Lichen Sclerosus: A Survey of Diagnosis and Management Among Pediatric Dermatologists and Gynecologists.

BACKGROUND/OBJECTIVES: Lichen sclerosus (LS) is a chronic condition that warrants close follow-up due to the risk of scarring. The optimal long-term management of pediatric vulvar and perianal lichen sclerosus (PVPLS) is unknown. This study aimed to identify diagnostic, treatment, and maintenance regimens among pediatric dermatologists and pediatric/adolescent gynecologists, as well as assess provider confidence and desire for guidance on long-term PVPLS management. METHODS: A cross-sectional 35-question survey was administered through the Pediatric Dermatology Research Alliance (PeDRA) and the North American Society for Pediatric and Adolescent Gynecology (NASPAG) between 7/13/2021 and 8/30/2021 to ascertain PVPLS diagnostic and management regimens. RESULTS: Most responders were attending-level pediatric/adolescent gynecologists (46%) and pediatric dermatologists (41%). Although 85% of participants felt completely or very confident in diagnosing PVPLS, the majority (86%) desired further management guidelines. While the initial treatment was similar among providers, maintenance regimens and follow-up varied considerably, with only 42% recommending lifelong monitoring despite potential persistence into adulthood. CONCLUSIONS: While initial treatment was similar among practitioners, there was variation by specialty in subsequent management and a lack of uniformity in long-term follow-up. Additional studies are needed to clarify the optimal management of PVPLS and to provide evidence-based guidelines regarding long-term follow-up.  J Drugs Dermatol. 2024;23(6):450-455.     doi:10.36849/JDD.8084.

Pediatric cutaneous T-cell neoplasm and mimics with gamma-delta expression: Not always aggressive.

Pediatric cutaneous T-cell lymphoma with γδ immunophenotype is extremely rare. Only a few cases of γδ T-cell neoplasm have been reported in the literature, and therefore little is known whether γδ T-cell neoplasms in children are distinct from their adult counterparts with respect to the clinicopathological presentation, behavior, and prognosis. In this study, we demonstrate three unique pediatric cutaneous T-cell neoplasm and mimics with increased γδ T cells. All cases showed an indolent clinical course.

Ulcerated diaper dermatitis: Child abuse mimicker.

Irritant diaper dermatitis is a common dermatologic problem among infants and young children. Severe erosive presentations, although uncommon, are diagnostically challenging and can mimic non-accidental trauma (NAT). Diagnosing inflicted injury and NAT where it does not exist can cause parental distress, yet failing to diagnose inflicted injury and NAT can result in re-injury. We describe three cases of severe erosive diaper dermatitis in pediatric patients aged 2-6 years that were initially concerning for inflicted scald burn or neglect.

Severe reactive infectious mucocutaneous eruption mimicking drug-induced epidermal necrolysis triggered by norovirus.

Reactive infectious mucocutaneous eruption (RIME) is an eruptive mucositis with varying degrees of cutaneous involvement presumed to be due to an immunologic response to various infectious pathogens. Most reported cases occur after a prodromal upper respiratory illness. We present a patient with a particularly severe case mimicking drug-induced epidermal necrolysis found to be triggered by asymptomatic norovirus infection, a virus not previously reported in association with RIME.

Insights from a joint pediatric dermatology-gynecology vulvar clinic: A retrospective study.

BACKGROUND/OBJECTIVES: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology-gynecology vulvar clinic at Children's National. METHODS: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology-gynecology clinic were reviewed retrospectively. RESULTS: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. CONCLUSIONS: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone.

Botulinum Toxin Injections as a Treatment of Refractory Vulvodynia in Adolescents: A Case Series.

BACKGROUND: Vulvodynia involves vulvar discomfort that occurs in the absence of an identifiable cause. Because vulvodynia is often accompanied by myofascial pain and pelvic floor tension, transvaginal botulinum toxin (BT) injection into the pelvic floor has been proposed as a possible treatment. METHODS: Retrospective case series RESULTS: Three adolescents with vulvodynia had a suboptimal response to treatment with several interventions, including neuromodulators (oral and topical), tricyclic antidepressants (oral and topical), and pelvic floor physical therapy. Subsequently, these patients underwent BT injections to the pelvic floor as treatment with varying responses. CONCLUSION: In select adolescent patients with vulvodynia, transvaginal BT injection into the pelvic floor can be an effective treatment. Further studies are needed to assess the optimal dosing, frequency, and sites of BT injections in the treatment of vulvodynia in pediatric and adolescent patients.

Myeloid sarcoma in a newborn: A rare manifestation of congenital acute myeloid leukemia.

Cutaneous myeloid sarcoma is rarely present prior to the diagnosis of congenital acute myeloid leukemia (AML); the former is typically diagnosed with or after the leukemia. We report a 2-day-old male born with multiple cutaneous red to violaceous nodules. Histopathologic and immunohistochemistry findings from a skin nodule were suspicious for myeloid sarcoma. Bone marrow biopsy was initially negative for aberrant blasts; however, at age 4 months, AML with a KMT2A gene rearrangement was identified via bone marrow biopsy.

A widespread blistering eruption: diffuse cutaneous mastocytosis.

