patterns included eclipse (n=18), cockade (n = 3), solid brown (n=42) and solid pink (n=25) naevi. Solid-coloured naevi showed the following dermoscopic patterns: globular (57%), complex (reticular-globular) (27%), reticular (9%), homogeneous (6%) and fibrillar (1%). The majority of naevi had a unifying feature - perifollicular hypopigmentation, which caused the appearance of scalloped, irregular borders if occurring on the periphery, or variegation in pigmentation, if occurring within the naevi., Conclusions: Older subjects and boys tend to harbour a larger proportion of scalp naevi. The main clinical patterns include solid-coloured and eclipse naevi. The most common dermoscopic pattern of scalp naevi is the globular pattern. Perifollicular hypopigmentation is a hallmark feature of signature scalp naevi. Dermoscopy is a noninvasive tool in the evaluation of cutaneous melanocytic lesions in children and may decrease the number of unnecessary excisions., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists 2011.)

Background: The morbidity and mortality conference is an educational tradition in American medicine that dates to the early 20th century. Traditionally, this conference has focused entirely on issues of diagnosis and treatment, in the context of a disappointing clinical outcome., Intervention: We report on a new method for teaching empathic doctor-patient communication skills at an obstetrics and gynecology morbidity and mortality conference. For each case presented, we identified the communications challenges and allowed faculty and residents to "practice" the discussion they would have with the patient and the patient's family in that situation. In some sessions, actors assumed the role of the patient. Following the discussion of the case, we offered didactic presentations on how we communicate with patients and their families. These focused on techniques for being patient centered and included the use of body language, open-ended questioning, reflective listening before offering to explain, and the importance of naming and validating emotions., Results: The majority of participants felt the sessions to be helpful, and after one month many were able to identify a positive change in their interactions with patients., Conclusions: We believe that this unique teaching format allows learners to refine their communication skills in the context of situations that they know to be both realistic and important.

Background: Voriconazole is a broad-spectrum antifungal agent associated with photosensitivity and accelerated photoaging. A possible link with aggressive squamous cell carcinoma (SCC) has also been reported., Objective: We sought to determine the incidence and frequency of cutaneous SCC among patients undergoing long-term treatment with voriconazole who also manifest features of chronic phototoxicity., Methods: We conducted a retrospective review of patients who developed one or more squamous cell neoplasms during long-term treatment with voriconazole at 3 academic dermatology centers., Results: A total of 51 cutaneous SCC were identified in 8 patients (median age 34.5 years, range 9-54) treated with chronic voriconazole (median duration 46.5 months, range 13-60). Underlying diagnoses included graft-versus-host disease, HIV, and Wegener granulomatosis. Signs of chronic phototoxicity and accelerated photoaging included erythema, actinic keratoses, and lentigo formation., Limitations: The retrospective nature of the study cannot determine the true population risk of SCC associated with voriconazole therapy. A prospective cohort study is needed., Conclusion: A high index of suspicion for photosensitivity and SCC may be warranted with chronic voriconazole use when used in the setting of concurrent immunosuppression.

Trichothiodystrophy (TTD) is a rare group of autosomal recessive disorders of DNA repair unified by the presence of sulfur-deficient brittle hair. We report a 3-year-old boy with classic clinical features of TTD, including ichthyosis, alopecia, developmental delay, and tiger-tail banding of the hair shaft on polarizing microscopy. Brain MR imaging showed both diffuse dysmyelination and osteosclerosis, findings that, in combination, may be specific for TTD.

Acanthosis nigricans is a dermatosis characterized by thickened, hyperpigmented plaques, typically of the intertriginous surfaces and neck. Common in some populations, its prevalence depends on race. Clinicians should recognize acanthosis nigricans; it heralds disorders ranging from endocrinologic disturbances to malignancy. In this review, we discuss the pathogenesis of acanthosis nigricans and its clinical implications and management.

We report the case of a male infant with incontinentia pigmenti (MIM 308310) and low-grade XXY mosaicism. Fluorescence in situ hybridization may reveal the underlying genetic alteration in male patients with incontinentia pigmenti and a normal karyotype.

Pemphigus vulgaris is an uncommon autoimmune blistering skin disorder that is particularly rare in children. Immunosuppressive treatment can be challenging. Rituximab (anti-CD20 monoclonal antibody) has been used to treat autoimmune disorders by depletion of CD20 B cells. Successful rituximab therapy has been reported in adults with refractory pemphigus vulgaris. We present a girl with childhood pemphigus vulgaris unresponsive to treatment with azathioprine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin with systemic prednisone who responded to treatment with rituximab. She had a corresponding decline in circulating antibodies against desmoglein 1 and 3 and a decline in diphtheria and tetanus-specific antibody titers.

