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Biomedical subjects

W D James

Publications and source records attributed to W D James.

At least 91 records · Page 5Linked to original sources
PubMed1992

Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid.

BACKGROUND: Childhood localized vulvar pemphigoid has been recently reported in four girls. A fifth child with this proposed rare variant of bullous pemphigoid is described. Moreover, findings in the various immunopathologic studies we performed establish this entity as a true morphologic variant of bullous pemphigoid. OBSERVATIONS: In situ deposits of IgG in this patient's epidermal basement membrane zone localized to the epidermal side of 1 mol/L of saline-split skin. Moreover, the patient had circulating IgG autoantibodies that bound the epidermal side of 1 mol/L of saline-split skin in indirect immunofluorescence microscopy and immunoprecipitated the 230-kd bullous pemphigoid antigen from biosynthetically radiolabeled human keratinocyte extracts. These laboratory findings are identical to those documented in patients with the generalized "classic" form of bullous pemphigoid. CONCLUSIONS: This study demonstrates that a child with clinical, histopathologic, and immunopathologic features of localized vulvar pemphigoid had circulating autoantibodies that identify a specific keratinocyte antigen, the bullous pemphigoid antigen, which may serve as a molecular marker for this disease.

Antigens↗
PubMed1992

Cutaneous histopathologic, immunohistochemical, and clinical manifestations in patients with hemophagocytic syndrome. Military Medical Consortium for Applied Retroviral Research (MMCARR).

BACKGROUND AND DESIGN: The hemophagocytic syndrome (HPS) is characterized by fever, wasting, generalized lymphadenopathy, hepatosplenomegaly, and pancytopenia, often with associated coagulopathy. The most common cutaneous manifestations are panniculitis and purpura. Cytophagic histiocytic panniculitis fits within the spectrum of HPS, and the most consistent histopathologic feature in HPS is a proliferation of mature histiocytes that exhibit prominent erythrophagocytosis and cytophagocytosis. The clinical spectrum, the underlying causes, and the histopathologic features found in HPS are broad. The characteristic phagocytic histiocytes seen in HPS have been confused with malignant histiocytes in the past, but are now known to be reactive. The clinical findings, histologic, and immunohistochemical features of 10 cases of HPS with cutaneous lesions were reviewed. Immunohistochemical markers included KP-1, beta F-1, UCHL-1, L-26, MAC-387, factor XIIIa, and S100 protein. RESULTS: The HPS was associated with T-cell lymphoma and/or viral infection. Most biopsy specimens showed edema and hemorrhage with a lymphohistiocytic infiltrate and prominent histiocytic cells showing erythrophagocytosis and, in some cases, cytophagocytosis. The histiocytic cells showed positive reactions for KP-1 and negative reactions for the lymphoid markers. In all cases the lymphoid cells showed a mixed pattern with most cells positive for beta F-1 and UCHL-1, and a small percentage positive for L-26. CONCLUSION: In HPS, the prominent phagocytic histiocytes are reactive and are stimulated by T-cell lymphocytes, either neoplastic or in response to viral infection. Many of the findings in the HPS may also be due directly or indirectly to cytokines produced by proliferating T-cell lymphocytes and/or reactive phagocytic histiocytes.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Tumor necrosis factor.

Tumor necrosis factor is important in systemic and cutaneous defense, homeostasis, and many disease states. The numerous and diverse effects of tumor necrosis factor are best understood when considered as concentration-dependent, with normal homeostasis progressing to defense followed by toxic effects. Understanding tumor necrosis factor is important for the dermatologist as more studies appear in our literature and potential clinical uses of tumor necrosis factor (and possible anti-tumor necrosis factor agents) are realized.

Communicable Diseases↗
PubMed1991

Concurrent epidermal involvement of cytomegalovirus and herpes simplex virus in two HIV-infected patients. Military Medical Consortium for Applied Retroviral Research (MMCARR).

Although cytomegalovirus has previously been reported in cutaneous lesions of patients infected with the human immunodeficiency virus, these reports are not common despite the prevalence of this infection and the significant pathologic characteristics that it induces in HIV disease. Rare reports of possible epidermal involvement by cytomegalovirus have never been fully documented and have been believed by some to represent epidermal involvement by varicella-zoster and/or herpes simplex infections, with dermal involvement of cytomegalovirus. We present two cases of concurrent epidermal involvement by cytomegalovirus and herpes simplex virus documented by immunohistochemical studies and DNA hybridization studies and correlate this with the distinctive morphologic features seen in these two viral infections on routine staining.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Cutaneous lesions of hemophagocytic syndrome in a patient with T-cell lymphoma and active Epstein-Barr infection.

