Staphylococcal scalded skin syndrome: An epidemiological and clinical review of 84 cases.

BACKGROUND: Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated, blistering skin disorder that mainly affects infants and children. There is limited literature regarding pediatric SSSS. The purpose of this study was to describe the epidemiology, clinical features, and management of pediatric SSSS. METHODS: Retrospective cohort study of pediatric patients with a clinical diagnosis of SSSS seen at the Hospital for Sick Children in Toronto, Ontario, Canada, from January 1994 to March 2016. RESULTS: We included 84 patients with a clinical diagnosis of SSSS; 49/84 (58%) were male. Mean age of diagnosis was 3.1 ± 2.4 years. All patients presented with erythema and exfoliation, while 64/84 (76%) presented with vesicles/ bullae. Skin tenderness was the most common symptom, present in 68/84 (81%) subjects. Staphylococcus aureus was more commonly isolated from periorificial cultures than from bullae. Mean hospitalization was 4.7 ± 2.3 days. No difference was found in admission duration between children receiving clindamycin and those that did not (3.6 ± 2.2 vs 3.9 ± 2.34 days, P = .63). Skin debridement was the only risk factor leading to more complications and prolonged hospitalization (P = .03). Severe complications were seen in 4 (5%) cases, and no fatalities were observed. CONCLUSIONS: Healthcare providers should be aware of SSSS and consider it in the differential diagnosis of infants and children with new onset erythema, exfoliation, and/or vesiculation. Suspected culprit pathogens were more often obtained from periorificial swabs; however, these isolates were not tested for exfoliative toxin to confirm causality. Antibiotic treatment should be guided by sensitivity testing. Addition of clindamycin as an anti-toxin agent had no effect on the duration of hospitalization, and this should be further investigated. Surgical debridement of the skin in patients with SSSS should be discouraged.

Parental use of sun protection for their children-does skin color matter?

BACKGROUND/OBJECTIVES: Excessive sun exposure during childhood is a risk factor for skin cancer. This study aimed to compare the frequency of ideal sun protection use between parents with lighter- and darker-skinned children and explore their attitudes and beliefs on sun safety and their choice of sun protection. METHODS: Parents of children aged 6 months to 6 years completed self-administered questionnaires about sun protection practices for their children. Parents assessed their child's Fitzpatrick phototype and were divided into lighter- (Fitzpatrick phototype I-III) and darker-skinned (Fitzpatrick phototype IV-VI) groups. Sun safety guidelines from the Canadian Dermatology Association were used to qualify ideal sun protection. RESULTS: A total of 183 parents were included. Overall, 31 parents (17%) used ideal sun protection for their children. As their children grew older, parents were less likely to use ideal sun protection (odds ratio = 0.69, 95% confidence interval = 0.53-0.90). Parents in the lighter-skinned group were more likely to use ideal sun protection for their children (odds ratio = 7.4, 95% confidence interval = 2.7-20.1), believe that sun exposure was harmful (odds ratio = 17.2, 95% confidence interval = 4.0-74.9), and perceive value in sun protection (odds ratio = 11.4, 95% confidence interval = 3.3-39.0); the darker-skinned group believed that darker skin tones provided more sun protection (odds ratio = 12.4, 95% confidence interval = 6.1-25.4). CONCLUSION: Ideal parental sun protection efforts are overall low, particularly in parents of darker-skinned children. The identified attitudes toward and beliefs about sun safety may aid in delivery of future sun protection interventions, especially in multiracial populations.

Pediatric Wells syndrome (eosinophilic cellulitis) after vaccination: A case report and review of the literature.

A 4-year-old boy presented with erythematous vesicular plaques, ulceration, edema, and pruritus on the left foot and ankle 10 days after receiving the tetanus, diphtheria, pertussis, and polio; measles, mumps, rubella, and varicella; and hepatitis A/B vaccines. Biopsy showed eosinophilic infiltrates and flame figures, suggesting Wells syndrome. Patch testing showed a 1+ reaction to neomycin and aluminum hydroxide, with a recall reaction of Wells syndrome of the feet bilaterally. We report a rare case of pediatric Wells syndrome triggered by nonthimerosal vaccine components confirmed by patch testing.

Pediatric Lip Adhesion Following Bullous Erythema Multiforme and Review of Similar Oral Complications.

