Acral Changes in pediatric patients during COVID 19 pandemic: Registry report from the COVID 19 response task force of the society of pediatric dermatology (SPD) and pediatric dermatology research alliance (PeDRA).

BACKGROUND/OBJECTIVE: In spring 2020, high numbers of children presented with acral pernio-like skin rashes, concurrent with the coronavirus disease 2019 (COVID-19) pandemic. Understanding their clinical characteristics/ infection status may provide prognostic information and facilitate decisions about management. METHODS: A pediatric-specific dermatology registry was created by the Pediatric Dermatology COVID-19 Response Task Force of the Society for Pediatric Dermatology (SPD) and Pediatric Dermatology Research Alliance (PeDRA) and was managed by Children's Hospital of Philadelphia using REDCap. RESULTS: Data from 378 children 0-18 years entered into the registry between April 13 and July 17, 2020 were analyzed. Data were drawn from a standardized questionnaire completed by clinicians which asked for demographics, description of acral lesions, symptoms before and after acral changes, COVID-19 positive contacts, treatment, duration of skin changes, laboratory testing including SARS-CoV-2 PCR and antibody testing, as well as histopathology. 229 (60.6%) were male with mean age of 13.0 years (± 3.6 years). Six (1.6%) tested positive for SARS-CoV-2. Pedal lesions (often with pruritus and/or pain) were present in 96%. 30% (114/378) had COVID-19 symptoms during the 30 days prior to presentation. Most (69%) had no other symptoms and an uneventful course with complete recovery. CONCLUSIONS AND RELEVANCE: Children with acral pernio-like changes were healthy and all recovered with no short-term sequelae. We believe these acral changes are not just a temporal epiphenomenon of shelter in place during the spring months of the first wave of the COVID-19 pandemic and may be a late phase reaction that needs further study.

Propranolol for infantile haemangiomas: initiating treatment on an outpatient basis.

INTRODUCTION: Propranolol was recently discovered to be an effective treatment for infantile haemangiomas, and varying doses and monitoring regimens have been proposed. Adverse events, although uncommon, have been reported. MATERIALS AND METHODS: This was a retrospective chart review of infants with haemangiomas who were started on propranolol at a dose of 3 milligrams per kilogram per day on an outpatient basis. After a baseline cardiac evaluation including an electrocardiogram and an echocardiogram, treatment was initiated during 6 hours of observation. RESULTS: A total of 15 patients were identified; however, only 13 returned for at least one follow-up visit. This cohort was followed up for a median of 2.8 months with a range from 0.2 to 10.0. No hypotension, hypoglycaemia, bronchospasm, or clinically significant bradycardia occurred during treatment. All patients had clinical improvement of their haemangiomas. CONCLUSIONS: This study suggests that initiating treatment during outpatient observation may be a reasonable alternative to inpatient admission. In addition, expensive testing may not be necessary during pre-treatment screening when the physical examination is normal.

Eczematous nevus sebaceus: a report of three cases.

Meyerson's phenomenon is a well-documented inflammatory reaction described in a variety of cutaneous lesions, including the original description in nevocellular nevi (1). Such an inflammatory reaction was subsequently described in melanocytic and a sundry of nonmelanocytic lesions alike, including vascular malformations (2-11). We present three cases of infants with nevus sebaceus on the scalp, which were obscured by an eczematous, eosinophilic reaction reminiscent of that first described by Meyerson. Two of the patients had concomitant atopic dermatitis, but one had no association. Recognition of this secondary feature is important in establishing the correct diagnosis.

Widespread granulomatous dermatitis of infancy: an early sign of Blau syndrome.

BACKGROUND: Pediatric sarcoidosis has traditionally been divided into 2 distinct groups: (1) school-aged children and adolescents with frequent involvement of the lungs and mediastinal lymph nodes (similar to adult sarcoidosis) and (2) infants and preschoolers with the triad of arthritis, uveitis, and a cutaneous eruption of discrete small papules, referred to as early-onset sarcoidosis. Blau syndrome, a rare autosomal dominant genodermatosis caused by mutations in the NOD2 (nucleotide-binding oligomerization domain 2) gene, has been considered as the familial form of early-onset sarcoidosis. OBSERVATIONS: A 9-month-old boy developed an asymptomatic eruption of 1- to 2-mm, red-brown to pinkish tan, flat-topped papules on the face, trunk, and extremities. There was no evidence of ocular involvement or arthritis. The skin lesions were characterized histologically by noncaseating granulomas in a periadnexal distribution within the dermis. A family history of uveitis supported a diagnosis of Blau syndrome, and analysis of the NOD2 gene revealed a heterozygous gain-of-function missense mutation (Arg334Trp) that has previously been detected in Blau syndrome kindreds. CONCLUSION: We draw attention to granulomatous dermatitis as an early manifestation of Blau syndrome and highlight emerging molecular evidence that this heritable autoinflammatory disorder and early-onset sarcoidosis represent a single disease entity.

"Pediatric blaschkitis": expanding the spectrum of childhood acquired Blaschko-linear dermatoses.

We describe two young children who developed relapsing, pruritic, papulovesicular eruptions in multiple bands along Blaschko lines on the neck, trunk, and extremities. Skin specimens in both revealed spongiotic dermatitis. This represents the first report of "blaschkitis" in children, providing further evidence that lichen striatus and blaschkitis are related acquired Blaschko-linear dermatoses that exist on a spectrum rather than as the childhood and adult form of a single disease entity. We highlight the features that differentiate blaschkitis from lichen striatus, review the potential roles of cutaneous mosaicism, environmental triggers, and background immunologic state in their pathogenesis, and discuss the spectrum of inflammatory dermatoses that can follow Blaschko lines.

