Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors are reporting a new case of widespread scabies in a baby. They take this opportunity to emphasize on the atypical erythematous and excoriated papular rash which sometimes may be vesicular and hyper-keratotic. This widespread eruption may mimic generalised dermatitis, pustular psoriasis and even histiocytosis X. They also underline importancy of longlasting ointment with fluorinated steroid being responsible for this widespread eruption.
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PMID:[Extensive scabies in a baby (author's transl)]. 23 38

Skeleton and soft tissue findings in systemic dermatoses sometimes allow early differentiation between diseases affecting the skin only and those involving other organs too. Rarely, ossious alterations can be detected even before any cutaneous manifestations have shown up. Apart from a tabular survey, we refer in detail to the frequency as well as the diagnostic value of ossious and soft tissue findings in dermatomyositis, lupus erythematosus, systemic sclerosis, psoriasis, Reiter's disease, sarcoidosis, neurofibromatosis (Recklinghausen's disease), histiocytosis X, and pretibial myxedema. Particular attention is paid to the problems of differential diagnosis with special regard to rheumatoid arthritis.
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PMID:[Roentgenmorphologic findings in systemic dermatoses]. 329 34

Dampness, maceration, fecal enzymes, chemicals, and other irritants lead to diaper dermatitis in infants. Most cases can be cleared with frequent diaper changes, use of superabsorbent disposable diapers (which contain gelling material in their core), and a low-potency topical corticosteroid. If the eruption lasts for more than 3 days or classic erythematous satellite lesions are present, addition of an antifungal agent should help resolve the condition. Recalcitrant or clinically atypical eruptions may signify rarer disorders, such as psoriasis, Langerhans' cell histiocytosis, Leiner's disease, or acrodermatitis enteropathica. Patients with these conditions should be referred to a dermatologist, if possible, for further evaluation and treatment.
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PMID:Diaper dermatitis. How to treat and prevent. 750 82

This article discusses many of the more common diagnoses that should be considered when managing a child with nonresponsive excema. Some of these possible diagnoses are infectious and parasitic disorders, psoriasis, heritable disorders, Langerhans cell histiocytosis, and syndromes with altered immunity and eczematous skin changes. This overview of diagnostic and laboratory evaluations of the child with eczema who fails standard therapy is presented as a useful guide in evaluating these patients.
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PMID:Laboratory evaluation of the child with recalcitrant eczema. 814 75

We report on 56 children with sclerosing cholangitis (SC) seen between 1972 and 1992. The first symptoms occurred at a mean age of 3.7 years; 15 infants had neonatal cholestatic jaundice. At diagnosis, cholestatic jaundice was present in 25 children, hepatomegaly in 54, splenomegaly in 41, and ascites in 12. Serum alkaline phosphatase activity was increased in 49 patients and gamma-glutamyltransferase activity in all patients tested. Most often the histopathologic findings were extensive portal fibrosis and neoductular proliferation. Cholangiography showed abnormal intrahepatic bile ducts in all children and abnormal extrahepatic bile ducts in 35 (63%). The children were separated into three groups: (1) those with SC of neonatal onset (27%); (2) those with SC of postneonatal onset associated with another disease (55%)--histiocytosis X in 14 children, immunodeficiency syndromes in 8, chronic inflammatory bowel disease or autoimmune hepatitis in 8, and congenital psoriasis in 1; and (3) those with SC of postneonatal onset without an associated disease (18%). Biliary cirrhosis was present in all but three children after 6 months to 19.3 years of follow-up. Eleven children died of portal hypertension or liver failure, and six died of a complication related to the associated disease. Fifteen children had liver transplantation; 11 of these are alive 6 months to 6 1/2 years later without recurrence of SC. The overall estimated median survival time of children with SC was 10 years from clinical onset. These results indicate that SC should be suspected in all children with a chronic cholestatic disease and increased serum gamma-glutamyl transferase activity, especially when diseases known to be associated with SC are present. The prognosis is poor, but liver transplantation should be considered except in those with severe immunodeficiency syndromes.
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PMID:Sclerosing cholangitis in children. 828 75

Sclerosing cholangitis in childhood is a heterogeneous condition, which has different aetiologies. Sclerosing cholangitis may be inherited and diagnosed in the neonatal period (neonatal sclerosing cholangitis); it may present later with features of autoimmunity (autoimmune sclerosing cholangitis); or it may be associated with a variety of disorders, including Langerhans cell histiocytosis, immunodeficiency, psoriasis, cystic fibrosis, reticulum cell sarcoma and sickle cell anaemia. In contrast to the experience in adult patients, sclerosing cholangitis occurring as an individual disease (primary sclerosing cholangitis) is rare. The initiating events and possible pathogenic mechanisms differ in the various forms of sclerosing cholangitis and are still obscure. Treatment and prognosis depend on the type of sclerosing cholangitis present.
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PMID:Sclerosing cholangitis in the paediatric patient. 1149 76

A 25-year-old man presented with a 13-year history of an erythematous, papular eruption of his face and trunk, which was treated in the past as acne and psoriasis with isotretinoin and methotrexate, respectively. Histopathologic examination demonstrated an infiltrate of Langerhans cells, which was consistent with Langerhans cell histiocytosis. The epidemiology, clinical presentation, and treatment options of this disease are reviewed.
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PMID:Perifollicular Langerhans cell histiocytosis. 2328 96

Diaper dermatitis is the most common dermatologic disorder of infancy. Its cause can often be determined clinically based on the clinical presentation. Primary diaper dermatitis is associated with irritants and spares the deep skin folds. Secondary diaper dermatitis is most often caused by Candida yeast overgrowth and typically presents as a well-defined area of beefy red erythema covering the diaper area and including the deep folds of skin with hallmark satellite pustules. Other causes include seborrheic dermatitis, psoriasis, acrodermatitis enteropathica, allergic contact dermatitis, Langerhans cell histiocytosis, and, in the setting of a primarily pustular eruption, bacterial folliculitis. A simple potassium hydroxide preparation (KOH) can confirm the diagnosis of candida diaper dermatitis and guide proper treatment.
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PMID:Case report: Diaper dermatitis presenting as pustules. 2536 47

We present a case of a three-yr-old child with a history of multisystem Langerhans cell histiocytosis treated with systemic chemotherapy, who developed progressive liver failure and received an orthotopic split liver transplant while continuing on chemotherapy. One month following transplant, he developed acute graft-vs.-host disease of the skin and gastrointestinal tract. Peripheral blood chimerism studies post-transplant demonstrated an increasing predominance of donor lymphocytes and granulocytes. Shortly after, the patient developed vitiligo, and two yr after transplantation, the patient developed skin manifestations of psoriasis. We discuss and review the current literature, which demonstrates that chimerism following liver transplantation is rare and in our patient may be related to his profound immunosuppression around the time of liver transplant as well the development of acute graft-versus-host disease. While autoimmune disease can occur after solid organ and stem cell transplant, our patient developed skin manifestations of autoimmunity after liver transplantation, which is also rarely described.
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PMID:Bone marrow engraftment and associated dermatologic sequelae in a three-yr-old after liver transplantation. 2551 32

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


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