1/4. Monoclonal rearrangement of the T cell receptor gamma-chain in lichenoid pigmented purpuric dermatitis of gougerot-blum responding to topical corticosteroid therapy.Lichenoid pigmented purpuric dermatitis of Gougerot-Blum belongs to a group of closely related disorders which are termed pigmented purpuric dermatoses. It clinically manifests itself with grouped lichenoid papules in association with purpuric lesions. We report a case of lichenoid pigmented purpuric dermatitis of Gougerot-Blum with a heavy band-like CD4-positive lymphocytic infiltrate and clonal rearrangements of the gamma-chain of the T cell receptors as detected by polymerase chain reaction/denaturing gradient gel electrophoresis. Monoclonal expansion of T cells in combination with certain histological features of mycosis fungoides (MF) might support a biological relationship between lichenoid pigmented purpuric dermatitis of Gougerot-Blum and MF. However, prompt clinical response to topical steroid therapy supports the benign clinical nature of our case.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/4. scleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-1a.BACKGROUND: Animal studies have shown that some human monoclonal antibodies promote myelin repair in models of demyelinating disease. scleromyxedema is a dermatologic disorder associated with a monoclonal gammopathy and neurologic manifestations. The reason for occurrence of cutaneous reactions in interferon treated patients is unknown. CASE DESCRIPTION: A 37-year-old woman was started on weekly interferon beta-1a (IFN beta-1a) following a diagnosis of multiple sclerosis (MS). After having been on interferon therapy for three years, she developed skin lesions secondary to scleromyxedema. Her IFN beta-1a was discontinued and intravenous immunoglobulin therapy was started for her scleromyxedema. At a six-month follow up, her skin lesions improved and there was no recurrence of neurologic symptoms. CONCLUSIONS: This is the first report of occurrence of scleromyxedema in a patient with MS. While this could be a chance association, it does raise the question if her neurologic manifestations could be secondary to scleromyxedema. Further research into the mechanism of IFN related cutaneous side effects is needed. Evidence regarding the remyelinating nature of human monoclonal antibodies raises interest in the potential therapeutic role these antibodies may have.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/4. Extensive ulcerative and erosive lichenoid dermatosis in a patient with malignant lymphoma.We report here an unusual case of extensive ulcerative and erosive lichenoid dermatosis in a 39-year-old man with non-Hodgkin centroblastic-centrocytic B-cell lymphoma. The simultaneous presentation of the lichenoid eruption and malignant lymphoma favored a causal relationship between the two conditions. It is suggested that the lichenoid cellular reaction may be an immunological response to some yet-unidentified tumor products. Electron-microscopic examination revealed apoptotic keratinocytes with filamentous degeneration in the epidermis. In the upper dermis many globular filamentous bodies (Civatte bodies) composed of densely packed wavy filaments 6-12 nm in diameter and a varying number of cytoplasmic organelles were identified. Serial skin biopsies showed the evolution of the lichenoid lesion with time and treatment. Large numbers of colloid bodies were seen in persistent lesions but were absent in healed ones. The characteristic wavy nature of the filaments was maintained and no transition to the straight fibrils of amyloid was observed. The relationship between colloid bodies and amyloid is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/4. Papular mucinosis: is the inflammatory cell infiltrate neoplastic? The presence of a monotypic plasma cell population demonstrated by in situ hybridization.Papular mucinosis is a condition reported to be associated with abnormal serum paraproteins and plasma cell dyscrasias. We report a patient with papular mucinosis, without a serum paraprotein or bone marrow plasmacytosis, in whom the affected skin contained a prominent perivascular plasma cell infiltrate. Using in situ hybridization, for kappa and lambda light chain mRNA, these plasma cells were demonstrably monotypic for lambda light chain and, therefore, presumably monoclonal and putatively neoplastic. We suggest that the absence of a serum paraprotein and marrow plasmacytosis does not exclude the existence of a plasma cell neoplasm in patients with papular mucinosis. Such plasma cell populations may exist in the affected skin, although their true nature and behaviour remains to be determined.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |