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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.
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PMID:Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report. 1555 21

Skin findings during the initial month of life are ubiquitous. One study estimated that more than 95% of newborns have cutaneous findings, which often are distressing to parents but frequently are benign and self-limited. Among them are milia, cutis marmorata, congenital dermal melanocytosis, and the benign neonatal pustular eruptions (eg, benign cephalic pustulosis, erythema toxicum neonatorum, transient neonatal pustular melanosis). Clinicians need to recognize these benign skin conditions and differentiate them from more serious conditions, such as infectious pustular eruptions from bacterial, viral, and fungal causes, and inflammatory conditions, such as Langerhans cell histiocytosis. Notable bacterial pustular eruptions are bullous impetigo and congenital syphilis. Viral pustular dermatoses include neonatal herpes simplex virus infection and varicella zoster virus infection, which consists of congenital varicella syndrome, perinatal varicella, and infantile zoster. Fungal pustular eruptions include congenital and neonatal candidiasis. Seborrheic dermatitis is a self-limited condition that occurs with varying severity; symptomatic treatment is reserved for the more severe forms. Diaper dermatitis encompasses a broad clinical diagnosis, including allergic and irritant contact dermatitis, atopic dermatitis, infections, psoriasis, and other dermatologic conditions. Critical components of newborn skin care are immersion bathing, umbilical cord care, and use of emollients to augment skin barrier function.
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PMID:Common Skin Conditions in Children: Neonatal Skin Lesions. 2819 16