BACKGROUND: Erythema multiforme (EM) is a mucocutaneous disease most often associated with preceding herpes simplex virus or Mycoplasma pneumoniae infection. It is characterized by targetoid lesions occurring on the limbs and head and neck. Mucosal involvement can be extensive. OBJECTIVE: We report a case of lip adhesion as a complication of EM in a child who required corrective surgery and review similar oral commissure complications. METHODS: We completed a review of the literature for similar cases using PubMed and Medline. CONCLUSIONS AND RELEVANCE: We present the youngest report of lip adhesion due to striking mucositis related to EM. Sequelae of erythema multiforme can be significant, in our case requiring surgery. Meticulous oral hygiene should be encouraged when mucosal lesions are identified.

A 13-Year-Old Presenting With Recurrent Angioma Serpiginosum.

BACKGROUND: Angioma serpiginosum (AS) is a rare vascular abnormality consisting of proliferation and dilation of superficial blood vessels in the skin. AS typically presents in the first 2 decades of life and remains stable with time. CASE SUMMARY: We report the case of a 13-year-old female with an acquired, recurrent erythematous lesion with serpiginous borders on her left upper arm. Over several years, the lesion reappeared then disappeared 12 to 15 times. At one point, she developed a tender red nodule within the lesion; consequently, a biopsy was taken, revealing dilated telangiectatic vessels in the dermal papillae with mild, focal extravasation of erythrocytes. CONCLUSION: This case demonstrates a lesion with suggestive clinical and histological features of AS. However, this may be the first case of recurrent AS with a symptomatic episode. This case may contribute to the expanding clinical spectrum of this interesting disease entity.

Subcutaneous Fat Necrosis of the Newborn With Initial Hypocalcemia and Familial Recurrence: A Case Report.

Subcutaneous fat necrosis of the newborn (SFNN) is a rare disorder characterised by indurated plaques or nodules with or without erythema, typically distributed over the cheeks, extremities, posterior trunk, buttocks, and thighs, and appears during the first 2 weeks of life. It is commonly associated with perinatal asphyxia and, while usually self-limited, can lead to severe hypercalcemia. There is no known familial association. The present article describes a neonate with extensive disease, counterintuitive hypocalcemia, and a family history of SFNN and reviews the existing literature on SFNN.

Rituximab Therapy for Childhood Pemphigus Vulgaris.

Pediatric pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis that poses a diagnostic and therapeutic challenge. Rituximab, an anti-CD20 monoclonal antibody, is an important medication in adult PV but has rarely been used to treat pediatric disease. We describe successful rituximab therapy in a 4-year-old, the youngest patient with PV ever reported to receive rituximab. A live attenuated vaccination was later given without incident. We also review rituximab clinical outcomes, toxicity, dosing protocols, and relapse in children with PV.

Idiopathic CD4 T-Cell Lymphocytopenia: A Case Report of a Young Boy With Recalcitrant Warts.

IMPORTANCE: Idiopathic CD4 T-cell lymphocytopenia (ICL) is an immunodeficiency disorder that presents with a decrease in CD4(+) T cells without evidence of a human immunodeficiency virus infection. It is most commonly diagnosed after a patient presents with an opportunistic infection and can also be associated with malignancies and autoimmune diseases. This case presentation and literature review highlights the common skin findings in patients with ICL, mainly recalcitrant warts, and discusses the treatment options available. OBSERVATIONS: The patient described is the youngest reported with ICL presenting with isolated cutaneous findings of recalcitrant warts and psoriasis. Many treatment options were tried for the warts, with the most significant response to acitretin. CONCLUSIONS AND RELEVANCE: This case highlights the importance of considering underlying immunodeficiency in patients with recalcitrant warts as well as developing treatment plans. Such patients require close follow-up by both dermatology and immunology to monitor for the development of other diseases related to ICL.

Impact of Cosmetic Camouflage on the Quality of Life of Children With Skin Disease and Their Families.

