Pediatric Dermatology最新文献

We describe four cases of neonatal epidermal skin loss presenting at birth, secondary to disseminated herpes simplex virus (HSV) infection. In three, there was a strikingly similar presentation of 80% epidermal skin loss with sparing of the hands, feet, and areas on either the face or scalp. In the fourth neonate, the skin loss was less extensive but followed a similar pattern. This case series highlights an under-recognized pattern of skin involvement in systemic neonatal HSV, and the importance of early recognition and optimal management to improve outcomes.

Congenital smooth muscle hamartomas (CSMH) are benign hamartomatous proliferations (of the smooth muscles of the arrector pili muscles). They are most often present at birth and associated with many clinical and pathological signs common to Becker's nevus (BN). Pathogenic mosaic variants in the beta actin gene (ACTB) have been described in both entities. We present two children with CSMH in whom an identical and previously unreported likely pathogenic variant of ACTB was identified. This supports the hypothesis that Becker's nevus and CSMH are part of the same nosological spectrum; therefore, the term CSMH is more appropriate.

Longitudinal melanonychia in children is usually caused by nail matrix nevi and often fades over time. Pediatric patients with this finding are frequently referred to dermatologists due to concerns that it may represent melanoma. We report a case of a 4-year-old girl with longitudinal melanonychia on the left second fingernail, accompanied by pigmented dots in the cuticle that persisted after the melanonychia had faded.

Dissecting cellulitis of the scalp (DCS) is a chronic inflammatory condition characterized by painful, draining nodules and progressive, scarring alopecia. It is often refractory to treatment, and there are currently no FDA-approved therapies. We present a case of a 15-year-old boy with keratitis-ichthyosis-deafness (KID) syndrome and DCS who experienced significant clinical response to treatment with adalimumab after failing multiple courses of oral antimicrobials, surgical interventions, intralesional corticosteroids, and topical therapies. Treatment with adalimumab led to reduction in drainage and pain and visible hair regrowth, highlighting its therapeutic potential in refractory, pediatric DCS including in the setting of syndromic skin disease.

Information is lacking on parental recognition and management practices for dermatophytosis, a common childhood infection associated with increasing antifungal resistance. We analyzed data from a nationwide survey of US mothers with children < 18 years living at home, using chi-squared tests to compare respondents who indicated they would treat dermatophytosis with over-the-counter corticosteroids versus those who would not. Among the 306 respondents, 47% correctly identified ringworm and 63% misattributed erythema migrans as ringworm; 17% said they would try an over-the-counter corticosteroid cream for suspected dermatophytosis, a practice more frequently reported among respondents who were non-Hispanic Black (29% vs. 11%), Hispanic/Latino (10% vs. 8%), and non-Hispanic multiracial (16% vs. 11%) (p = 0.009) and who had a high school education or less (45% vs. 27%, p = 0.032). Our study emphasizes the challenges of visually diagnosing dermatophytosis and the importance of early healthcare evaluation to ensure accurate diagnosis and proper treatment.

We describe a rare case of aggressive systemic mastocytosis (ASM) in a preterm neonate born at 33 weeks' gestation, with prenatal findings of polyhydramnios and hepatosplenomegaly. Postnatal evaluation revealed extensive cutaneous lesions. Her skin and liver biopsies confirmed mast cell infiltration. Molecular analysis identified a somatic KIT D816V mutation. Treatment with midostaurin, a multikinase inhibitor, led to significant clinical improvement, including resolution of skin lesions and reduction in hepatosplenomegaly. This case highlights the importance of early recognition of cutaneous signs in neonatal ASM and supports the potential role of targeted therapy with midostaurin.

Regional (segmental) infantile hemangioma (IH) can be associated with arterial anomalies, as seen in PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies) and LUMBAR (lower body infantile hemangiomas, urogenital anomalies and ulceration, myelopathy, bony deformities, anorectal malformations and arterial anomalies, and rectal anomalies) associations. We describe two patients with a regional IH on the hand associated with Raynaud phenomenon. We hypothesize that Raynaud phenomenon in the setting of a regional IH may be due to post-traumatic vasospastic response, arterial anomalies, cutaneous atrophy due to the IH, and/or changes in vasomotor regulation.