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Biomedical subjects

W W Piette

Publications and source records attributed to W W Piette.

28 records · Page 2Linked to original sources

An approach to cutaneous changes caused by hematologic malignancies.

Most hematologic malignancies can present with or later develop cutaneous lesions. The proper diagnosis of such patients through the evaluation of their cutaneous lesions is complicated both by the confusing variety of clinical findings and by the shifting classifications of hematologic malignancies in the face of evolving knowledge. The spectrum of T-cell malignancies continues to increase and now includes several disorders formerly attributed to B-cells or macrophages. The cutaneous findings of malignant and malignant behaving hematologic disorders are grouped by the presumed cellular etiology and by the clinical subset of tumor. Clinical and histopathologic features unique to individual disorders are emphasized, as well as those features that may be shared by otherwise quite dissimilar malignancies.

Hematologic Diseases↗

Xanthoma disseminatum. An unusual histiocytosis syndrome.

The histiocytoses are a diverse group of illnesses that present a variety of diagnostic and therapeutic dilemmas. In this article, we describe the case of an 8-year-old boy with xanthoma disseminatum, a histiocytic disorder involving the skin, eyes, and brain. In our discussion, we have emphasized the clinical, pathologic, and radiographic features that distinguish this entity from clinically similar juvenile xanthogranuloma and Langerhans' cell histiocytoses (histiocytosis X) such as the Hand-Schuller-Christian syndrome. The importance of differentiating the various histiocytoses, in view of their varying natural histories and therapeutic responsiveness, is reviewed in relation to the new classification system for these disorders proposed by the Histiocyte Society.

Brain Diseases↗

The incidence of second malignancies in subsets of Kaposi's sarcoma.

There is strong evidence linking Kaposi's sarcoma with an increased risk of developing a second primary malignancy. This risk of a second malignancy appears to depend in part on the clinical setting in which Kaposi's sarcoma develops. This paper examines these risks by relating them to specific subsets of Kaposi's sarcoma and to specific types of second malignancies.

Adolescent↗

Successful treatment of hypertrophic lupus erythematosus with isotretinoin.

A patient with systemic lupus erythematosus had the additional finding of hypertrophic lupus erythematosus. The lesions cleared with an 11-week course of isotretinoin alone. She has remained without recurrence for 9 months. This is the first reported case of total resolution of hypertrophic lupus erythematosus with a short course of isotretinoin.

Adult↗

Myeloma, paraproteinemias, and the skin.

This review focuses on those systemic diseases or syndromes associated with monoclonal plasma cell disorders that may present with important cutaneous manifestations. Amyloidosis, POEMS syndrome, cutaneous plasmacytoma, xanthomas, benign hypergammaglobulinemic purpura of Waldenström, and scleromyxedema are emphasized.

Amyloidosis↗

Urticating histiocytosis: a mast cell-rich variant of histiocytosis X.

Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.

Adolescent↗

Clinical and serologic expression of localized scleroderma. Case report and review of the literature.

A patient is presented in whom both linear scleroderma and eosinophilic fasciitis developed. The latter is rare in childhood. A positive antinuclear antibody, a positive rheumatoid factor, and seizures also developed. In reviewing the literature, we concluded that the clinical and histologic pattern of cutaneous sclerosis may be more valuable than serologic findings in predicting both the likelihood of, and the expected sites for, systemic involvement. The literature also suggests that central nervous system (CNS) involvement is rare in most sclerosing syndromes but that linear scleroderma may be associated with CNS or spinal disease. The ipsilateral association of linear scleroderma and seizure focus in this patient, as well as the focal onset of these seizures, is unusual and suggests an association between the seizure disorder and the linear cutaneous sclerosis.

Adolescent↗