BACKGROUND/OBJECTIVES: Cosmetic camouflage is known to improve quality of life in adults. Few data are available regarding cosmetic camouflage in children, and thus it is not often selected as a mode of treatment. We sought to determine whether cosmetic camouflage leads to improved quality of life of pediatric patients with visible dermatoses and their parent or primary caregiver. METHODS: Patients aged 5 to 17 years with visible skin disease and their parent were assessed with the Children's Dermatology Life Quality Index (CDLQI) and the Family Dermatology Life Quality Index (FDLQI) before and after consultation regarding cosmetic camouflage. RESULTS: Twenty-two children with skin conditions were included in the study. The mean CDLQI decreased from 6.82 (SD = 1.28) to 3.05 (SD = 0.65; P = .0014), while the mean FDLQI decreased from 7.68 (SD = 1.15) to 4.68 (SD = 0.92; P = .0012). CONCLUSIONS: Our study highlighted improvement in quality of life in patients with skin disorders who were managed with cosmetic camouflage.

Camptodactyly and knuckle pads coexisting in an adolescent boy: connection or coincidence?

Camptodactyly is a condition characterized by a nontraumatic, fixed flexion contracture at the proximal interphalangeal joint, typically involving the fifth finger. Most occurrences are sporadic, but autosomal dominant transmission and syndromic associations have been described in the literature. We describe the case of an adolescent boy who presented to our clinic with a 2-year history of bilateral, nonsyndromic camptodactyly and knuckle pads.

Retrospective review of relapse after systemic cyclosporine in children with atopic dermatitis.

Cyclosporine is a systemic therapy used for control of severe atopic dermatitis (AD) in children. Although traditionally recommended at a dose of 5 mg/kg/day for 6 months, a longer duration of treatment may be necessary to bring a child with active and severe disease into remission. There are few data on the short- and long-term effectiveness of longer courses of therapy. This was a retrospective chart review of children treated with cyclosporine at a Canadian hospital-affiliated clinic between 2000 and 2013. Fifteen patients with adequate follow-up were identified. Twelve (80%) were male and the mean age at initiation of cyclosporine was 11.2 ± 3.4 years. The mean duration of cyclosporine therapy was 10.9 ± 2.7 months (range 7-15 months) at a starting dose of 2.8 ± 0.6 mg/kg/day. Of 12 patients (80%) who responded to cyclosporine, 5 patients (42%) had relapsed at a follow-up of 22.7 ± 15.0 months. The duration of therapy was longer in patients who did not relapse (17.7 ± 10.7 months) than in those who did (10.2 ± 2.7 months) (p = 0.06). Adverse events led to discontinuation in three patients (20%) and included infection-related complications in two patients and reversible renal toxicity in one. These results suggest that a longer duration of low-dose cyclosporine may help decrease the risk of relapse in patients with severe AD who are resistant to topical therapies.

14-year-old boy with painful nail changes.

Doxycycline is a classically implicated cause of phototoxic reactions. Photo-onycholysis is a rare manifestation of phototoxicity. Nail changes may typically include nail pain, subungual hemorrhages, or distal onycholysis. Self-resolution can be expected in 3 to 6 months. Patients prescribed doxycycline should be counseled about the potential for phototoxicity and the need for appropriate sun protection while taking the medication.

Development of a disease severity score for newborns with collodion membrane.

BACKGROUND: Collodion membrane in the neonate may be the initial presentation of a number of different conditions. There is a lack of data correlating the extent of clinical involvement to the underlying disease and prognosis. OBJECTIVE: We sought to identify features predictive of the final outcome and complications in a cohort of patients with collodion membrane, using a disease severity score. METHODS: This was a retrospective cohort study of newborns with collodion membrane at a tertiary care institution over a period of 31 years. We designed and applied a 0- to 15-point severity score and correlated the results with the final diagnoses and complications. Data on demographics, membrane shedding, and treatment were collected. RESULTS: We identified 29 cases. Congenital ichthyosiform erythroderma and lamellar ichthyosis were the most common final diagnoses with 7 of 29 cases (24%) each; 3 patients were given the diagnosis of a syndromic ichthyosis. The classic nonsyndromic ichthyoses had higher average score results (7.33) than the syndromic ichthyoses (2.0) and other presentations (4.0), (P = .0097). Patients with major complications had higher, but nonsignificant, average severity scores (P = .64). LIMITATIONS: The retrospective design and small number of patients with a syndromic ichthyosis are limitations. CONCLUSIONS: Prospective studies are required to validate the proposed disease severity score.

The efficacy of trimethoprim in wound healing of patients with epidermolysis bullosa: a feasibility trial.