The hemophagocytic syndrome (HPS) was first described in immune-compromised patients with secondary viral infections. Immunodeficiency has not always been diagnosed before the onset of HPS, but most patients who develop HPS have known immune deficits. HPS has also been reported in association with lymphoreticular malignancies. In HPS the most prominent histologic change is phagocytosis of red blood cells and other bone marrow-derived elements by cytologically benign histiocytes. Considerable confusion exists about the similarities and differences between HPS and other conditions in which hemophagocytosis may occur, for example, histiocytic medullary reticulosis, T-gamma lymphoma, malignant histiocytic lymphoma, and cytophagic histiocytic panniculitis. We report a patient with HPS and cutaneous lesions of cytophagic histiocytic panniculitis who also had an active Epstein-Barr virus infection, and a CD8+ T-cell lymphoma.

Adult↗
PubMed1991

Cutaneous lesions of listeriosis in a newborn.

Although HIV-infected patients are commonly infected by organisms that require an intact T cell immune system for control or eradication, there are some exceptions. The intracellular pathogen Listeria monocytogenes is one such organism. Listeriosis occurs primarily in neonates, elderly patients, patients on immune suppressive medications, cancer patients, and during pregnancy. However, listeriosis is an uncommon opportunistic infection in HIV-infected patients. We report a case of listeriosis with cutaneous lesions in a neonate born to an HIV-infected woman.

Humans↗
PubMed1991

Papular eruption of human immunodeficiency virus disease. A review of the clinical, histologic, and immunohistochemical findings in 48 cases. The Military Medical Consortium for Applied Retroviral Research.

The papular eruption (PE) associated with human immunodeficiency virus infection, although described as a distinctive clinicopathologic entity, has shown a wide range of histologic findings ranging from eosinophilic folliculitis to granuloma annulare. We examined 48 cases of the PE in order to define the histologic spectrum of these lesions, and to correlate these findings with clinical presentations. The most distinctive clinical features are the frequency of these lesions in this population, the large number of lesions, pruritus, and the chronic nature of the lesions. Histologically, the distinctive features are the prominent perivascular factor XIIIa-positive dermal dendritic cells, atypical vascular proliferation, and dermal fibrosis sometimes associated with diffuse necrobiotic changes.

Adult↗
PubMed1991

Parapoxvirus infections acquired after exposure to wildlife.

The histopathologic and electron microscopic findings in two patients with skin lesions that developed after exposure to deer and other wildlife were consistent with a parapoxviral infection. Human infections that were morphologically similar to Parapoxvirus infection have been previously described concerning exposure to cervids (deer and related animals). Ours are the first reported cases in which viral particles were demonstrated by electron microscopy.

Aged↗
PubMed1990

Cutaneous histopathologic findings in 'antiphospholipid syndrome'. Correlation with disease, including human immunodeficiency virus disease.

The term antiphospholipid syndrome is used to characterize a complex of clinical and pathologic findings mediated by a group of antibodies formed against a family of antiphospholipids. These antiphospholipid antibodies were originally found in patients with lupus erythematosus in whom the partial thromboplastin time was prolonged and in patients with other autoimmune diseases; subsequently, they have been observed in association with a variety of other conditions, including infections, reactions to drugs, malignant neoplasms, human immunodeficiency virus disease, and as an isolated finding. In recent years, there has been some clarification of the significance of the various tests for antiphospholipid antibodies, including the lupus anticoagulant test and the anticardiolipin antibody tests, in predicting the antiphospholipid syndrome. The mechanism of disease, however, has not been well defined. The most common cutaneous lesion seen in seven patients with lupus anticoagulant and anticardiolipin antibody who have the antiphospholipid syndrome was ulceration due to thrombosis of dermal veins and arteries. Often there is a reactive vascular proliferation around the thrombosed vessels. The presence of primary thrombosis of both veins and arteries in thrombotic disorders is unusual and may provide insight into the mechanism of thrombosis in antiphospholipid syndrome.

AIDS-Related Complex↗
PubMed1990

Segmental neurofibromatosis: immunocytochemical analysis of cutaneous lesions.

Cutaneous lesions from three patients with segmental neurofibromatosis were evaluated. Routine histologic studies revealed the presence of redimentary neural structures within an abundant collagenous matrix. The majority of the cells in all three cases expressed S-100 protein, suggesting their identity as Schwann cells. The stromal component stained positively for fibronectin and type IV collagen; the latter indicated the presence of basement membrane material. Embedded in the tumor mass were glandular epithelial structures that stained with epithelial membrane antigen antibody. Staining for factor VIII-related antigen revealed vascular endothelium and multiple scattered mast cells. In one case strands of cells stained with antibodies to desmin, suggesting muscle cell differentiation. This case may represent a distinct subset of neurofibromas.

Adult↗
PubMed1990

Cutaneous and ocular manifestations of Down syndrome.

A patient with Down syndrome and extensive elastosis perforans serpiginosa is presented. There is an increased incidence of elastosis perforans serpiginosa, alopecia areata, syringomas, and Norwegian scabies in patients with this syndrome. Ocular findings include slanting of the palpebral fissures, Brushfield's spots, nystagmus, and epicanthal folds. Serious ocular complications that may impair vision include keratoconus, congenital cataracts, and retinal detachment.

Adenoma, Sweat Gland↗