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.
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.
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.
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.
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