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Mucin Deposition (mucin + deposition)
Selected AbstractsNephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: a case series of nine patients and review of the literatureINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 5 2007Camille E. Introcaso MD Background, Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a fibrosing cutaneous disorder recently recognized to have systemic manifestations. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and histologically by an increase in spindle-shaped cells, collagen, and sometimes mucin deposition in the dermis. The only common exposure amongst patients is acute or chronic renal failure. The pathophysiology of the disease remains to be elucidated, and there is currently no consistently effective treatment for this unremitting disease. Methods, We report a case series of nine patients seen at the University of Pennsylvania between 1998 and mid-2004. The clinical, laboratory, and pathologic data of these patients are reviewed. Results, All patients had renal disease, received peritoneal or hemodialysis, and five had received at least one renal transplant. All patients had characteristic fibrotic cutaneous lesions involving the trunk, extremities, or both, and eight of the nine patients had scleral plaques. There were no other common findings amongst the histories, medications, or laboratory results of the patients. Conclusion, Our report confirms the clinical and histologic characteristics of NFD that have been described previously, and raises new issues regarding the possible subtypes. A review of the current literature stresses that further basic science and translational studies are necessary to understand the disease mechanism and to propose effective therapy, and emphasizes the importance of recognizing the systemic effects of NFD. [source] Primary cutaneous myxoid spindle cell squamous cell carcinoma: a clinicopathologic study and review of the literatureJOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2010Aparche Yang Mucocutaneous squamous cell carcinoma (SCC) may rarely exhibit intracellular mucin production. Extracellular mucin production is an even rarer finding in SCC that is not well documented in the literature. Here, we report six cases of primary cutaneous and mucocutaneous SCC with prominent extracellular stromal mucin deposition and an epithelial spindle cell component. We propose the term ,yxoid spindle cell SCC' (MSC SCC) to describe the histologic characteristics of these six cases. We also propose a set of histologic and immunohistochemical findings for distinguishing MSC SCC from primary cutaneous and metastatic spindle cell neoplasms including other sarcomatoid carcinomas, myxoid sarcomas and the spindle cell variant of atypical fibroxanthoma (AFX). The criteria can also help discern MSC SCC from spindle cell melanomas, which may rarely show a prominent myxoid stroma. Given the small numbers of cases reported to date, the presence of prominent myxoid stroma in primary cutaneous spindle cell SCC has unknown prognostic significance at this time. Yang A, Hanley A, Velazquez EF, Cassarino DS. Primary cutaneous myxoid spindle cell squamous cell carcinoma: a clinicopathologic study and review of the literature. [source] Lupus erythematosus panniculitis (lupus profundus): Clinical, histopathological, and molecular analysis of nine casesJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2005Cesare Massone Background:, The diagnosis of lupus erythematosus panniculitis (LEP) may be very difficult in cases in which involvement of the subcutaneous fat is the only manifestation of the disease. The main differential diagnosis is subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Methods:, We performed a retrospective study reviewing the histopathologic features of 11 biopsy specimens from nine patients with LEP (M : F = 2 : 7; median age: 48 years; range: 20,71 years). Results:, Histopathologically, all biopsies revealed a lobular panniculitis, with concomitant septal involvement in 82% of them. Dermal changes included the presence of superficial and deep infiltrates (82%) and mucin deposition (73%). The majority of cases (73%) presented also some form of epidermal involvement. The subcutaneous infiltrate was composed of lymphocytes in all cases, admixed with plasma cells in 91% of cases. Lymphoid follicles with reactive germinal centers were detected in 45% of cases. Immunohistochemistry showed a predominance of ,/,-T-helper and cytotoxic lymphocytes in 80% of cases admixed with B lymphocytes. The polymerase chain reaction analysis of the T-cell receptor (TCR)-, gene showed a polyclonal smear in all cases. Conclusions:, Our study shows that the most useful histopathologic criteria for distinguishing LEP from SPTCL are the presence of involvement of the epidermis, lymphoid follicles with reactive germinal centers, mixed cell infiltrate with prominent plasma cells, clusters of B lymphocytes, and polyclonal TCR-, gene rearrangement. [source] Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead-Ringer for Lupus ErythematosusJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Ma We report a 48-year-old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4,CD8+/,CD56+. Monoclonal rearrangements of T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis-like T-cell Lymphoma (SPTCL) with systemic involvement. [source] | ||||||||||