Hidradenitis suppurativa (HS) is a common skin disease in children and young adults. In this report, we describe an unusual case of HS presenting as a mammillary fistula (MF) in a teenage female. A ...thorough dermatologic history and exam resulted in diagnosis of HS. Identifying the underlying disease is key to appropriate treatment of a relapsing MF in the setting of HS.
Acne vulgaris is an extremely common chronic disease of the pilosebaceous unit. Despite its ubiquity, acne in the childhood years of approximately ages 1–6 years is exceedingly rare. Physicians ...should be suspicious of underlying systemic disease processes in patients of this age who present with onset of acne lesions, as pre‐pubertal acne in childhood has a distinctly different pathology than that of other age groups. Through a case series, we highlight the importance of a thorough work‐up and provide a review on when to refer to pediatric endocrinology to rule out precocious puberty and tumors as the cause of pre‐pubertal acne.
Keratosis pilaris is a common skin condition associated with a number of syndromes, including collagen type VI‐related disorders. Our patient, recently diagnosed with Ullrich congenital muscular ...dystrophy, presented with severe keratosis pilaris, hypotonia, and velvety skin on the palms and soles. We present this case to highlight the importance of including cutaneous findings, such as keratosis pilaris, to aid in the diagnosis when evaluating patients with syndromic features.
Congenital erosive and vesicular dermatosis (CEVD) is a rare skin condition that most commonly presents as erosive and vesicular lesions on the trunk and extremities in premature infants and heals ...with characteristic reticulated and supple scarring (RSS). The exact pathogenesis of CEVD is unknown and is typically a diagnosis of exclusion. We present the cases of two extremely premature neonates with Candida septicemia who were found to have diffuse, erythematous skin eruptions shortly after birth that ultimately healed with RSS. Through these cases, we highlight the importance of including fungal infection in the work-up of CEVD healing with RSS.
Hemangioma-related syndromes Valdebran, Manuel; Wine Lee, Lara
Current opinion in pediatrics,
08/2020, Letnik:
32, Številka:
4
Journal Article
Recenzirano
There is a growing understanding of complications and anomalies associated with infantile hemangiomas. The current review will discuss recent clinical advances in syndromes associated with segmental ...hemangiomas, including PHACE and LUMBAR syndrome. In addition, the importance of recognizing visceral hemangiomatosis is highlighted.
Ongoing longitudinal studies of PHACE and LUMBAR syndromes associated with segmental infantile hemangiomas have led to improved diagnosis and recommendations for screening for associated anomalies. Characterization of a growing spectrum of associated anomalies as well as better classification of at-risk patients will improve diagnosis and outcomes. In addition, visceral hemangiomatosis recognition and understanding of the potential association with consumptive hypothyroidism will improve initiation of appropriate screening.
Clinicians should be aware of infantile hemangiomas associated with potential syndromic complications and recognize the need to initiate appropriate work-up. Segmental hemangiomas of the head and neck region may indicate a risk of PHACE syndrome and associated developmental anomalies. Although LUMBAR syndrome is the association of lower body segmental hemangioma with developmental anomalies. Visceral hemangiomas most commonly affect the liver and may be associated with complications such as consumptive hypothyroidism and heart failure.
Hereditary alpha tryptasemia (HaT), an autosomal dominant condition first described in 2014, has previously been associated with multiple dermatologic, allergic, gastrointestinal, neuropsychiatric, ...autonomic, and connective tissue abnormalities. We describe a pediatric patient with predominantly mixed cutaneous inflammatory manifestations and atopic manifestations resistant to treatment who was found to have HaT. HaT should be considered in individuals with refractory inflammatory dermatologic disease and signs and/or symptoms concerning for mast cell activation.
Many pediatric psoriasis patients suffer from nail involvement and psoriatic arthritis. In adults, biologic agents have demonstrated success in treating refractory nail psoriasis and arthritis, but ...studies are limited in children. In this report, we present a pediatric patient with severe, recalcitrant nail and joint psoriasis, successfully treated with secukinumab.
Current regulatory labeling recommends avoiding live vaccine use in dupilumab-treated patients. Clinical data are not available to support more specific guidance for live or live attenuated vaccines ...administration in dupilumab-treated patients.
Children (6 months-5 years old) with moderate-to-severe atopic dermatitis (AD) enrolled in a phase 2/3 clinical trial of dupilumab (LIBERTY AD PRESCHOOL Part A/B; NCT03346434) and subsequently participated in the LIBERTY AD PED-OLE (NCT02612454). During these studies, protocol deviations occurred in nine children who received measles, mumps, rubella (MMR) vaccine with or without varicella vaccine; five with a ≤12-week gap between dupilumab administration and vaccination and four with a >12-week gap after discontinuing dupilumab.
Nine children (1 female; 8 male) had severe AD at baseline (8-56 months old). Of the nine children, five had a ≤12-week gap ranged 1-7 weeks between dupilumab administration and vaccination who received MMR vaccine (n = 2) or MMR and varicella vaccines (n = 3); among these, one resumed dupilumab treatment as early as 2 days and four resumed treatment 18-43 days after vaccination. No treatment-emergent adverse events, including serious adverse events and infections, were reported within the 4-week post-vaccination period in any children.
In this case series of dupilumab-treated children with severe AD who received MMR vaccine with or without varicella vaccine, no adverse effects (including vaccine-related infection) were reported within 4 weeks after vaccination. Further studies are warranted to evaluate the safety, tolerability, and immune response to live attenuated vaccines in dupilumab-treated patients.
Intravenous immunoglobulin (IVIg) is a frequently used treatment modality in the pediatric inpatient population for acute diseases such as Kawasaki disease and Stevens‐Johnson syndrome. There are few ...reported cutaneous adverse events after IVIg in the pediatric population. Here, we present two patients with psoriasiform dermatitis appearing after IVIg treatment for two different disease processes, Kawasaki disease and mycoplasma‐associated mucositis, suggesting an association with the treatment instead of the disease process.
Variants in RAS are known drivers of certain pediatric blood and solid cancers, including brain tumors. Though most RAS‐driven cancers are thought to occur sporadically, genetic syndromes caused by ...germline RAS variants portend a slightly higher risk of rhabdomyosarcoma (RMS) development. Three new cases and a review of the literature demonstrate that in rare cases, certain somatic RAS variants are associated with an increased risk of RMS and that RMS development may be heralded by the presence of concomitant RAS‐driven birthmarks. Further prospective studies are needed to establish incidence and recommend appropriate monitoring guidelines for patients at risk.
