Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epidermolysis bullosa dystrophica (EBD) is a rare inherited skin disease generally presenting in newborns. It is characterized by noninflammatory bullous lesions which can involve the mucous membranes of the oral cavity and oropharynx. If death occurs, it is usually the result of septicemia or fluid and electrolyte imbalance. Although mucous membrane involvement may be extensive and despite the reported evidence of tracheal and indirect evidence of laryngeal involvement, airway obstruction has not been implicated as a possible contributor to mortality in these patients. Since the presence of EBD in the larynx of a newborn might be expected to produce upper airway obstruction, however, and because of the absence of reports of laryngeal EBD, a case is presented of EBD-induced airway obstruction accompanied by photographic evidence of laryngeal and tracheal involvement with EBD. A three-week-old boy with biopsy-proven EBD present at birth was admitted to UCLA Medical Center with increasing stridor. The patient's extremities, diaper area, and numerous pressure-bearing sites on the back and elbows demonstrated erythematous denuded skin with occasional bullae up to 2 cm in size. The mucous membranes of the mouth and oropharynx showed similar denuded lesions. A tracheotomy was performed followed by a direct microlaryngoscopy and bronchoscopy revealing EBD on the supraglottic structures, vocal cords, and trachea. Otolaryngologists who are involved in the care of newborns should be aware of the possible, nearly fatal laryngeal involvement that can accompany this disease.
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PMID:Epidermolysis bullosa dystrophica of the larynx and trachea. Acute airway obstruction. 743 45

Researchers analyzed data on 52 HIV-positive patients with Kaposi's sarcoma (KS) aged 23-67 (74% Black, 26% White; male/female ratio = 2.8:1) referred to the Johannesburg General Hospital in South Africa during 1980-1990 to examine the hospital's experience with these patients. 23 patients had a fever and/or at least 10% weight loss. 34% had prior or coexistent opportunistic infection, particularly Pneumocystis carinii pneumonia, fungal disease, or tuberculosis. Possible risk factors among 21 patients were homosexual intercourse, history of sexually transmitted disease, and drug abuse. Almost all patients had skin disease, either localized or disseminated. Other KS sites included the oral cavity, regional lymph nodes, and large bowel. 90% of 20 patients treated with radiation responded to treatment. Response rates for radiation treatment among the 20 patients were 80% for symptomatic relief, 45% for complete remission, 45% for partial remission, and 10% for tumor progression. The recurrence-free period among irradiated patients was five months. Five patients developed radiation-induced mucositis of the oropharyngeal region. None of the 32 patients treated with chemotherapy and not radiation experienced complete remission. Chemotherapy induced partial remission in 38% and tumor progression in 62% of patients. 9% of chemotherapy-treated patients experienced symptomatic relief. Deteriorating performance status and/or debilitating side effects (severe mucositis and neutropenic sepsis) necessitated cessation of chemotherapy or dose modification. The clinical course of AIDS-related KS in this population paralleled that in Western countries. Based on these findings, the authors recommend local radiation therapy to treat AIDS-related KS or a watch-and-wait policy for asymptomatic, minimal disease in patients with an intact immune status.
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PMID:Epidemic AIDS-related Kaposi's sarcoma in southern Africa: experience at the Johannesburg General Hospital (1980-1990). 757 Aug 33

Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis occurring most frequently during induction chemotherapy for a variety of malignancies. We report a case of NEH in a 41-year-old woman with acute myeloblastic leukemia under daunorubicin, cytarabine and etoposide chemotherapy. She developed red, tender and painful nodules on a shoulder. The lesions resolved spontaneously. Histological examination is mandatory as the clinical presentation of this dermatosis is highly polymorphic. Leukemia cutis, sepsis, deep fungal infection and Sweet's syndrome must be excluded as these implicate different therapies.
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PMID:Neutrophilic eccrine hidradenitis. A case report. 806 73

Crusted scabies (Norwegian scabies) is uncommon in clinical practice and may present as papulosquamous dermatosis. A case of crusted scabies in systemic lupus erythematosus (SLE) is described. The skin lesions simulated those of subacute cutaneous lupus erythematosus of the papulosquamous type, and became the source of epidemic scabies in the hospital. The colonization with Staphylococcus aureus within crusted lesions may have contributed to the patient's sepsis. This case illustrates the pitfalls in recognition of crusted scabies and the importance of rapid diagnosis.
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PMID:Crusted scabies in systematic lupus erythematosus: a case report. 813 60

