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

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
 ...
PMID:New insight into the causes of immunodeficiency disorders. 638 1

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.
 ...
PMID:Diaper dermatitis. How to treat and prevent. 750 82

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.
 ...
PMID:Case report: Diaper dermatitis presenting as pustules. 2536 47

Perianal lesions in children are common reasons for dermatology clinic visits and a well-defined approach to diagnosis and management is helpful to the practicing clinician. In this article, we review and update various etiologies of perianal lesions in the pediatric population, including infectious, papulosquamous, vascular, and neoplastic. We provide a standard initial approach to diagnosis and updates on current management. Infectious etiologies of perianal lesions discussed in this article include fungal, bacterial, parasitic, and viral. Perianal papulosquamous lesions often encountered in children, and discussed in this article, include acrodermatitis enteropathica, psoriasis, contact dermatitis, and many others. We also discuss the diagnosis and management of other entities including infantile hemangiomas, Langerhans cell histiocytosis, and fibrous hamartoma of infancy.
 ...
PMID:Perianal Lesions in Children: An Updated Review. 2828 85