Malassezia-associated skin diseases in the pediatric population.

Malassezia are yeast species that commonly colonize healthy skin. However, they have been associated with or implicated in the pathogenesis of numerous skin disorders, particularly in the setting of pediatric populations. In this review, we will focus on several Malassezia-associated skin conditions manifesting in infants, children, and adolescents: pityriasis versicolor, Malassezia folliculitis, infantile and adolescent seborrheic dermatitis, head and neck dermatitis, and neonatal cephalic pustulosis. We examine the literature and provide an overview of these conditions, including clinical presentation in diverse skin colors, diagnosis, risk factors, and treatment and management. Additionally, we summarize and highlight some of the proposed theories on the role of Malassezia spp. in the pathogenesis of these skin conditions.

A case of reactive granulomatous dermatitis associated with neonatal lupus erythematosus.

Neonatal lupus erythematosus (NLE) is an uncommon disorder affecting approximately one out of 20 000 live births in the United States. Common manifestations of NLE include cutaneous eruptions and cardiac involvement. The typical rash of NLE most closely resembles the rash of subacute cutaneous lupus erythematosus both clinically and histopathologically. We present a case of reactive granulomatous dermatitis (RGD) associated with NLE in a 3-month-old male in whom the initial histopathology and immunohistochemistry were concerning for hematologic malignancy. RGD is a unifying term used to describe cutaneous granulomatous eruptions that occur in response to a variety of stimuli, including autoimmune connective tissue diseases. Our case demonstrates the range of histopathological findings that may be present in the setting of NLE.

Pediatric hidradenitis suppurativa: Describing care patterns in the emergency department.

BACKGROUND/OBJECTIVES: The emergency department (ED) is a common point of care for many pediatric hidradenitis suppurativa (HS) patients; however, ED care may not align with recommended treatment standards. The objective of this study is to describe the care pediatric HS patients receive in the ED compared to both HS management guidelines and the management of pediatric skin and soft tissue abscess (SSTA) patients. METHODS: We performed a retrospective chart review of pediatric HS and SSTA patients who presented to a single pediatric ED. Demographic and ED encounter data including medications, procedures, recommendations, consults, and referrals were collected. RESULTS: 58 HS and 175 SSTA charts were reviewed. 69.5% of patients were female and 93.6% were African American/Black. 29.3% of HS and 61.7% of SSTA encounters involved incision and drainage (I&D); 62.1% of HS and 70.1% of SSTA encounters involved the prescription or administration of oral clindamycin; 3.4% of HS and 0.6% of SSTA encounters involved dermatology consultation in the ED; and 22.4% of HS and 5.1% of SSTA encounters involved outpatient referrals to dermatology. CONCLUSIONS: Our data reveal a pediatric HS and SSTA population that is predominantly female and African American/Black. Significant differences were found in the average ages and number of lesions between HS and SSTA patients. I&D and oral clindamycin were the most common treatments across groups; however, I&D was performed significantly more frequently among SSTA encounters than HS encounters. Our results suggest that subtle knowledge gaps may exist in ED provider management of pediatric HS.

Vitiligo-like manifestations of graft-versus-host disease in a pediatric population.

Vitiligo-like changes are an uncommon cutaneous manifestation of graft-versus-host disease (GVHD). We report three cases and review the literature of pediatric patients with vitiligo-like changes associated with GVHD. Improved characterization of this phenomenon may lend insight into the biologic pathways that underlie both vitiligo and GVHD.

A case of non-disseminated herpes zoster as the presenting sign of HIV in a 3-year-old.

Herpes zoster is rare in healthy and immunocompetent children. While disseminated presentations of zoster are often concerning for underlying immunodeficiency, non-disseminated zoster can also be a presenting illness in such patients. Here, we report a case of non-disseminated herpes zoster in a presumably healthy immunized child that led to a diagnosis of human immunodeficiency virus infection.

Follicular keratosis of the face in pediatric patients of color.

BACKGROUND/OBJECTIVES: Follicular keratosis (FK) is a poorly understood disorder presenting with multiple, grouped hyperkeratotic follicular papules typically affecting the chin or jawline. This study describes the clinical presentation, histopathology, management, and outcomes of a series of pediatric patients of color with FK of the face, thought to be related to rubbing or friction on the skin. METHODS: Retrospective review of 20 pediatric patients with FK of the face who presented to our pediatric dermatology practice between April 2019 and October 2021. RESULTS: Twenty patients (mean age 12.1 years, 13 females), all self-identified as Black/African American, were included. All presented with an initially asymptomatic, hyperpigmented patch containing multiple hyperkeratotic follicular papules, located on the cheek, chin, upper lip, and/or jawline. Five patients endorsed a history of rubbing the site. Nine patients had onset of the lesions during the COVID-19 pandemic. Treatments included topical vitamin D analogs, corticosteroids, and/or retinoids. Topical vitamin D analogs and retinoids improved the texture and hyperpigmentation of the follicular lesions in only four patients, while topical corticosteroids had no effect. Histopathological examination of two patients revealed multiple dilated follicles containing keratinized material and associated with a sparse dermal inflammatory infiltrate in one patient and granulomatous inflammation within the dermis in the other. CONCLUSIONS: In our cohort of pediatric patients with FK, patients of color were preferentially affected, and all cases were associated with hyperpigmentation. Some patients presented during the COVID-19 pandemic suggesting that friction from facial mask wearing may have induced or exacerbated this uncommon condition.

Quantitative analysis of the natural history of prolidase deficiency: description of 17 families and systematic review of published cases.

PURPOSE: Prolidase deficiency is a rare inborn error of metabolism causing ulcers and other skin disorders, splenomegaly, developmental delay, and recurrent infections. Most of the literature is constituted of isolated case reports. We aim to provide a quantitative description of the natural history of the condition by describing 19 affected individuals and reviewing the literature. METHODS: Nineteen patients were phenotyped per local institutional procedures. A systematic review following PRISMA criteria identified 132 articles describing 161 patients. Main outcome analyses were performed for manifestation frequency, diagnostic delay, overall survival, symptom-free survival, and ulcer-free survival. RESULTS: Our cohort presented a wide variability of severity. Autoimmune disorders were found in 6/19, including Crohn disease, systemic lupus erythematosus, and arthritis. Another immune finding was hemophagocytic lymphohistiocytosis (HLH). Half of published patients were symptomatic by age 4 and had a delayed diagnosis (mean delay 11.6 years). Ulcers were present initially in only 30% of cases, with a median age of onset at 12 years old. CONCLUSION: Prolidase deficiency has a broad range of manifestations. Symptoms at onset may be nonspecific, likely contributing to the diagnostic delay. Testing for this disorder should be considered in any child with unexplained autoimmunity, lower extremity ulcers, splenomegaly, or HLH.

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

BACKGROUND: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized. OBJECTIVE: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations. METHODS: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software. RESULTS: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4). LIMITATIONS: Retrospective nature and the inclusion of only patients with clinical photographs. CONCLUSION: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.

Erythema ab igne in pediatric patients remote schooling during the COVID-19 pandemic: A case series.

Erythema ab igne is an uncommon dermatosis characterized by erythematous or hyperpigmented reticular patches that appear after prolonged localized heat exposure. We present three cases of erythema ab igne in pediatric patients who presented in March and April of 2021 and share a history of space heater usage while engaging in remote schooling during the COVID-19 pandemic.

Facial hypopigmentation in skin of color: An atypical presentation of lichen nitidus.

Lichen nitidus is a benign skin condition of unknown etiology that is classically described on the trunk, extremities, and genitalia as pinpoint flat-topped papules. In dark-skinned persons, the lesions may appear shiny or even hypopigmented. Lichen nitidus is less often described on the face. We describe a series of pediatric patients with skin of color who presented with the chief complaint of facial skin lightening and had associated clinical findings consistent with lichen nitidus.

Retrospective review of confluent and reticulated papillomatosis in pediatric patients.

