Search PubMed⌕ Search

Biomedical subjects

W D James

Publications and source records attributed to W D James.

At least 127 records · Page 7Linked to original sources

Inherited patterned lentiginosis in blacks.

We present 10 patients with autosomal dominant transmission of a pigmentary pattern characterized by facial, lip, extremity, buttock, and palmoplantar small, discrete hyperpigmented macules. None of the patients had lesions of the oral mucosa or internal organ system abnormalities. These patients' pigmentary pattern can resemble the ones seen in other lentiginosis syndromes. Those most closely mimicked--the Peutz-Jeghers syndrome, the centrofacial lentiginosis syndrome, and Carney's syndrome--all have associated internal abnormalities. We believe the pattern of pigmentation in these 10 black patients is distinct.

Adult↗

Melanophages in inflammatory skin disease demonstrate the surface phenotype of OKM5+ antigen-presenting cells and activated macrophages.

Previous studies in our laboratory revealed that ultraviolet light induces the appearance of epidermal melanophages that have potent antigen-presenting ability, that can activate autoreactive T cells, and that exhibit the immunophenotype OKM5+, OKM1-, T6-, and DR+. Therefore we undertook immunophenotyping of melanophages in selected inflammatory mucocutaneous lesions to determine whether the OKM5+, OKM1- phenotype was limited to ultraviolet-induced inflammation or whether the occurrence of melanophages expressing this antigen-presenting cell phenotype was a more generalized phenomenon. Ten of the eleven patients with chronic inflammatory skin lesions demonstrated melanophages expressing the OKM5+, OKM1-, T6-, DR+ immunophenotype. In addition, they often expressed Mo3e, a marker of activated macrophages. In contrast, melanophages of a patient with post-inflammatory hyperpigmentation, although DR+, failed to express OKM5. These results are taken to support an active role for OKM5+, OKM1-, T6-, DR+ melanophages in inflammatory skin disease because these cells demonstrate the phenotype of activated inflammation-producing monocytes and potent antigen-presenting cells.

Antibodies, Monoclonal↗

Acute pseudomonas infection of the external ear (malignant external otitis).

Pseudomonas aeruginosa commonly causes low-grade infections of the external auditory canal. If these infections are inadequately treated, they can progress into a severe form of external otitis called malignant external otitis (MEO). MEO usually occurs in elderly diabetic patients and demands immediate diagnosis and medical therapy in order to prevent systemic invasion, neurologic sequelae, and even death. We present two cases of acute pseudomonas infections of the ear, neither of which progressed to MEO due to early implementation of antibiotic therapy.

Acute Disease↗

Microbial ecology of the skin.

Humans exist in an environment replete with microorganisms, yet only a few of these microorganisms become residents on the skin surface. These resident flora and the skin constitute a complex ecosystem in which organisms adapt to changes in the microenvironment and to coactions among microorganisms. The skin possesses an assortment of protective mechanisms to limit colonization, and the survival of organisms on the surface lies in part in the ability of the organisms to resist these mechanisms. Microbial colonization on the skin adds to the skin's defense against potentially pathogenic organisms. Although microbes normally live in synergy with their hosts, at times colonization can lead to clinical infection. Common infections consist of superficial infections of the stratum corneum or appendages, which can respond dramatically to therapy but commonly relapse. In rare circumstances these infections can be severe, particularly in immunocompromised patients or hospitalized patients with indwelling foreign devices.

Actinomycetales↗

Acquired dyskeratotic leukoplakia.

A 36-year-old woman developed progressive dyskeratotic leukoplakia of the palate, lips, attached gingiva, and labial surfaces of the genitalia. On microscopic examination, clusters of dyskeratotic cells were found throughout the epithelium, except in the basal cell layer. The clinical and histologic differential diagnosis included candidiasis, oral hairy leukoplakia, lichen planus, condyloma acuminatum, Darier's disease, hereditary benign intraepithelial dyskeratosis, and white sponge nevus. Her clinical and histologic findings do not permit classification into any of these well-recognized entities.

Adult↗

Oral hairy leukoplakia. A distinctive marker of human T-cell lymphotropic virus type III (HTLV-III) infection.

