Biopsy-proven spontaneous regression of a rhabdomyomatous mesenchymal hamartoma.

Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare, benign, congenital tumor of the dermis and subcutaneous tissue comprised of skeletal muscle and adipose and adnexal elements. Although the majority of cases are an incidental finding in otherwise healthy patients, some have been reported in association with other anomalies. We present a full-term boy evaluated on day 2 of life for two lesions located on the midline of the lower back and right buttock that each appeared clinically as an atrophic, pink plaque. Ultrasound of the midline lesion revealed an underlying lipomyelomeningocele with a tethered cord in the spinal canal. Histopathology of the right buttock cutaneous lesion was consistent with a diagnosis of RMH. Surgical excision was performed on the midline intradural lipoma and the lesion on the buttock was monitored clinically. Repeat biopsy of this site at 1 year of age revealed complete spontaneous regression. This case highlights three interesting features: the association with an occult spinal dysraphism lipomyelomeningocele and tethered cord, the clinical presentation of an atrophic plaque as opposed to the more commonly reported raised lesions, and the phenomenon of spontaneous regression of the lesion. Most importantly, this final feature of regression in our patient suggests that, in the absence of symptoms, clinical observation of RMH lesions is warranted for spontaneous regression for 1 to 2 years provided that no functional deficit is noted and that the cutaneous or deeper lesions are not causing a medical problem.

Aplasia cutis congenita in a setting of fetus papyraceus associated with small fetal abdominal circumference and high alpha-fetoprotein and amniotic acetylcholinesterase.

Fetus papyraceus is the fetal death of one or more fetuses in a multiparous pregnancy. The surviving infants can experience extensive aplasia cutis in an H-shaped distribution over the flanks and abdomen as a consequence of the loss of their fetal sibling. We report the case of a monochorionic, diamniotic pregnancy complicated by a single fetal death at 13 weeks of gestational age. Aplasia cutis of the surviving twin was suggested in utero by three criteria: high amniotic and maternal alpha-fetoprotein, detectable acetylcholinesterase, and small abdominal circumference on prenatal ultrasound. This constellation of findings in the setting of fetus papyraceus can be an indicator of aplasia cutis in the surviving fetus.

Pediatric dermatology training survey of United States dermatology residency programs.

Variability exists in pediatric dermatology education for dermatology residents. We sought to formally assess the pediatric dermatology curriculum and experience in a dermatology residency program. Three unique surveys were developed for dermatology residents, residency program directors, and pediatric dermatology fellowship program directors. The surveys consisted of questions pertaining to residency program characteristics. Sixty-three graduating third-year residents, 51 residency program directors, and 18 pediatric dermatology fellowship program directors responded. Residents in programs with one or more full-time pediatric dermatologist were more likely to feel very competent treating children and were more likely to be somewhat or extremely satisfied with their pediatric curriculums than residents in programs with no full-time pediatric dermatologist (50.0% vs 5.9%, p = 0.002, and 85.3% vs 52.9%, p < 0.001, respectively). Residents in programs with no full-time pediatric dermatologist were the only residents who were somewhat or extremely dissatisfied with their pediatric training. Residency program directors were more satisfied with their curriculums when there was one or more pediatric dermatologist on staff (p < 0.01). Residents in programs with pediatric dermatology fellowships were much more likely to report being extremely satisfied than residents in programs without a pediatric dermatology fellowship (83.3% vs 21.2%; p < 0.001). The results of this survey support the need for dermatology residency programs to continue to strengthen their pediatric dermatology curriculums, especially through the recruitment of full-time pediatric dermatologists.