Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience).

BACKGROUND/OBJECTIVE: Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center. METHODS: Fourty-four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Data related to epidemiologic, clinical, and histopathologic features were extracted from hospital records. RESULTS: The mean age of the affected patients was 4.6 years old (range: 0-17 years old) at the time of diagnosis. Twenty-five patients (56.8%) were male, and 19 patients were female (43.2%). Thirty-six children (81.8%) had solitary JXG, one of which was a giant congenital JXG; eight children (18.2%) had eruptive JXG. The heterozygote mutation associated with neurofibromatosis 1 gene was detected in one patient who had both eruptive JXG and numerous café-au-lait spots. Another patient with eruptive JXG was identified to have hypercholesterolemia. None of the children with eruptive JXG developed symptoms or signs of extracutaneous involvement during their clinical follow-up. CONCLUSION: Since JXG is rarely encountered, there may be a tendency toward over-treatment, given concerns for extracutaneous involvement. However, our review revealed no instances of extracutaneous involvement.

Patch Test Results of Contact Sensitization in Children Without Atopic Dermatitis: A Single Tertiary Center Experience.

BACKGROUND: Allergic contact dermatitis (ACD) has become more frequent in children. The prevalence of contact sensitization varies with respect to age, sex, and geographic localization. OBJECTIVE: The aim of the study was to investigate the experience of a tertiary health center regarding the patch test results of contact sensitization in children without atopic dermatitis. METHODS: This is a retrospective review of 89 children (30 boys and 59 girls) who were aged between 3 and 18 years and who were diagnosed with ACD between July 2013 and July 2017. Children with a known history of atopic dermatitis were excluded. All patients were tested with the TRUE (Thin-layer Rapid Use Epicutaneous) test series. RESULTS: The most frequently determined allergens by TRUE test were methylchloroisothiazolinone (n = 7 [16.3%]), disperse blue 106 (n = 5 [11.6%]), and bacitracin (n = 5 [11.6%]). Formaldehyde-related allergens produced 15 positives. CONCLUSIONS: Preservatives, such as methylchloroisothiazolinone, formaldehyde, and formaldehyde releasers, emerge as the most frequent allergens in children who undergo patch testing because of ACD. This finding might be attributed to the increase in the utilization of these chemical compounds in personal hygiene products for children.

A rare vascular lesion of newborn: cutis marmorata telangiectatica congenital.

Cutis marmorata telangiectatica congenita is a rare, benign, sporadic and cutaneous vascular disease. A newborn female baby whose mother was aged 29 years and used propylthiouracil during pregnancy was hospitalized because of varicose lesions on the skin of the lower extremity and on the back, which were present at birth. It was observed that the lesions did not disappear, although appropriate room temperature was provided. The patient was diagnosed as having cutis marmorata telangiectatica congenita and screened for additional anomalies. She had no additional anomalies, and she was discharged and monitored. At the postnatal sixth month, the cutaneous vascular lesions disappeared spontaneously. Cutis marmorata telangiectatica congenita, which is a rare condition, should be kept in mind in the differential diagnosis of physiologic cutis marmoratus, which occurs frequently in the neonatal period.

Kutis marmorata telenjiektatika konjenita; nadir görülen, selim, sporadik, deriyi tutan vasküler bir hastaliktir. Yirmi dokuz yasinda hipertroidi nedeni ile propiltiyourasil kullanan anneden dogan kiz bebek, her iki alt ekstremitede ve sirtta yerlesim gösteren renk degisikliginin eslik ettigi variköz lezyonlar nedeniyle yatirildi, lezyonlarin uygun ortam sicakligi saglanmasina ragmen düzelmedigi saptandi. Kutis marmorata telenjiektatika konjenita tanisi konan olgu; eslik edebilecek ek anomaliler açisindan tarandi; ek anomali saptanmayan olgu taburcu edilerek izleme alindi, postnatal altinci ayda kutanöz vasküler lezyonlarin kendiliginden kayboldugu görüldü. Yenidogan döneminde sik olarak karsimiza çikan fizyolojik kutis marmoratus ayirici tanisinda, ender görülen bir durum olan kutis marmorata telenjiektatika konjenita da akilda tutulmalidir.

Pediatric lichen nitidus: A single-center experience.

OBJECTIVE: Lichen nitidus (LN) is an uncommon inflammatory skin eruption. The present study aims to describe a case series of children with LN seen at a tertiary-care health center. METHODS: Retrospective study of 17 children with biopsy-proven LN between January 2007 and March 2017. Data related with epidemiologic, clinical, and histopathologic characteristics were recorded. RESULTS: The mean age for the onset of LN was 9 years (range: 5-17 years), and the mean duration of the skin lesions was 13 months (range: 1-48 months). Fifteen were boys (88.2%). Seven children had a generalized form of LN (41.1%) and of these, two children had severe pruritus (11.8%). Seven children had a history of co-morbid skin conditions (41.1%), including lichen planus in one patient (5.9%), lichen striatus in one patient (5.9%), psoriasis and longitudinal ridges in the nails in one patient (5.9%), and cutaneous features of atopic skin in four patients (23.5%). All of the reviewed patients had lymphohistiocytic infiltration in the dermis and basal vacuolar degeneration. Multinucleated giant cells were present in 11 (64.7%). CONCLUSION: This case series found LN in a generalized form as well as other concurrent dermatologic conditions in nearly half of those reported. The boy predominance was also noteworthy.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a report of six cases.

Kara A, Devrim I, Çaglar I, Bayram N, Kundak S, Apa H, Altan EV. Stevens-Johnson syndrome and toxic epidermal necrolysis: a report of six cases. Turk J Pediatr 2019; 61: 538-543. Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous adverse reactions commonly caused by exposure to drugs and can end up with significant morbidity and mortality. We reported our experience with six patients who were diagnosed with Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis with a different clinical presentation. In patients, drugs and Mycoplasma pneumoniae infection were implicated as a trigger. Intravenous Immunoglobulin treatment was given to all patients, and intensive treatment was applied for skin and mucosal lesions. The median period of stay in hospital was 13.5 days. The most common long-term complication was ocular involvement. Among six patients, corneal epithelial defects occurred in one patient. Consequently, ophthalmological evaluation should be performed both at the time of diagnosis and before hospital discharge.

A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease).

Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent dermatosis characterised by sterile pustules. It develops mainly in middle-aged or elder women, but is also rarely seen in children. The exact aetiology of the disease is unknown. In literature, cases associated with IgA gammopathy have been reported. In this article; we report a case of a five-year-old girl who was diagnosed as SPD by clinical features, histopathological characteristics, and direct immunofluorescence analysis results. IgA was high, and IgG-IgM and CD19+ B cell were low. We noticed that during IVIG treatment for immunodeficiency, dermatological symptoms were recovered rapidly. Clinical profile of SPD and its association with systemic diseases may provide early detection of immune dysfunction.

Pediatric langerhans cell histiocytosis: single center experience over a 17-year period.

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.