Eosinophilic Granulomatosis with Polyangiitis in a 4-Year-Old Child: Is Montelukast and/or Clarithromycin a Trigger?

The aim of the presentation of this case is to discuss whether there is an association with eosinophilic granulomatosis with polyangiitis (EGPA) and the use of montelukast, and clarithromycin and to discuss a successful treatment course. A 4-year-old girl with a preceding history of asthma attacks and increased eosinophil counts was admitted. She had been using clarithromycin for five days and montelucast for a month. She was eventually diagnosed with EGPA with detailed examination. Clinicians should remember EGPA in children with asthma and hypereosinophilia. Patients receiving leukotriene receptor antagonists and/or macrolides should be monitored for developing a multisystem disease. Treatment with immunosuppressive agents may be required to ensure a good prognosis.

Pediatric cutaneous mastocytosis and c-KIT mutation screening.

Background: Cutaneous mastocytosis (CM) is a heterogeneous disease that commonly presents with skin lesions in childhood. Objective: In this study, we aimed to evaluate the clinical and laboratory test results of our patients with CM to ascertain prognostic factors by using patients' long-term follow-up results and to determine c-KIT (receptor tyrosine kinase) mutation from peripheral blood samples, which might be responsible for the etiopathogenesis of pediatric mastocytosis. Methods: The clinical observation data of 32 children who had been diagnosed with CM were retrospectively researched. Exon 8, 9, 11, 13, and 17 c-KIT gene locations were analyzed from DNA material that was obtained from peripheral blood samples of all the patients by using polymerase chain reaction analysis and automatic DNA sequencing. Results: The tryptase level was higher in patients with familial cases and in cases of patients who had gastrointestinal mediator releasing symptoms (p = 0.017, p = 0.038, respectively). The use of clarithromycin and the use of vitamin D were determined as triggers for mediator release. Hypogammaglobulinemia was found in six patients (18.8%). Indoor tobacco exposure was seen to be higher in patients not in remission than in patients in remission (59.1% and 20%, respectively) (p = 0.040). Allergic diseases were observed in 80% of patients in complete remission and 22.7% of patients not in remission (p = 0.002). Concomitant allergic diseases were found to be good prognosis markers among pediatric patients with CM. No c-KIT mutation was discovered in any of the patients. Conclusion: In this study, tobacco exposure would seem to be a barrier for remission, and concomitant allergic diseases were seen to be a good prognosis marker. Evaluation of peripheral c-KIT mutation had no diagnostic contribution among pediatric patients with CM in contrast to adults.

Dermatologic Manifestations of Colchicine Intoxication.

Colchicine is an anti-inflammatory drug that has a narrow therapeutic index. Poisoning typically shows 3 phases with systemic symptoms. Gastrointestinal symptoms dominate in the first phase. Dermatologic manifestations usually appear, with skin eruptions in the second phase where multiorgan failure occurs and alopecia in the third phase where organ derangements resolve. Alopecia is a cardinal feature of the third phase, but there is no specifically defined eruption for toxication. Here, we report a case of colchicine intoxication in a 16-year-old girl with maculopapular/purpuric rash and alopecia.

Comparative Clinical Outcomes of Pediatric Patients Presenting With Eyelid Nodules of Idiopathic Facial Aseptic Granuloma, Hordeola, and Chalazia.

PURPOSE: To present the clinical features of the eyelid nodules observed in a newly determined dermatologic disorder, idiopathic facial aseptic granuloma (IFAG), and to compare them with children having acute hordeolum and chalazion. METHODS: Duration of the lesion, localization, presence of coexisting facial nodules, management strategies, and response time to topical/oral antibiotics were retrospectively reviewed in 50 children with IFAG, acute hordeolum, or chalazion. RESULTS: Fourteen children with one or more IFAG nodules on their eyelids, 28 children with one or more acute hordeolum, and 8 children with one or more chalazion were examined. Children with IFAG on their eyelids and face presented earlier than children with acute hordeolum (P = .006). The duration of this lesion was similar among patients with IFAG on their eyelids and acute hordeolum (P = .53). Duration of the lesion and treatment response time were shorter in children with IFAG on their eyelids and face (P = .004) than in those with IFAG on their eyelids (P = .013). The lesions of patients with chalazion had a longer duration compared to those with IFAG on their eyelids (P = .005), IFAG on their eyelids and face (P < .001), and acute hordeolum (P = .04). Twenty patients with acute hordeolum recovered after topical antibiotics and had a similar treatment response time to those with IFAG on their eyelids and face (P = .06) and those with IFAG on their eyelids (P = .16). CONCLUSIONS: IFAG should be considered in the differential diagnosis of painless eyelid nodules in children. Because IFAG on the eyelids has many overlapping features with hordeola/chalazia, its differentiation may be difficult in the absence of accompanying facial granulomas. Chronic subepidermal eyelid nodules resembling skin abscess should alert clinicians for IFAG. Because IFAG responds well to oral clarithromycin, unnecessary surgical interventions should be avoided in these cases. [J Pediatr Ophthalmol Strabismus. 2016;53(4):206-211.].