BACKGROUND/OBJECTIVES: Confluent and reticulated papillomatosis is a skin condition with unclear etiology and limited understanding of risk factors, comorbidities, and treatment strategies in the pediatric population. This study aims to describe the varied presentations and outcomes of confluent and reticulated papillomatosis and report associated comorbidities in pediatric patients. METHODS: In this retrospective single-institution case-control study, pediatric patients with a diagnosis of confluent and reticulated papillomatosis seen between 2012 and 2020 were matched approximately 1:5 with an acne vulgaris cohort based on diagnosis, setting, and time period when seen. The primary measures were the clinical features, demographics, comorbidities, treatment, and outcomes of patients with confluent and reticulated papillomatosis. Univariate and multivariable analyses were conducted to describe the association of confluent and reticulated papillomatosis with several potential risk factors and comorbidities. RESULTS: Patients with confluent and reticulated papillomatosis typically presented in adolescence with a median age of 14 years and female predominance. In a multivariable analysis, patients with confluent and reticulated papillomatosis were significantly more likely to identify as Black, be overweight or obese, and have concurrent acanthosis nigricans compared to control patients. Most of the confluent and reticulated papillomatosis patients were treated with oral minocycline or doxycycline. Although all patients who received antibiotics responded to treatment, approximately half presented with recurrence, typically within 1-2 years of first treatment. CONCLUSIONS: Confluent and reticulated papillomatosis is a disorder that presents in adolescence and appears to be more frequent in patients who are Black, obese, or overweight, and also have acanthosis nigricans. Clinicians should assess patients with confluent and reticulated papillomatosis for comorbidities, particularly those associated with insulin resistance, which may help reduce long-term disease burden.

Training in pediatric skin of color: Suggested curricular guidelines of the pediatric dermatology research alliance special interest group in pediatric skin of color.

BACKGROUND: Deficiencies in the skills necessary to diagnose and manage patients with skin of color may contribute to health disparities. The Pediatric Dermatology Research Alliance Special Interest Group in Pediatric Skin of Color (PSOCG) convened to generate a curriculum of topics required for basic pediatric skin of color (PSOC) education for medical students and residents in dermatology to improve the quality of education in PSOC. METHODS: A survey was distributed to the PSOCG members to assess expert opinion regarding critical topics for inclusion in a basic PSOC syllabus. Video conference and two rounds of survey were used to rank topics for inclusion and to highlight the underlying need for inclusion. RESULTS: Group members composed of academic pediatric dermatologists with teaching responsibilities including skin of color topics for dermatology residents and medical students. Learning objectives were developed for an educational lecture on basic science, and clinical conditions affecting PSOC were grouped by age-infantile, pediatric, and adolescent skin conditions affecting the PSOC population were identified for inclusion with rank score based on specific parameters including greater frequency in skin of color, nuances in skin of color, and need for medical workup. CONCLUSIONS: Increased focus on PSOC education is needed to improve quality of care for children of color through enhanced knowledge. Inflammatory, genetic, and particularly primary pigmentary disorders should be the focus of a broad curriculum in pediatric skin of color education for medical trainees. Objectives should include improved diagnosis, treatment, and ability to educate patients and their families regarding the nature of their conditions.

Novel ERCC2 mutation in two siblings with trichothiodystrophy.

Trichothiodystrophy describes a group of recessively inherited multisystem neuroectodermal disorders that takes its name from the characteristic feature of brittle, sulfur-deficient hair. We describe two siblings with trichothiodystrophy due to a novel genotype. The maternal mutation (p.Arg722Trp) is a previously described pathogenic mutation in ERCC2 that has been shown to result in a severe phenotype, while the paternal mutation (c.1480-1G > C) has not been previously reported. Our cases confirm the severe phenotype associated with the p.Arg722Trp mutation and expand the known genetic mutations associated with trichothiodystrophy by demonstrating a novel pathogenic mutation in ERCC2.

A review of cutaneous hypersensitivity reactions in infants: From common to concerning.