Diffuse cutaneous mastocytosis with bullous formation is a rare childhood disease. We report a 5-month-old male who presented with a 3-week history of cutaneous bullae and pruritus. On examination, he had erythema of the cheeks bilaterally and diffuse slightly hyperpigmented, indurated skin on his trunk and abdomen. There were tense vesicles, bullae, and erosions linearly arranged on his trunk and extremities. Both the laboratory and imaging workup were normal. Subsequently, a punch biopsy of a vesicle on the abdomen was obtained and findings confirmed a diagnosis of diffuse cutaneous mastocytosis. An EpiPen(r) was prescribed due to the slightly increased anaphylaxis risk compared to other forms of mastocytosis. There are many purported triggers of diffuse cutaneous mastocytosis and there is currently no known cure which makes management of this disease challenging. This case highlights a rare condition for which official treatment guidelines do not exist. A prompt dermatologic diagnosis is necessary to ensure proper workup and regulation is in place.

Numerous Angiomatous Lesions in an Infant.

A 3-month-old infant presented with bright red macules, papules, and plaques that appeared at birth and grew in size and number. What is your diagnosis?

Facial cleft presenting as a congenital facial papule.

Tessier number 3 craniofacial clefts are a rare congenital deformity of the oronasoocular region with variable severity, most often with serious impacts on appearance and function due to involvement of the bone and soft tissue. However, they can occasionally manifest mildly as a skin-colored congenital facial papule present with subtle anatomic anomalies and signs of deeper involvement, such as crusting and oozing. Recognizing that a congenital facial papule, including non-midline lesions, may be the presenting sign of an underlying developmental anomaly is important to avoid missing the diagnosis of a more extensive underlying congenital defect. We present a rare case of a forme fruste variant of a Tessier number 3 craniofacial cleft to raise awareness of its presentation and advise initial management in hopes of improving outcomes.

Congenital Pigmentary Anomalies in the Newborn.

Congenital pigmentary anomalies may be evident at birth or soon after, with some birthmarks becoming apparent later in infancy or early childhood. It is important to recognize various pigmentary anomalies in the neonate, most of which are benign but a subset of which are associated with cutaneous morbidity or systemic ramifications and require further evaluation. This review will focus on pigmentary mosaicism, congenital melanocytic nevi, nevus spilus, dermal melanocytosis, and pigmentary anomalies associated with neurofibromatosis type 1 (café au lait spots, freckling, plexiform neurofibromas, nevus anemicus), tuberous sclerosis (hypomelanotic macules), and incontinentia pigmenti.

Use of psoralen therapies obtained abroad in two children with Vitiligo.

We describe two American-born children with vitiligo, each of whom travelled to their family's ancestral home (India and Ethiopia), where their skin conditions were treated with PUVAsol, which involves the use of topical or oral psoralens followed by exposure to natural sunlight. Both children experienced modest repigmentation and were subsequently seen in our dermatology clinics. PUVAsol may be an attractive treatment option for some families, but there are potentially serious side effects including phototoxicity and cutaneous malignancy. Dermatologists should be aware of the existence of this treatment modality as well as its complications.

Current Issues in the Treatment of Acne Vulgaris.

Acne vulgaris is an extraordinarily common skin condition in adolescents. The mainstays of acne treatment have remained largely unchanged over recent years. In the context of increasing antibiotic resistance worldwide, there is a global movement away from antibiotic monotherapy toward their more restrictive use. Classically reserved for nodulocystic acne, isotretinoin has become the drug of choice by dermatologists for moderate to severe acne. Given the virtually ubiquitous nature of acne in teenagers, there remains an appreciable need for novel therapies. In this article, we will cover the currently used acne treatments, evaluate the issues and data supporting their use, explore the issues of compliance and the mental health implications of acne care, and recommend directions for the field of acne management in adolescents in the years ahead.

The case of missing skin: Congenital Volkmann ischemic contracture.

Congenital Volkmann ischemic contracture (CVIC) is an exceedingly rare neonatal compartment syndrome caused by intrauterine ischemia and external compression. It presents at birth with necrotic cutaneous lesions and neurologic impairment, typically in a distal upper extremity. Diagnosis and treatment are often delayed in neonates, leading to long-term neurologic sequelae. We present a rare case of CVIC in order to raise awareness of its presentation and management in hopes of improving outcomes.

Treatment of benign cephalic histiocytosis with topical 1% rapamycin ointment.

We report a pediatric case of extensive, progressive benign cephalic histiocytosis (BCH) involving the face, trunk, and extremities with response of facial lesions to treatment with topical 1% rapamycin. A split-face model was used to demonstrate improvement on the treated side versus the untreated side. After physician and parental perception of effectiveness, based in part on photodocumentation, subsequently both cheeks were treated with continued improvement.

Common Neonatal Skin Lesions: Melanocytic Nevi, Pigment Alterations, and Nonmelanocytic Nevi.

Birthmarks are common in the healthy population and are generally harmless. Certain presentations, however, raise concern for associated syndromes or potential complications. It is important for pediatricians to be familiar with both harmless and potentially concerning birthmarks. This article discusses congenital melanocytic nevi, café-au-lait macules, hypomelanotic macules, nevus depigmentosus, nevus anemicus, epidermal nevi, and nevus sebaceous, including potential syndromes and complications. [Pediatr Ann. 2019;48(1):e23-e29.].