BACKGROUND: There are no systemic therapies known to facilitate wound healing in patients with recessive dystrophic epidermolysis bullosa (RDEB). OBJECTIVES: We sought to assess the feasibility of a trial to examine the efficacy of trimethoprim (TMP) in healing chronic wounds in patients with RDEB and to examine the effect of TMP on lesion counts, quality of life, and emergence of antibiotic resistance. METHODS: We conducted a feasibility study using a prospective, randomized, double-blinded, placebo-controlled, crossover design. The study took place between October 2006 and September 2007 in the epidermolysis bullosa clinic at the Hospital for Sick Children in Toronto, Ontario, Canada. Liquid TMP or placebo was given orally or via gastrostomy tube in two divided doses for 2 months; the main outcome measure was a decrease in surface area of selected chronic wounds. RESULTS: Ten subjects with RDEB were enrolled in the study; 7 completed both study arms (4 male, 3 female). Age at enrollment was 14 ± 5.4 years. Although all patients showed improved wound healing on TMP, the crossover analysis, TMP versus placebo, approached but did not reach statistical significance (P = .08). While receiving TMP, 6 of 7 patients had more than 50% reduction in chronic wound surface area; while receiving placebo, 2 of 6 patients had more than 50% reduction in wound surface area (P = .03). Secondary outcome measures did not achieve statistical significance. LIMITATIONS: Small sample size is a limitation. CONCLUSIONS: This proof-of-concept study demonstrates the potential efficacy of TMP in improving wound healing in RDEB, and provides useful information for further prospective studies.

Kasabach-Merritt phenomenon: a single centre experience.

OBJECTIVE: Kasabach-Merritt phenomenon (KMP) can lead to life-threatening bleeding, and its optimum treatment has not been established. We review the experience of managing KMP in a single institution. METHODS: A retrospective chart review on all children with KMP treated at the Hospital for Sick Children, Toronto, over an 18 yr period was carried out. RESULTS: All 15 patients had profound thrombocytopenia and hypofibrinogenemia at presentation, half had bleeding symptoms, and three had cardiac failure. All patients received corticosteroids. Five responded to steroids alone, given for an average of 13 wk, increasing platelets to >20 x 10(9)/L at a mean of 6.2 d and fibrinogen >1 g/dL at 25.6 d. Ten patients received at least one other therapeutic modality in addition to steroids, including vincristine, interferon, anti-platelet agents and pentoxifylline. Five patients received vincristine, for a mean of 6 wk, with two patients responding. Eight patients received interferon, for a mean of 4 months, with two patients responding. Overall, the mean time to increasing platelets >20 x 10(9)/L was 56 d, to >150 x 10(9)/L was 88 d and fibrinogen >1 g/dL 49 d. Ten patients showed a partial response to embolisation, with a mean of 2.8 procedures performed. Thrombotic complications occurred in 7%. Twelve patients remain alive, with relapse in six patients, all treated successfully. One patient died, and two patients have been lost to follow-up. CONCLUSION: KMP is a rare condition, with significant morbidity and mortality. The therapeutic approach should include a multidisciplinary team and consensus on guidelines.

Scurvy: An Unexpected Nutritional Complication in an Adolescent Female With Anorexia Nervosa.

Vitamin C deficiency results in the clinical presentation of scurvy, a disease that is rare among the adolescent population. Individuals with unusual dietary habits, mental illness, or physical disability are more prone to develop scurvy. We present a case report of a previously healthy 16-year-old female presented to the hospital with a 12-month history of anorexia nervosa, restrictive subtype. She was admitted to the intensive care unit and transferred to a tertiary care pediatric eating disorder program for the treatment of extreme weight loss, cardiovascular instability, and refeeding syndrome. On examination, she had multiple tiny hyperpigmented perifollicular petechial papules on the lower abdomen, dorsum thighs, and extensor surfaces of the arms with corkscrew hairs on the abdomen. Dermatologic examination and laboratory investigations were consistent with a diagnosis of scurvy. The patient's vitamin C serum level was 21 µmol/L (.23 mg/dL; reference range 25-114 µmol/L [.28-1.28 mg/dL]). She was treated with ascorbic acid orally. Objective cutaneous findings improved within 4 weeks of supplementation, and after 6 weeks, repeat levels of vitamin C levels were 102 µmol/L (1.15 mg/dL). To the best of our knowledge, this is the first case of an adolescent female with anorexia nervosa and cutaneous manifestations of scurvy that resolved after the oral administration of vitamin C.