Cutaneous hypersensitivity reactions in infants present in a variety of patterns. These skin eruptions can be dramatic, causing alarm in parents and medical personnel. Many of these syndromes have overlapping features, which adds to the confusion and uncertainty regarding diagnosis and management. This review discusses the spectrum of hypersensitivity responses with a focus on their presentation in infants. The clinical findings, pathophysiology, histopathology, management, and complications of these conditions will be reviewed.

Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate.

Langerhans cell histiocytosis is exceedingly rare in premature infants, and the few cases reported suggest a poor prognosis with systemic involvement. We present a case of Langerhans cell histiocytosis limited to a single cutaneous lesion, presenting in a 27-week-gestation infant, which is the youngest gestational age of reported Langerhans cell histiocytosis cases. The lesion showed spontaneous resolution by 41 weeks corrected gestational age, and systemic involvement was absent, demonstrating a mild course of skin-only Langerhans cell histiocytosis in a premature infant.

Alopecia areata treated with hydroxychloroquine: A retrospective study of nine pediatric cases.

BACKGROUND/OBJECTIVES: Alopecia areata is a common hair loss condition that is often emotionally devastating for patients. There is a paucity of effective treatments available. Hydroxychloroquine has been reported as variably effective in inducing significant hair regrowth in adults with alopecia areata. The objective of this retrospective study was to assess the benefit and tolerability of hydroxychloroquine in pediatric alopecia areata. METHODS: We conducted a retrospective review of nine children with a history of alopecia areata treated with hydroxychloroquine. Clinical data were obtained from patients treated at two tertiary care centers in the United States between July 1, 2013, and July 1, 2015. RESULTS: Alopecia scores of five patients improved by 6 months of treatment. Four patients experienced no improvement from baseline evaluation. The most common side effect associated with treatment was gastrointestinal intolerance and headache. CONCLUSION: This retrospective series suggests that hydroxychloroquine can be considered as a treatment option for alopecia areata in children.

Vitiligoid variant of lichen sclerosus in young girls with darker skin types.

BACKGROUND/OBJECTIVES: Vitiligo and lichen sclerosus are autoimmune disorders characterized by white discoloration, and both frequently affect the anogenital region. Vitiligoid lichen sclerosus refers to a superficial variant of lichen sclerosus in which the lesion appears clinically to be vitiligo based on the predominant presentation of depigmentation and minimal inflammation and sclerosis but histologically is consistent with lichen sclerosus. A limited number of reports have described vitiligoid lichen sclerosus, and from these reports, it appears to primarily affect darker-skinned people. METHODS: We retrospectively reviewed the records of 7 girls with darker skin types seen in our pediatric dermatology clinic who presented with a clinical overlap of vitiligo and lichen sclerosus. All had primarily well-demarcated, depigmented patches characteristic of vitiligo, but the lesions were symptomatic (pruritus, pain, bleeding, constipation), a presentation more consistent with lichen sclerosus. RESULTS: The girls were all treated with high-potency topical steroids, calcineurin inhibitors, or both. The associated symptoms improved or resolved, but most had minimal improvement of the depigmentation. CONCLUSIONS: The girls presented in this series appear to have had vitiligoid lichen sclerosus, given the clinical overlap of lichen sclerosus and vitiligo affecting the anogenital region, particularly given that they did not have depigmented patches elsewhere on their body. Previous cases of vitiligoid lichen sclerosus have been reported in darker skin types, and our findings support this possible predisposition. It is important for clinicians to assess patients presenting with genital depigmentation for overlapping features of vitiligo and lichen sclerosus and determine appropriate management.

Molluscum Contagiosum of the Gluteal Cleft: Observations and Implications for Management in Five Children.

Molluscum contagiosum (MC) is a common, self-limited skin infection caused by a double-stranded DNA virus of the family Poxviridae. Although the morphology of the disease is well described, MC presenting at unusual sites can exhibit atypical morphology, resulting in misdiagnosis. This observational, retrospective case series discusses the novel morphology of MC presenting in the intergluteal cleft of five children seen in the outpatient dermatology clinic of an academic medical center. We present this case series to highlight this novel presentation of MC in children and to emphasize a conservative approach to management given the sensitive location and the self-limited nature of the infection.