Scurvy masquerading as IgA vasculitis.

BACKGROUND: Vitamin C deficiency, or scurvy, is rare but poses risks for children with poor diets, limited resources, or malabsorption issues. It may also be common in children with restrictive or selective dietary habits in children with global developmental delay, autism spectrum disorder, and physical disabilities. Symptoms include fatigue, irritability, joint and muscle pain, joint swellings, edema, swollen gums, easy bruising, and delayed wound healing. Early recognition and prompt intervention are essential to prevent the progression of symptomatic vitamin C deficiency in children. CASE PRESENTATION: We present a case of a 13-year-old boy with developmental delay secondary to Lennox Gastaut syndrome referred for suspected recurrent, severe, and atypical IgA vasculitis. He presented with irritability, loss of appetite, petechial and ecchymotic lower limb lesions, unilateral gum swelling, severe arthritis, peripheral oedema, severe weight loss, anaemia, and raised inflammatory markers. Multiple investigations were performed before the diagnosis of scurvy was made. A surgical finding of friable gingival tissue with multiple loose teeth, a skin biopsy with follicular hyperkeratosis and extravasated perifollicular red blood cells, and a typical X-ray finding led to the diagnosis of scurvy. CONCLUSION: Scurvy should be given careful consideration as a differential diagnosis in patients presenting with musculoskeletal issues, mucocutaneous complaints, and constitutional symptoms such as malaise, asthenia, irritability, and loss of appetite. A focused and detailed dietary history looking for a lack of good sources of vitamin C can be an easy indicator of this differential. Imaging studies revealing the typical features can also help make the diagnosis. Pathology of the skin revealing pathognomonic features can add to the certainty of the diagnosis. In the absence of all else, the rapid response to treatment with an appropriate dose of vitamin C has a diagnostic and therapeutic role.

Balamuthia mandrillaris Granulomatous Amoebic Encephalitis: The First African Experience.

We report the first case of Balamuthia mandrillaris granulomatous amoebic encephalitis definitively acquired in Africa. Our case emphasizes initial nonspecific dermatological features, delays in confirmation of the diagnosis, difficulties accessing recommended medication, and uncertainty about optimal treatment of a disease with a frequently fatal outcome.

Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Deep forehead lipomas in children-A series and review.

Deep forehead lipomas are rare in children and may be confused with other more concerning soft tissue masses. We describe four children with deep forehead lipomas, diagnosed between 2 months and 1 year of age, three of them congenital. Notable findings included association with intracranial lipoma and seizures in one patient and the development of marked alopecia overlying the lipoma in another. While deep forehead lipomas may become less visible over time, alopecia and non-syndromic extracutaneous involvement may be important associations.

The prevalence of human T-lymphotropic virus type 1 & 2 (HTLV-1/2) in South African blood donors.

BACKGROUND AND OBJECTIVES: Donated blood is not currently screened for human T-cell lymphotropic virus (HTLV) in South Africa. Several small studies have detected HTLV-1 in South Africa, but prevalence by geographic region or population group is unavailable. MATERIALS AND METHODS: We performed a large seroprevalence study of South African blood donors during 3 months in 2013. All geographic regions except the Western Cape were included, and Black and Coloured (local term for mixed race) donors were oversampled. Identity-unlinked plasma samples were screened with the Abbott Prism HTLV-1/2 assay, and repeatedly reactive samples were tested by the Inno-LIA HTLV-1/2 Score confirmatory assay. Odds ratios were calculated with multivariable logistic regression. RESULTS: Of 46 752 donors tested, 133 (0·28%) were initially reactive, 111 (0·24%) repeatedly reactive and 57 (0·12%) confirmed positive for HTLV-1; none were HTLV-2 positive. Prevalence was 0·062% weighted to annual blood donations but highly concentrated in the Black population group (OR = 20·24 CI: 2·77-147·88); higher in females than males (OR = 1·81 CI: 1·06-3·08); and in donors aged >50 years compared to ages 16-19 (OR = 6·4 CI: 2·95-13·86). After controlling for age, sex and population group, there was no difference in prevalence between new and repeat blood donors or among geographic regions within South Africa. CONCLUSIONS: We conclude that HTLV-1 infection is widespread among the Black population of South Africa and its epidemiology is similar to other endemic areas. Because South Africa is increasing its recruitment of Black blood donors, the implications for blood screening require further consideration.

Focus on the Top Ten Diagnoses Could Reduce Pediatric Dermatology Referrals.

There is a sense that many patients seen at referral centers could be managed at a primary health care level. The objective of the current study was to examine the range of diagnoses among consultations at the Red Cross Children's Hospital in Cape Town, South Africa, to help develop a strategy for targeted education of primary health care personnel. This was a retrospective review of data for children seen at a pediatric dermatology clinic from 2005 to 2010, recorded according to International Classification of Diseases coding and compared with published data from similar clinical settings. There were 13,253 clinic visits, with 4,789 patients seen (median age 4.8 yrs, range 2 days to 18.6 yrs). The top 10 diagnoses accounted for 88.5% of consultations (59.5% atopic eczema [AE], 7.1% seborrheic dermatitis [SD], 4.2% superficial mycoses, 3.1% molluscum contagiosum, 2.8% vitiligo, 2.7% viral warts, 2.4% prurigo or scabies, 2.3% psoriasis, 2.3% hemangioma, 2.1% impetigo). Disease prevalence was somewhat different during the first year of life (AE 43.7%, SD 18.6%, hemangiomas 13.4%). Inflammatory dermatoses (76.6%) were more prevalent than infections and infestations (14.5%). The disease spectrum was similar to that in developed countries, although AE prevalence was higher in this study (followed by London 36%, Greece 35%, and Hong Kong 33%) than in 19 published studies. The top 10 diagnoses accounted for more than 70% of diagnoses in 12 studies. The retrospective nature and setting at a specialist clinic increased bias and limited generalizability. Focused education on the optimal care of common diseases, especially AE, could reduce referrals, improve access, and allow specialists at tertiary centers more time to manage complex and uncommon dermatoses.

