Acute acral eruptions in children during the COVID-19 pandemic: Characteristics of 103 children and their family clusters.

BACKGROUND: A marked increase in frequency of acute acral eruptions (AAE) was observed in children during the COVID-19 pandemic in the spring period. OBJECTIVES: In this observational multicenter study, based on children with AAE, we aimed to assess the proportion of household members possibly infected by SARS-CoV-2. METHODS: We collected data from all children observed with AAE, prospectively from April 7, 2020 to June 22, 2020, and retrospectively since February 28, 2020. The primary outcome was the household infection rate, defined as the proportion of family clusters having at least one member with COVID-19 infection other than the child with AAE ("index child"). The definition of a case was based on characteristic clinical signs and a positive PCR or serology. RESULTS: The study included 103 children in 10 French departments and in Quebec. The median age was 13 years and the interquartile range [8-15], with a female-to-male ratio of 1/1.15. In children with AAE, all PCR tests were negative (n=18), and serology was positive in 2/14 (14.3%) cases. We found no significant anomalies in the lab results. A total of 66 of the 103 families (64.1%) of included children had at least one other infected member apart from the index child. The total number of household members was 292, of whom 119 (40.8%) were considered possibly infected by SARS-CoV-2. No index children or households exhibited severe COVID-19. DISCUSSION: Among the 103 households included, 64.1% had at least one infected member. Neither children with AAE nor their households showed severe COVID-19.

Short- and long-term outcome of linear morphoea in children.

BACKGROUND: Linear morphoea (LM) is a rare fibrosing disorder of the limbs or the face that may cause functional disability and severe aesthetic sequelae. Despite a wide range of therapeutics reported for LM, there is currently a lack of consensus on the optimal therapy. Little is known about the long-term outcome of this disease. OBJECTIVES: To describe the short- and long-term outcome of a large series of patients with LM acquired in childhood. METHODS: A retrospective chart review of 52 paediatric patients with LM seen in our centre during a 20-year span (1990-2010) and a telephone survey in 2011 to assess the long-term outcome of these patients. RESULTS: Limbs were affected twice as often as the face, with a higher proportion of female patients. Stabilization was obtained after a mean disease duration of 5·4 years. Patients sometimes experienced long stretches of disease quiescence followed by reactivation; 31% of patients reported active disease after 10 years. All but one patient had aesthetic sequelae, and 38% had functional limitations. The effectiveness of methotrexate and systemic corticosteroids was apparent in the short term. CONCLUSIONS: LM needs prolonged monitoring as the disease can have very long periods of quiescence followed by reactivation. The combination of methotrexate and systemic corticosteroids was effective in the early stages of the disease but did not seem to prevent long-standing active disease or relapse in the long term.

Clouston hidrotic ectodermal dysplasia (HED): genetic homogeneity, presence of a founder effect in the French Canadian population and fine genetic mapping.

HED is an autosomal dominant skin disorder that is particularly common in the French Canadian population of south-west Quebec. We previously mapped the HED gene to the pericentromeric region of chromosome 13q using linkage analysis in eight French Canadian families. In this study, we extend our genetic analysis to include a multiethnic group of 29 families with 10 polymorphic markers spanning 5.1 cM in the candidate region. Two-point linkage analysis strongly suggests absence of genetic heterogeneity in HED in four families of French, Spanish, African and Malaysian origins. Multipoint linkage analysis in all 29 families generated a peak lod score of 53.5 at D13S1835 with a 1 lod unit support interval spanning 1.8 cM. Recombination mapping placed the HED gene in a 2.4 cM region flanked by D13S1828 proximally and D13S1830 distally. We next show evidence for a strong founder effect in families of French Canadian origin thereby representing the first example of a founder disease in the south-west part of the province of Quebec. Significant association was found between HED in these families and all markers analysed (Fisher's exact test, P < 0.001). Complete allelic association was detected at D13S1828, D13S1827, D13S1835, D13S141 and D13S175 (P(excess) = 1) spanning 1.3 cM. A major haplotype including all 10 associated alleles was present on 65% of affected chromosomes. This haplotype most likely represents the founder haplotype that introduced the HED mutation into the French Canadian population. Luria-Delbrück equations and multipoint likelihood linkage disequilibrium analysis positioned the gene at the D13S1828 locus (likely range estimate: 1.75 cM) and 0.58 cM telomeric to this marker (support interval: 3.27 cM) respectively.

Soft-tissue venous malformations in pediatric and young adult patients: diagnosis with Doppler US.

PURPOSE: To describe the diagnostic features, appearance, and vascularization pattern of venous malformations (VMs) at Doppler ultrasonography (US). MATERIALS AND METHODS: Between February 1991 and May 1997, 51 soft-tissue VMs were studied with Doppler US in patients between 1 day and 21 years of age (mean age, 9 years). These VMs were located in the maxillofacial region (n = 19), trunk (n = 5), and upper (n = 10) and lower (n = 17) extremities. Twenty-three VMs had venographic confirmation, seven had only histologic confirmation, and 21 had both venographic and histologic confirmation. US was performed with 7.5- or 7-10-MHz linear transducers, a low pulse repetition frequency (mean, 1,680 Hz), and the lowest wall filter (25-50 Hz). RESULTS: At gray-scale US, VMs appeared as hypoechoic, heterogeneous lesions in 82% of cases. All lesions displayed compressibility. In eight lesions (16%), phleboliths were identified, thus confirming the diagnosis of VM. Analysis of vascular flow revealed monophasic, low-velocity flow in 40 VMs (78%), with an average flow velocity of 0.22 kHz. Biphasic flow was noted at the periphery of three lesions, which is indicative of a mixed capillary-venous malformation. The remaining eight lesions did not display any flow. CONCLUSION: In pediatric patients, Doppler US is a noninvasive, easily available, and rapid mode of investigation of vascular lesions and can help confirm the diagnosis of VM when it shows a characteristic flow pattern.

