ABSTRACT
Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder usually showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. However, in some reported cases, Birbeck granules could not be demonstrated and only cytoplasmic dense bodies were seen. If the lesion is more advanced, Birbeck granules are transformed to lysosomes, i.e., 'unique phagosomes', in which they are degraded. A 2-month-old Korean girl presented with congenital, numerous red-brown pigmented papules on the left side of trunk and upper extremity without systemic symptoms. A biopsy specimen demonstrated papillary dermis containing epidermotropic infiltrates of histiocytes with abundant eosinophilic cytoplasm. Some had kidney-shaped nuclei and PAS-positive cytoplasmic inclusions. Immunohistochemically, infiltrating cells expressed S-100 protein and ultrastructurally, no Birbeck granules but many dense laminated bodies and unique phagosomes were found. It was ten months since the skin lesions developed that they have started resolving.
Subject(s)
Female , Humans , Infant , Biopsy , Cytoplasm , Dermis , Diagnosis , Eosinophils , Histiocytes , Inclusion Bodies , Lysosomes , Microscopy, Electron , Phagosomes , S100 Proteins , Skin , Upper ExtremityABSTRACT
BACKGROUND: Childhood atopic dermatitis(AD) has a profound effect on the quality of life, disturbing family and social relationships. Stressful familial condition from the child's AD may aggravate AD itself but no information concerning the effect of childhood AD on family function is available in Korea. OBJECTIVES: The aim of this study was to show how AD effects families, to identify the areas of life most affected and to evaluate the relationship between the effect and severity of AD. METHOD: The 10-questionnaire item(Dermatitis Family Impact questionnaire[DFI]) with assessment of severity of AD was studied in AD patients and compared with control subjects. RESULTS: The average of the total score of the DFI questionnaire in the AD group was significantly higher than that of the control group (P<0.05) and was linearly related with AD severity, but not as highly (R2=0.161, P=0.003). The highest scoring question in the 10 questions of the DFI questionnaire was that of causing physical tiredness or mental exhaustion, and the lowest, that of time spent on shopping for the family. CONCLUSION: There is an obvious need to identify and to reduce the suffering of families of children with AD. The DFI may potentially be of value to help guide appropriate management of AD and as an added parameter in clinical trials concerning AD management.
Subject(s)
Child , Humans , Dermatitis, Atopic , Korea , Quality of Life , Surveys and QuestionnairesABSTRACT
We experienced three cases of arteriovenous hemangioma in middle-aged patients. Each of them an asymptomatic small red to purple papule on the face and arm, which showed typical microscopic features of proliferating variously sized vessels in the dermis. An interesting finding was the increased number of mast cells identified in the stroma and perivascular channels, which may be associated with angiogenesis in inflammatory and neoplastic processes. Although arteriovenous hemangioma has been reported infrequently, its true incidence is considered to be higher than expected. Thus biopsy should be performed for exact diagnosis of small vascular lesions.
