Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prostaglandin E1 is commonly used in the management of cyanotic congenital heart disease. While cutaneous flushing and peripheral edema are well recognized side effects of prostaglandin E1 therapy, other cutaneous effects have not been described in the dermatologic literature. We report a neonate with transposition of the great vessels who developed urticaria during treatment with prostaglandin E1.
Pediatr Dermatol
PMID:Neonatal urticaria due to prostaglandin E1. 1072 Sep 90

Pachydermoperiostosis (PDP), a rare genodermatosis, occurred in a 38-year-old Indian male. He presented with progressive thickening of the skin on the face and scalp of 15 years duration. Widening of his wrists and ankles and broadening of the fingers and toes had also developed since then. He was born of a consanguineous marriage and had no family history of a similar disorder. He had the typical findings of complete form of PDP including cutis verticis gyrata, coarse facial features, clubbing of the digits in the skin, and periostosis and cortical thickening at the distal ends of long bones of the extremities and small bones of the hands and feet. PDP has two different forms--primary and secondary. These two entities are differentiated by family history and presence or absence of a primary lesion, usually in the lungs. Clinically, in secondary PDP, the cutaneous findings (pachydermia, seborrhoea, oiliness) are less severe than primary PDP; osteoarthropathy is more severe and painful in secondary PDP, especially with congenital heart disease. The present case was suffering from primary PDP that had expressed itself in its complete form.
J Dermatol 2000 Feb
PMID:Primary pachydermoperiostosis: a case report. 1072 58

Atopic dermatitis (AD) is frequently complicated by minor bacterial superinfections. Invasive infections such as osteomyelitis have rarely been reported. We describe two children with staphylococcal septicemia during an exacerbation of their AD. Cellulitis and underlying congenital heart disease, respectively, were considered predisposing factors for the development of bacteremia. Identical strains were isolated from the skin, and there was a significant increase in antibodies against Staphylococcus aureus capsular polysaccharide in one child. Our cases demonstrate the potential severity of bacterial skin infections in AD, especially when associated with an underlying condition that increases vulnerability to bacteremia. While their true incidence in children with AD is currently unknown, it is conceivable that systemic staphylococcal infections may be more common than previously thought. Staphylococcal bacteremia has to be considered in the differential diagnosis of fever in children with severe AD. Conversely, episodes of staphylococcal bacteremia should prompt a search for underlying predisposing factors.
Pediatr Dermatol
PMID:Staphylococcal septicemia in children with atopic dermatitis. 1079 98

Kawasaki disease is one of the commonest vasculitides seen in children. It presents with prolonged fever and a polymorphic exanthem. It is a major cause of acquired heart disease in western society. Its exact cause is not known, but exposure to a superantigen has been suggested as a possible aetiological factor. Diagnosis of Kawasaki disease still relies on clinical criteria (Table 1) and investigations are done mainly to exclude other diseases and to detect early or established cardiac complications. Coronary complications can be reduced significantly by the use of intravenous immunoglobulin therapy combined with oral aspirin. The serious consequences of Kawasaki disease require a heightened awareness of this condition when dealing with childhood exanthems.
Clin Exp Dermatol 2001 Jan
PMID:Kawasaki disease: an update. 1126 Jan 68

Ellis-van Creveld syndrome (EVC) or chondroectodermal dysplasia, a rare autosomal recessive disorder, is a tetrad of chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital heart disease, of which chondrodystrophy of the tubular bones is the most common feature, while central nervous system (CNS) and urinary tract anomalies are some of its rarer associations. This report describes EVC syndrome in two sisters of Indian origin, ages 8 and 6 years, the products of nonrelated, unaffected parents. The patients had chondrodysplasia of tubular bones resulting in disproportionate dwarfism, polydactyly, severely dystrophic nails, partially absent teeth, and short and bound-down upper lips with multiple frenulae. Other features noted in the girls were syndactyly and mild mitral regurgitation. All four of the classic features of EVC syndrome were present in patient 1 and three in patient 2. Additional findings were ichthyosis and plantar keratoderma in the former and absent clavicles in the latter, which have not been reported previously. The importance of prenatal diagnosis of EVC is stressed and a multidisciplinary approach for the management of these patients is highlighted.
Pediatr Dermatol
PMID:Ellis-van Creveld Syndrome: a report of two cases. 1184 33