Erythema multiforme limited to the oral mucosa in a teenager on oral contraceptive therapy.

BACKGROUND: Erythema multiforme has been linked to numerous drugs and infectious agents. A link to oral contraceptive use has been reported in the past in the adult population but thus far has not been reported in children or adolescents. CASE: We report the case of an 18-yr-old female who developed oral erosions consistent with erythema multiforme two and a half weeks after initiating therapy with an oral contraceptive agent. A thorough examination for other inciting factors was negative, and the lesions slowly resolved over the course of 3 weeks. CONCLUSIONS: This case illustrates that erythema multiforme should be considered in the differential diagnosis of adolescents with oral erosions who have been prescribed oral contraceptives.

Diagnosis and management of diaper dermatitis.

This article presents an overview of diaper dermatitis for the pediatric community. The pathogenesis, differential diagnosis, and management of this common condition in infancy are reviewed. This information will assist in making the appropriate diagnosis and managing this irritant contact dermatitis of the diaper area. With conservative management, most cases of irritant diaper dermatitis are self-limited. When the condition persists, one must consider other diagnoses.

The misnomer "macrocephaly-cutis marmorata telangiectatica congenita syndrome": report of 12 new cases and support for revising the name to macrocephaly-capillary malformations.

BACKGROUND: The condition known as macrocephaly-cutis marmorata telangiectatica congenita syndrome (M-CMTC) is a rare congenital syndrome of unknown etiology characterized by macrocephaly and vascular lesions that have been described as either cutis marmorata or cutis marmorata telangiectatica congenita (CMTC). Most patients also exhibit facial and limb asymmetry; somatic overgrowth; developmental delay; capillary malformations of the nose, philtrum, and/or upper lip; neurologic abnormalities; syndactyly or polydactyly; craniofacial abnormalities; and joint laxity or soft skin. OBSERVATIONS: We describe 12 patients with this condition from tertiary care medical centers (8 cases) and accrued via an M-CMTC support group Web site (4 cases). All patients showed reticulated or confluent port-wine stains (PWS), not CMTC. Seven of the 12 patients also had centrofacial capillary malformations. In our comprehensive review of 100 previously reported cases, only 34 were accompanied by photographs that were sufficiently clear to review for diagnostic purposes. None had true CMTC, with most having reticulated PWS or persistent cutis marmorata. CONCLUSIONS: Reticulated or confluent PWS and persistent capillary malformations of the central face, rather than CMTC, are the most characteristic cutaneous vascular anomalies seen in so-called M-CMTC syndrome. The name macrocephaly-capillary malformations (M-CM) more accurately reflects the features of this syndrome.

Primary cutaneous zygomycosis in two immunocompromised children.

Zygomycosis, often referred to as ''mucormycosis'' or ''phycomycosis,'' is a rapidly progressive fungal infection which usually occurs in immunocompromised individuals, and is characterized by soft tissue destruction and invasion of blood vessels. The rare and easily misdiagnosed primary cutaneous form may present as a superficial erosion with a painless, gradual onset and slow progression of symptoms or a gangrenous, necrotic ulceration due to rapid tissue and vascular invasion. With the latter form, the mortality rate among affected individuals is high even after aggressive surgical debridement and amphotericin B administration, emphasizing the importance of early recognition and proper diagnosis. We present two instances of gangrenous cutaneous zygomycosis in immunocompromised children and review the literature with regard to etiology, diagnosis and treatment, highlighting the pediatric population.

Diaper dermatitis that does not quit.

Diaper dermatitis is one of the most common skin disorders in infants. The humid, moist environment under the diaper makes the skin more susceptible to injury from exposure to irritants particularly related to urine and feces. A gentle cleansing routine, frequent diaper changes, and a thick barrier cream help control this condition. Irritant diaper dermatitis should be distinguished from other skin conditions that may develop in this sensitive area.

Confluent and reticulated papillomatosis associated with tinea versicolor in three siblings.

We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and reticulated papillomatosis, and the only report describing family members having both confluent and reticulated papillomatosis and tinea versicolor. This report lends further evidence to the hypothesis that confluent and reticulated papillomatosis may be etiologically linked to tinea versicolor, and also suggests a genetic predisposition for it.

Fabry disease: an atypical presentation.

Fabry disease is a rare X-linked recessive lysosomal storage disease. Patients typically have angiokeratomas distributed between the umbilicus and knees, painful crises of the hands and feet, and renal, ophthalmologic, and cardiac abnormalities. An 11-year-old boy presented with a 6-year history of widespread petechial-like lesions and painful crises of the hands and feet. On physical examination, he had numerous erythematous, nonblanching pinpoint macules and rare papules with an overlying crust. These lesions were widely distributed on his trunk, palms, and soles, while sparing the area between the umbilicus and knees. Histologic evaluation of one of these lesions found several dilated, blood-filled vessels in the upper dermis beneath a thinned epidermis. The patient also had markedly decreased alpha galactosidase A levels. Although the distribution of the angiokeratomas was atypical, the clinical and histologic findings were consistent with a diagnosis of Fabry disease.

Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas.

An association exists among neurofibromatosis 1 (NF1), juvenile xanthogranulomas (JXGs), and juvenile myelomonocytic leukemia (JMML). The authors describe a patient with the triple association of JXG, NF1, and JMML initially presenting with features of hemophagocytic lymphohistiocytosis (HLH). An 18-month old boy had multiple cutaneous and gastrointestinal JXG and NF1. At 3 years of age he developed anemia, thrombocytopenia, and hepatosplenomegaly. A bone marrow biopsy revealed features of HLH. Despite chemotherapy, he went on to develop JMML, which proved fatal.

Infantile systemic hyalinosis.

Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.