Ophthalmic Manifestations in Children With Congenital Hypothyroidism.

PURPOSE: To evaluate the frequencies of ophthalmic abnormalities in children with congenital hypothyroidism. METHODS: A total of 121 patients with a history of congenital hypothyroidism, aged 1 to 216 months, were included in the study. The initial ophthalmological examinations and demographic data of patients who were examined between April 2013 and April 2015 were retrospectively reviewed. Accompanying systemic abnormalities were also noted. RESULTS: Ophthalmic pathology was determined in 40.4% (49 of 121) of patients with congenital hypothyroidism, of which 79.5% (39 of 49) had significant refractive errors and 32.6% (16 of 49) had strabismus. Systemic abnormalities were observed in 74.3% (90 of 121) of the children, among which 47.1% (57 of 121) were congenital heart defects and 38.0% (46 of 121) were neurologic abnormalities. A high prevalence of Down syndrome (17.3%; 21 of 121) and atopic dermatitis (8.2%; 10 of 121) was detected among patients with congenital hypothyroidism. CONCLUSIONS: In addition to many associated systemic abnormalities, ophthalmic pathologies may also occur in children with congenital hypothyroidism. Thus, routine ophthalmological examination is recommended for these children, starting from the time of initial diagnosis.

Serum Cytokine Profiles in Infants with Atopic Dermatitis.

Atopic dermatitis (AD) in infancy is believed to have distinct features as compared to AD in other age groups, and little is known about cytokine production in infants with AD. We aimed to measure the serum cytokine levels of infants with atopic dermatitis and evaluate the association of new anti-inflammatory cytokines with the disease. Eighty-one infant patients with AD and 52 healthy controls were involved in this study. The serum levels of major pro- and anti-inflammatory cytokines of the T-helper (Th) subtypes, as well as more recently defined interleukins (IL-27, IL-35, and IL-37), were measured using the ELISA method. The serum levels of IL-35, IL-5, and interferon (IFN)-γ were found to be significantly higher, while the levels of transforming growth factor (TGF)-ß1 and IL-13 were found to be significantly lower in patients with AD as compared to controls. There was no statistically significant correlation between serum cytokine levels and objective SCORAD index or total immunoglobulin (Ig) E levels. We did not observe prominent serum Th2 polarization in atopic infants. The immunopathogenesis of atopy onset at an early age may be more complicated than that at older ages.

A case of Langerhans cell histiocytosis mimicking child abuse.

Langerhans cell histiocytosis is a rare non-malignant disease with clinical heterogeneity. The disease may present with various clinical findings and may imitate many other conditions. In this report we describe a 34-month-old girl who presented with chronic otitis and otorrhea, skull fracture, rash, vulvar edema, erythema and erosion in labia majors which initially suggested child abuse but the patient was diagnosed with Langerhans cell histiocytosis.

Recurrent Aphthous Stomatitis in Childhood and Adolescence: A Single-Center Experience.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is a common oral ulcerative condition in children. The objective was to describe the clinical features of RAS in children with accompanying clinical and laboratory findings. METHODS: The study included 120 patients younger than 18 years of age (mean age 9.6 ± 4.3 years) with three or more oral aphthous ulcers per year between August 2008 and February 2014. Demographic characteristics of the patients, clinical features of the ulcers, and associated clinical and laboratory findings were evaluated. RESULTS: The mean number of aphthae per year was 12.8 ± 8.5 and the mean duration of the disease was 3.6 ± 2.9 years. Minor aphthae were the most common type (87%), papulopustules were the most common accompanying cutaneous lesions (13.3%), and family history of RAS was the most common associated factor (35.8%). Genital scarring (p = 0.04) and pathergy (p = 0.01) were significantly more common in the adolescent group. Pathergy was significantly related to genital scarring (p = 0.04) and Behçet's disease (p = 0.02). There was no association between the number of aphthae per year and the duration of the disease and hematologic and immunologic abnormalities. CONCLUSION: Our study is consistent with previous reports in terms of clinical features of aphthous ulcers, related diseases, and family history of RAS, but no associated laboratory abnormalities were noted.

Localized bullous eruptions away from infusion site due to intravenous acyclovir administration in a child.

Acyclovir is an antiviral agent against herpes virus. Its local adverse effects are common and typically consist of inflammation or phlebitis at the site of intravenous infusion. Here we present a child with bullous eruptions away from infusion site due to acyclovir administration. It is exceptionally rare with only one adult case has been reported to date.