The incidence of skin disease secondary to infestation with the human bedbug, Cimex lectularius, has increased dramatically in the United States and in the United Kingdom. We describe a child with a recurrent pruritic eruption of urticarial, erythematous papules on the face, neck, and extremities. The etiology of her cutaneous lesions was discovered to be a bedbug infestation in the home. The epidemiology, entomology, presentation, and treatment of bedbugs and their bites are discussed.

Precalcaneal congenital fibrolipomatous hamartomas are uncommon, congenital, nontender papules located on the medial plantar aspects of the heel. We report the occurrence of this rare disorder in two half brothers, suggesting that it may occur in a familial pattern.

Congenital cutaneous candidiasis (CCC) is usually a benign condition characterized by various skin manifestations and is rarely associated with nail changes. We report a premature infant with CCC who developed dystrophy of all 20 nails at about 1 month of age. Nail dystrophy due to Candida albicans in the young infant may be differentiated from other congenital or hereditary nail malformations by appearance, recovery of the organism in nail culture, and complete resolution over a period of several months.

Purpose: Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP., Methods: We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe., Results: All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability., Conclusion: Vincristine presents a safe and sometimes effective treatment option in the management of KMP.

Hereditary benign intraepithelial dyskeratosis is a rare autosomal dominant disorder of the oral and ocular mucosa initially described in the Haliwa-Saponi Native American tribe of North Carolina. We describe 2 sisters with the characteristic oral and ocular findings. This entity should be distinguished from several other diseases that cause white lesions in the mouth including white sponge nevus.

Waxy keratoses of childhood is a rare genodermatosis previously noted in both familial and isolated presentations. Three previous cases have been described in which the lesions appeared over the trunk and extremities. We report a case in which the waxy keratoses of childhood lesions appeared in a segmental distribution along a single lower extremity, differing from the more extensive patterns reported previously. We suggest a possible postzygotic mutation hypothesis for this unique segmental distribution, addressing both the possibility of a genomic mosaicism and loss of heterozygosity.

Background: Epidermal necrosis in a neonate is an uncommon event with a variety of potential cases., Result: We report a case of intrauterine epidermal necrosis in a preterm infant, with death occurring soon after birth. The histopathology of the denuded skin revealed full-thickness epidermal necrosis and calcification within both the epidermis and follicular structures., Conclusion: We believe this represents the fourth reported case of lethal intrauterine epidermal necrosis and follicular calcification.

Two infants, one with a T-cell-signaling defect resulting in a primary immunodeficiency syndrome and the other with severe combined immunodeficiency (SCID), are described. Both infants developed cutaneous infections secondary to their bacillus Calmette-Guérin (BCG) vaccinations. Both patients were from countries where BCG is routinely administered in infancy. The infant with the T-cell-signaling defect developed a disseminated infection involving the skin, while the infant with SCID developed a localized cutaneous infection at the site of his BCG immunization. These two cases resemble other reported cases of cutaneous BCG infection following routine vaccination in immunocompromised patients. Mycobacterium bovis infection should be considered in patients with cutaneous eruptions who have received BCG vaccination, especially those who are immunocompromised.

Skin and nail changes from long-term hydroxyurea therapy are reported in adults. Skin and nail changes, including nail hyperpigmentation, longitudinal bands, and hyperpigmentation of the palms and other skin surfaces, developed in 7 children with sickle cell anemia after 6 to 16 weeks of hydroxyurea therapy. Cutaneous and nail changes may occur in children receiving hydroxyurea.

Background: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations., Objective: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies., Methods: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas., Results: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis., Conclusion: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.

Purpose: To describe the magnetic resonance (MR) imaging findings of subcutaneous granuloma annulare (SGA), a rare mass of early childhood., Materials and Methods: MR imaging studies and clinical records in six children aged 2 1/2-4 years in whom SGA was diagnosed between 1993 and 1997 were retrospectively reviewed. All MR imaging examinations included T1-weighted and fast spin-echo T2-weighted sequences. Three children received intravenous contrast material. The diagnosis was established by using excisional biopsy results, with confirmation by means of characteristic features in mucin-stained specimens., Results: All children presented with a nontender, nonmobile mass. The lesion in all six children was confined to the pretibial (n = 5) or prepatellar (n = 1) soft tissues. MR imaging characteristics were uniform. Masses were subcutaneous in location and had ill-defined margins. On T1-weighted images, the lesion was isointense or slightly hyperintense to muscle. On T2-weighted images, the signal intensity was more heterogeneous, but the lesion was predominantly hyperintense. In three children who received contrast material, the lesion enhanced nearly homogeneously., Conclusion: Although SGA can often be diagnosed on the basis of clinical characteristics, MR imaging may be requested by practitioners unfamiliar with the lesion. In these cases, with recognition of typical MR imaging features, a limited biopsy and specific histologic preparation that includes mucin staining can be recommended.