A 74-year-old woman was referred to our department in December 1999 for a pyoderma gangrenosum (PG) arising at the edges of chronic leg ulcer. The history was positive for benign monoclonal gammopathy, ischemic hypertensive cardiopathy, polyarthrosis and venous lower leg deficiency. Monoclonal gammopathy of IgA Kappa type was diagnosed 10 years before with a continued benign nature. In 1990 a post traumatic PG of the left leg was diagnosed and a therapy with Cyclosporine A was started with healing of the lesion. In June 1999, 6 months before the hospitalization, a typical venous ulcer of the right leg appeared and was treated with bed-rest, compression bandaging and topical desloughing therapy. In the last month, after a minor surgical debridement of the wound, the lesion developed pustules evolving into a painful, necrotic ulcer with ragged, purple-red, undetermined borders (Fig. 1). A relapse of PG was suspected. Histological examination was consistent with pyoderma gangrenosum and showed massive neutrophilic infiltration, hemorrhage and necrosis of the overlying epidermis. Wound culture was negative. Other laboratory examinations only showed IgA = 8.15 g/L. Investigation of other underlying medical conditions were normal or negative. The venous leg ulcer gradually healed with antiseptic and compression-bandage therapy. After a 4-month course of topical steroid therapy with good results, the PG recurred also involving the proximal area of the leg. Methylprednisolone 50 mg/day was started. Healing began 10 days later and 2 months later the wound healed and epithelialized. Steroid was reduce to 5 mg daily for 4 months. No recurrence was seen when the drug was stopped.
Int J Dermatol 2002 Mar
PMID:Pathergic pyoderma gangrenosum in a venous ulcer. 1201 Mar 42

Cardio-facio-cutaneous syndrome is a genetic disorder with a characteristic facies, abnormal skin and hair, mental retardation and congenital heart disease. It may be confused with Noonan's syndrome, which has a familial pattern and does not present hyperkeratotic skin lesions and abnormal hair, and there are few cases reported in the literature. We describe the first case of typical cardio-facio-cutaneous syndrome observed in Brazil.
Int J Dermatol 2002 Dec
PMID:Cardio-facio-cutaneous syndrome: a case report. 1249 91

Psoriasis is associated with significant psychosocial morbidity and a decrease in health-related quality of life. It is important to view psoriasis as a serious disease and resist the tendency to underestimate its impact on overall patient well-being. The disability experienced by psoriasis sufferers is comparable to that of patients with other chronic illnesses such as heart disease, diabetes, cancer, and depression. Aggressive intervention is warranted in order to improve patient quality of life and decrease the potential for psychosocial sequelae. Health-related quality of life measures are becoming a necessary adjunct to traditional clinical assessments in the evaluation and treatment of psoriasis patients by the individual clinician. They also provide valuable information to government agencies and third party payers in the determination of resource allocation and reimbursement.
J Am Acad Dermatol 2003 Aug
PMID:Quality of life issues in psoriasis. 1289 27

The Alagille syndrome is one of the most common inherited disorders causing chronic liver disease during childhood. During the 1990s, 38 children with Alagille syndrome were evaluated at two pediatric centers in Buenos Aires, Argentina. Characteristic clinical, humoral, and cutaneous features were analyzed. The average age of diagnosis was 29 months old (range of between 2 months and 15 years). Cholestasis was evident in 92% of patients during the neonatal period. Family antecedents related to the syndrome were found in 18.5% of the patients. Peculiar facies developed in 85% of patients. Chronic cholestasis and pruritus were observed in all of the patients and jaundice was evident in 78%. Eighty-four percent of the patients had heart disease (pulmonary stenosis, intraauricular communication, intraventricular communication), 76% of them showed growth retardation, and vertebrae abnormalities were found in 63%. Embryotoxon appeared in 76% of patients, and renal disturbances in 21%. Eleven children (28%) had xanthomas, in the neck, elbows, palms, helixes, inguinal area, gluteus, and knees. The earliest findings appeared in the first months of life, and the latest at 5 years of age. The xanthomas located in the folds had a stony aspect. Cholesterol levels ranging from 220 to 1600 mg percentage (mg%) were demonstrated in all of the children with xanthomas. Liver transplantation was performed in seven of the patients (18.4%). Two of them died after this operation. The disappearance of xanthomas after transplantation was remarkable in all of the patients.
Pediatr Dermatol
PMID:Alagille syndrome: cutaneous manifestations in 38 children. 1566 Aug 89

Cardiovascular heart disease due to atherosclerosis is the commonest cause of mortality and morbidity in the Western world. Atherosclerosis is directly related to disorders of lipid metabolism, diabetes mellitus (DM), and insulin resistance. Disorders of thyroid function and porphyrin metabolism occur less frequently but may cause life-threatening situations if unrecognized. All these disorders are associated with characteristic dermatoses, which should permit early diagnosis and meaningful intervention.
Clin Dermatol
PMID:Life-threatening dermatoses due to metabolic and endocrine disorders. 1589 41


<< Previous 1 2 3 4 5 6 Next >>