A Review and Proposed Approach to the Neutrophilic Dermatoses of Childhood.

Neutrophilic dermatoses (NDs) are inflammatory skin conditions that are not associated with infection. The classification and clinical approach to these conditions in children is poorly described. This review classifies these conditions into five nosological subtypes: Sweet's syndrome, pyoderma gangrenosum, aseptic pustules, neutrophilic urticarial dermatoses, and Marshall's syndrome. In addition, we review the various secondary diseases that need to be excluded in the clinical management of the NDs of childhood, with a focus on the autoinflammatory conditions that the reader may not be familiar with. We propose a practical clinical approach to these disorders.

Infective dermatitis associated with HTLV-1 mimics common eczemas in children and may be a prelude to severe systemic diseases.

Infective dermatitis associated with human T-cell lymphotropic virus type 1 (HTLV-1) (IDH) is a chronic dermatitis that has been observed in a variable proportion of HTLV-1-infected children. IDH may serve as an early clinical marker for HTLV-1 infection and an indicator of increased risk for developing other HTLV-1-associated conditions. Factors that lead only some infected children to develop IDH are poorly understood. The variable clinical presentation of IDH, in particular its chronicity, the morphology and distribution of the lesions, and its clinical resemblance to other cutaneous inflammatory conditions, make it necessary to distinguish it from other common dermatoses.

Strongyloidiasis and infective dermatitis alter human T lymphotropic virus-1 clonality in vivo.

Human T-lymphotropic Virus-1 (HTLV-1) is a retrovirus that persists lifelong by driving clonal proliferation of infected T-cells. HTLV-1 causes a neuroinflammatory disease and adult T-cell leukemia/lymphoma. Strongyloidiasis, a gastrointestinal infection by the helminth Strongyloides stercoralis, and Infective Dermatitis associated with HTLV-1 (IDH), appear to be risk factors for the development of HTLV-1 related diseases. We used high-throughput sequencing to map and quantify the insertion sites of the provirus in order to monitor the clonality of the HTLV-1-infected T-cell population (i.e. the number of distinct clones and abundance of each clone). A newly developed biodiversity estimator called "DivE" was used to estimate the total number of clones in the blood. We found that the major determinant of proviral load in all subjects without leukemia/lymphoma was the total number of HTLV-1-infected clones. Nevertheless, the significantly higher proviral load in patients with strongyloidiasis or IDH was due to an increase in the mean clone abundance, not to an increase in the number of infected clones. These patients appear to be less capable of restricting clone abundance than those with HTLV-1 alone. In patients co-infected with Strongyloides there was an increased degree of oligoclonal expansion and a higher rate of turnover (i.e. appearance and disappearance) of HTLV-1-infected clones. In Strongyloides co-infected patients and those with IDH, proliferation of the most abundant HTLV-1⁺ T-cell clones is independent of the genomic environment of the provirus, in sharp contrast to patients with HTLV-1 infection alone. This implies that new selection forces are driving oligoclonal proliferation in Strongyloides co-infection and IDH. We conclude that strongyloidiasis and IDH increase the risk of development of HTLV-1-associated diseases by increasing the rate of infection of new clones and the abundance of existing HTLV-1⁺ clones.

The prevalence of human T-cell lymphotropic virus type 1 in the general population is unknown.

Human T-cell lymphotropic virus type 1 prevalence estimates are usually based on serological screening of blood donors, pregnant women, and other selected population groups. Previously, data on the global epidemiology of human T-cell lymphotropic virus type 1 infection have been summarized unsystematically and without a focus on general populations. To assess the implications of the virus for healthcare systems it is essential to know its past and present prevalence. The widely cited estimate that 10-20 million people are infected with human T-cell lymphotropic virus type 1 worldwide was calculated from data that are now 25 years old. This estimate may therefore no longer reflect the global epidemiology. The objective of this study was to collate published data that are truly representative of the general population through a systematic review of the literature. Fifty-nine relevant studies were identified and the 17 that met the inclusion criteria were all cross-sectional designs; none reported incidence. The prevalence of human T-cell lymphotropic virus type 1 was highest in the two studies of Japanese islands (36.4%; 95% CI: 29.9-42.8) and lowest in studies from Mongolia, Malaysia and India. In Haiti the prevalence was 3.8% (95% CI: 1.78-5.86); in Africa between 6.6% (95% CI: 4.0-9.9) and 8.5% (95% CI: 6.99-10.10) in Gabon, and 1.05% (95% CI: 0.63-1.47) in Guinea. Only three studies were from West Africa and none were from the South; the only study from India was from the north of the country. We conclude that there is a paucity of general population data from countries in which human T-cell lymphotropic virus type 1 is endemic, and that new studies are required to reevaluate the global burden of infection.

Tracing the origin of Brazilian HTLV-1 as determined by analysis of host and viral genes.

We compared the genetic diversity of the Brazilian human T-cell lymphotropic virus type 1 isolates with those found in KwaZulu-Natal (KZN), South Africa, and with the genetic background of the hosts. The seroprevalence rate in KZN was 1.7%. All sequences belonged to the A subgroup. The presence of South African sequences in two different clusters from Brazil, and the finding of the beta-globin haplotype in infected hosts are consistent with the transmission of this virus from southern Africa to Brazil.