Unilateral laterothoracic exanthem. A clinicopathologic study of forty-eight patients.

BACKGROUND: Four years ago, we began seeing young children with an unusual, predominantly unilateral, morbilliform and eczematous, self-limited cutaneous eruption. It appeared to correspond to unilateral laterothoracic exanthem (ULE) reported from France and to an eruption described as "a new papular erythema of childhood" in the United States. OBJECTIVE: We conducted a prospective study of ULE to define its clinical evolution, pathology, and therapy. In addition, we performed epidemiologic and microbiologic investigations in an attempt to determine the cause of ULE. METHOD: We studied 48 children with ULE. In some patients, blood, urine, stool, as well as skin biopsy specimens were analyzed. RESULTS: ULE is a morbilliform, eczematous eruption that often begins close to the axilla and spreads to become bilateral, although it usually retains a unilateral predominance. Patients' mean age at onset is 24.3 months, with a female predominance (2:1) and mean duration of 5 weeks, followed by spontaneous resolution that may or may not be improved with topical corticosteroids. It is characterized by a unique eccrine lymphocytic infiltration. Although signs of infection were reported by most patients, no one infectious agent was identified. No significant epidemiologic factor was found. CONCLUSION: ULE, in young children, is a self-limited morbilliform and scarlatiniform eruption that may represent a specific skin reaction to one or more infectious agents.

Trichothiodystrophy associated with photosensitivity, gonadal failure, and striking osteosclerosis.

Three new unrelated cases of PIBIDS (Photosensitivity, Ichthyosis, Brittle, sulfur-deficient hair [trichothiodystrophy], Impaired intelligence, Decreased fertility, and Short stature) are reported. Decreased survival of skin fibroblast lines after UVB exposure was found. All three male patients had hypogonadism and primary end-organ gonadal failure. Striking osteosclerosis was present in all three patients. To the best of our knowledge the third patient is the first reported case of a black man with PIBIDS.

Eosinophilic pustular folliculitis (Ofuji disease) in childhood: a review of four cases.

Four children under 2 years of age were treated at our hospital in the last three years with a recurrent vesiculopustular eruption of the scalp, variably extending to the face and limbs. All cases followed a cyclical pattern. Three of the children had a moderate response to topical steroids, and one went into remission after a two-month course of erythromycin. Ethnic origin may be an important predisposing factor. Histologically, all patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Peripheral white blood cell count showing leukocytosis with eosinophilia was observed in those cases measured, but no consistent immunologic abnormalities could be identified.

Intralesional cyclosporine in the treatment of psoriasis. A clinical, immunologic, and pharmacokinetic study.

In a double-blind, vehicle-controlled study, all of six psoriatic plaques treated with intralesional cyclosporine administered three times weekly for 4 weeks showed complete clearing or incomplete but significant clearing in comparison with vehicle-treated plaques (p less than 0.01). Epidermal thickness decreased from 0.42 +/- 0.07 to 0.27 +/- 0.08 mm at 4 weeks (p less than 0.03). Biopsy specimens obtained on day 5, before any clinical improvement, revealed a significant reduction of epidermal DR+CD1- antigen-presenting cells, epidermal and dermal monocytes, and keratinocyte intercellular adhesion molecule-1 expression. By day 5 the stratum corneum reverted to normal in the plaques receiving cyclosporine. Pain at the injection site was the major side effect. Steady-state blood cyclosporine levels ranged from 20 to 30 ng/ml during the first 12 hours after injection and became undetectable at 48 hours. These data suggest that cyclosporine improves the skin of patients with psoriasis by a local mechanism of action.

Mucocutaneous complications of intraarterial 5-bromodeoxyuridine and radiation.

5-Bromodeoxyuridine (BUDR), a halopyrimidine thymidine analogue, is incorporated into the DNA of dividing cells and causes photoradiosensitization. Twenty-five patients with malignant astrocytomas were treated with continuous intracarotid BUDR radiosensitization and radiotherapy for 8 1/2 weeks. Unique dose-limiting mucocutaneous complications were encountered. Ipsilateral facial dermatitis with epilation of eyebrows and eyelashes, ocular irritation, and bilateral nail dystrophy developed in all patients. Less common reactions included oral ulceration in six patients, body exanthem on the trunk in five, and atypical erythema multiforme major in one.

Epidermolysis bullosa acquisita in childhood. Differentiation from hereditary epidermolysis bullosa.

Epidermolysis bullosa acquisita (EBA), rarely reported in childhood, is described in a 10-year-old black girl. The age of onset during infancy and the clinical appearance mimicked hereditary dystrophic epidermolysis bullosa. Epidermolysis bullosa acquisita was diagnosed by direct immunofluorescence of perilesional skin, indirect immunofluorescence on normal epithelium and saline-split skin, direct immunoelectron microscopy, and immunoblotting of the patient's serum sample against partially purified EBA antigen/carboxyl domain of type VII collagen. Differentiation of mechanobullous disease in children is critical in that significant clinical benefit may be achieved in EBA with prednisone and/or dapsone therapy. A search for associated immunologic abnormalities and HLA-DR typing may help our understanding of EBA.