The diagnosis of a pustular dermatosis occurring during the first months of life is usually based on clinical findings. However, some cases may require simple investigations including microscopic examination of pustular content, cultures, and skin biopsies. The main benign transient neonatal types of pustulosis include erythema toxicum neonatorum, infantile acropustulosis, transient neonatal pustular melanosis, and neonatal acne. The most common causes of infectious pustular skin lesions include bacterial infections, which may be initially localized (Staphylococcus aureus) or septicemic (with Listeria monocytogenes as the leading causitive agent); viral infections (herpes simplex, varicella-zoster, and cytomegalovirus infections); fungal infections (candidiasis); or parasitic disorders (scabies). The main objective of this article is to propose a systematic approach to pustular eruptions in the neonate. The need for investigating every neonate with pustules for an infectious disease is emphasized. The Tzanck smear, the Gram's stain, and a potassium hydroxide preparation are the most important quick diagnostic tests. The Tzanck smear is a very easy, rapid, and sensitive test for detection of a herpetic infection (multinucleated giant cells) as well as noninfectious pustular eruptions (eosinophils, neutrophils). Therefore the Tzanck smear should be the first test performed. Moreover, a Gram's stain and potassium hydroxide preparation should be performed in cases of neonatal pustular disorders to detect bacterial and fungal infections. The goal of this diagnostic approach is to spare a healthy neonate with a benign transient condition an invasive evaluation for sepsis, potentially harmful antibiotic therapy, and prolonged hospitalization, with its own inherent morbidity.
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PMID:Diagnosis and treatment of pustular disorders in the neonate. 914 1

From 1984 through 1992, staff at The Marine Mammal Center (TMMC, Sausalito, California, USA) examined 207 northern elephant seals (Mirounga angustirostris) with a condition of unknown etiology called northern elephant seal skin disease (NESSD). The skin lesions were characterized by patchy to extensive alopecia and hyperpigmentation, punctate or coalescing epidermal ulceration, and occasionally, massive skin necrosis. Microscopic lesions included ulcerative dermatitis with hyperkeratosis, squamous metaplasia and atrophy of sebaceous glands. All diseased seals were less than 2 years of age and suffered from emaciation, depression, and dehydration. Mortality from septicemia increased significantly with severity of skin ulceration. Compared to 14 apparently unaffected seals, diseased seals had depressed levels of circulating thyroxine, triiodothyronine, retinol, serum iron, albumin, calcium, and cholesterol. Levels of alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, gamma glutamyl transpeptidase, blood urea nitrogen, and uric acid were elevated. Morphometrically, diseased animals were approximately 15% smaller than normal seals of the same sage. Serum and blubber concentrations of 36 polychlorinated biphenyl congeners (sigma PCB) and dichloro-diphenyl-dichloroethylene (p,p'-DDE) were negatively correlated with body mass. Mean concentrations of sigma PCB and p,p'-DDE in serum in diseased seals were elevated as compared to apparently normal seals. Etiology of this syndrome remains unknown, but the possibility of PCB toxicosis cannot be ruled out.
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PMID:Clinical and pathological characterization of northern elephant seal skin disease. 924 88

Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features. We describe two patients who had recurrent episodes of pruritic follicular papular and pustular lesions on the face, extremities, and trunk. The eruptions lasted for 1 month with intermittent remissions. Laboratory tests disclosed no infectious or parasitic etiology in patient 2. In patient 1 we isolated methicillin-resistant Staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). Histologically both of our patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Their lesions responded well to topical corticosteroids.
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PMID:Eosinophilic pustular folliculitis in infancy: report of two new cases. 1033 75