Oral hairy leukoplakia (HL) is a newly described lesion occurring principally on the lateral borders of the tongue in immunosuppressed homosexual men infected with human T-cell lymphotropic virus type III (HTLV-III). Clinically, HL appears as a slightly raised, poorly demarcated lesion with a corrugated or "hairy" surface. Histologically, the lesion is characterized by keratin projections on the surface (which often resemble hairs), parakeratosis, and acanthosis. In addition, large pale-staining cells with pyknotic nuclei are seen in the upper stratum malpighii, which appear similar to the koilocytes described in uterine condylomata. Candida organisms are frequently observed on the lesion surface. Little, if any, subepithelial inflammation is present. Human papillomavirus and Epstein-Barr virus have been identified in biopsy specimens from lesions of oral HL. The association of this lesion in patients with HTLV-III infection has been established. We saw a patient with HTLV-III infection and HL, in whom the immunochemical and ultrastructural findings revealed the presence of a mixed viral infection. Because oral HL may be of diagnostic value as an early indicator of HTLV-III infection, awareness of its characteristic clinical, histologic, immunochemical, and ultrastructural features is important.

Acquired Immunodeficiency Syndrome↗

Fibrofolliculomas, trichodiscomas, and acrochordons: the Birt-Hogg-Dubé syndrome.

The Birt-Hogg-Dubé syndrome is a triad of cutaneous lesions including multiple fibrofolliculomas, trichodiscomas, and acrochordons. The inheritance is autosomal dominant in nature. We present a family of three with multiple firm, skin-colored papules in whom the three types of lesions are documented. In addition, we discuss the clinical differential diagnosis of multiple firm, skin-colored papules.

Adult↗

Pigmentary demarcation lines: a population survey.

An examination of 380 patients was accomplished in order to determine the frequency of occurrence of pigmentary demarcation lines in an unselected population. Both white and black patients were included in the study, and there was an equal sex distribution. Separation of data by age groups enabled us to determine that the age of onset is in early childhood in the majority of cases. Seventy-nine percent of black female adults have at least one type of pigmentary demarcation line, with types A and B being present in over 50% of the cases. Seventy-five percent of black male patients had at least one pigmentary demarcation line, with type C being most prevalent. Fifteen percent of white female patients had one pigmentary demarcation line. Finally, it was determined that seven of fifty black women (14%) had the new appearance of type B lines with pregnancy.

Adolescent↗

Inflammatory acquired oral hyperpigmentation: association with melanophages demonstrating phenotypic characteristics of antigen presenting cells and activated monocytes.

A case of a 30-year-old black woman who developed acute, extensive intraoral hyperpigmentation is reported. Its relationship to previously described entities is discussed. The condition was associated with the presence of melanophages both in the submucosa and in the oral epithelium. The melanophages displayed phenotypic markers of a population of intraepidermal melanophages previously described as potent T cell-activating, antigen presenting cells (T6- DR+ OKM5+ OKM1-) and of activated monocytes (Mo3e+), as well as other monocyte-macrophage markers (Mono 1 and My7). The distinct phenotype of these melanophages suggests that they could play an active role in the promotion of inflammatory disease.

Adult↗

Segmental neurofibromatosis.

Three cases of segmental neurofibromatosis (NF) and one case of bilateral segmental NF are described. Previous cases described as segmental NF are reviewed and evaluated in light of Riccardi's rigid definition of segmental NF. The previously reported cases are then placed in four subgroups. Segmental NF may evolve into a generalized form over time. Also, this disorder may occur in a heritable manner, Genetic counseling of affected individuals must include these facts.

Adult↗

Staphylococcus aureus infection as a complication of isotretinoin therapy.

Eighteen patients developed Staphylococcus aureus infection during or shortly after a five-month course of therapy with isotretinoin. Staphylococcus aureus was recovered both from lesions and from the anterior nares. In a prospective study, 4% of control patients treated with isotretinoin alone developed S aureus infection, while none who applied topical antibiotic to the anterior nares developed infection. Control patients had a 64% prevalence of anterior nares colonization with S aureus, compared with 18% in the topical antibiotic group.

Administration, Topical↗

Emotional eccrine sweating. A heritable disorder.

A family with hereditary emotional hyperhidrosis is described. The inheritance pattern is autosomal dominant. A simple quantitative palmar sweat test was used to objectively confirm historical data. Of two family members tested, both had a marked decrease in palmar sweat secretion during administration of diltiazem, a calcium-channel blocker. Additional studies in a large group of patients are needed to extend this observation.

Adolescent↗