Toxic epidermal necrolysis (TEN) is an exfoliative skin disorder that may involve a large body surface area and mucosal surfaces. The microscopic changes that occur with this condition are similar to those that occur with superficial dermal burns, such as dermal detachment from the underlying dermis. Complications of TEN are related to the loss of the epithelial skin barrier and include pain, fluid and electrolyte loss, and an increased risk of sepsis. The treatment of a patient with TEN is best accomplished in a burn unit, where expert treatment of these complications can be provided. Medical treatment includes the administration of immunosuppressive therapy and the discontinuation of any previous corticosteroid treatment. Surgical management includes the debridement of necrotic areas. In this article, the surgical management of 8 consecutive patients with TEN who were admitted to the intensive care burn unit at the Hospital Universitario de Getafe in Madrid, Spain, from 1996 to 1998 is described. These patients were treated with extensive early debridement of necrotic skin areas followed by wound coverage with Biobrane (Dow B. Hickam, Inc, Sugarland, Tex), a temporary semisynthetic skin substitute. Skin coverage with this material decreases pain and fluid loss, and it possibly facilitates epithelization and decreases the risk of sepsis, without adverse side effects. This semisynthetic material meets some standards of an ideal skin substitute: it is easy to use, provides several beneficial physiologic effects, and improves patients' comfort. In the 8 cases of patients with TEN that were studied, the use of Biobrane skin substitute for the coverage of massive areas of detached skin was found to be an important aspect of treatment.
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PMID:Skin coverage with Biobrane biomaterial for the treatment of patients with toxic epidermal necrolysis. 1050 29

The aim of the study was to determine for the first time the response of systemic sclerosis (SSc) patients to treatment with intravenous immunoglobulin (IVIg). Three patients with progressive and rapidly deteriorating disease (mainly affecting the skin) were planned to receive six monthly courses of high-dose IVIg (2 g/kg). All had a thorough physical examination, clinical evaluation by the modified Rodnan total skin thickness score, and measurement of the titres of PM-Scl antibodies before and after the treatment, and before and after each treatment course. Two of the three patients received six IVIg courses as planned and no adverse effects or disease progression occurred during the therapy. The third patient received three courses, after which he developed renal failure and later died of sepsis. All three patients had a large decrease in their skin score after the treatment compared to that before the treatment. No modification of PM-Scl antibody titres was noted in any patient. Intravenous immunoglobulin (IVIg) may have a role in the treatment of SSc patients with rapidly deteriorating skin disease. The specific indications, as well as the safety of this treatment, should be further researched.
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PMID:Skin score decrease in systemic sclerosis patients treated with intravenous immunoglobulin--a preliminary report. 1087 Jun 56

The differential diagnosis for pustular skin disorders is extensive. The distribution of the lesions and the age of the patient are characteristics that may provide strong clues to the etiology of cutaneous pustular eruptions. In adults, generalized pustular dermatoses include pustular psoriasis, Reiter's disease and subcorneal pustular dermatosis. Medications can cause generalized pustular eruptions, such as in the case of acute generalized exanthematous pustulosis; or more localized reactions, such as acneiform drug eruptions, which usually involve the face, chest and back. Localized pustular eruptions are seen on the hands and feet in adults with pustulosis palmaris et plantaris and acrodermatitis continua (both of which may be variants of psoriasis); on the face in patients with acne vulgaris, rosacea, and perioral dermatitis; and on the trunk and/or extremities in patients with folliculitis. A separate condition known as eosinophilic folliculitis occurs in individuals with advanced human immunodeficiency disease. Severely pruritic, sterile, eosinophilic pustules are found on the chest, proximal extremities, head and neck. Elevated serum immunoglobulin E and eosinophilia are often concurrently found. In neonates, it is especially important to make the correct diagnosis with respect to pustular skin disorders, since pustules can be a manifestation of sepsis or other serious infectious diseases. Generalized pustular eruptions in neonates include erythema toxicum neonatorum and transient neonatal pustular melanosis, both of which are non-infectious. Pustules are seen in infants with congenital cutaneous candidiasis, which may or may not involve disseminated disease. Ofuji's syndrome is an uncommon generalized pustular dermatosis of infancy with associated eosinophilia. As in adults, neonates and infants may develop acne or scabies infestations. In this article, we review the most common pustular dermatoses and offer a systematic approach to making a diagnosis. We also report the most up-to-date information on the treatment of these various cutaneous pustular conditions.
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PMID:Pustular skin disorders: diagnosis and treatment. 1211 48


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