1/19. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
2/19. common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor-alpha receptor fusion protein.common variable immunodeficiency (CVI) is characterized by a failure in B-cell differentiation and impaired immunoglobulin secretion, but with a variable clinical presentation, including the development of sarcoidal granulomas and autoimmune diseases, as well as an increased incidence of malignancies. We present a 21-year-old white man who carried a diagnosis of juvenile rheumatoid arthritis and presented 6 years later with scarring alopecia showing sarcoidal granulomas. Further work confirmed the diagnosis of CVI, and with increasing systemic symptoms, it was elected to treat the patient with a tumour necrosis factor (TNF)-alpha antagonist, a TNF-alpha receptor IgG1 fusion protein. The patient showed improvement in his systemic symptoms and some hair regrowth after 3 months of therapy, and continued improvement in his systemic disease with only mild scalp hair thinning in the areas of prior involvement after almost 1 year of therapy. CVI and sarcoid may have overlapping clinical and immunological findings. Previous therapies for CVI, including intravenous immunoglobulin, have not altered the mortality of the disease. TNF-alpha is a primary cytokine and is elevated in CVI, and specific inhibition of TNF-alpha in this patient was effective in moderating his disease, including his skin disease.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
3/19. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases.Two patients presenting with chronic pigmented purpuric dermatosis (CPPD) on the dorsum of both feet were found to show granulomatous inflammation superimposed on the pathological changes of CPPD. Two similar cases have been reported from japan. The unique clinicopathological features of this group of patients suggest that they have a rare granulomatous variant of CPPD.- - - - - - - - - - ranking = 4.1651939472341keywords = dermatosis (Clic here for more details about this article) |
4/19. A case of primary cutaneous CD30 T-cell lymphoproliferative disorder with features of granulomatous slack skin disease.We present a patient with primary CD30 cutaneous T-cell lymphoma whose histological and clinical features overlapped with those of granulomatous slack skin disease (GSSD). A 26-year-old woman had infiltrative erythema on the abdominal wall and an incurable ulcerative lesion on the left knee. Her skin progressively became atrophic and pendulous, showing a hyperpigmented appearance over almost the whole body. Histopathologically, a dense lymphoid cell infiltrate accompanying numerous macrophages and multinucleated giant cells (MGC) extended into the subcutaneous tissue. Most lymphoid cells were small and positive for T-cell markers. Some relatively large atypical cells were scattered in the lesion, most of which (60%) were positive for CD30. T-cell receptor-beta gene rearrangement was confirmed in the abdominal lesion. MGC infiltrated more dominantly into a deeeper layer of the skin with the elastic fibres there almost completely disappearing. Immunoreactivity for CD30 of MGC was negative and overexpression of elastolytic metalloproteinases was observed. The association between primary cutaneous CD30 lym- phoproliferative disorders and GSSD has not previously been reported. Overexpression of elastolytic metalloproteinases in MGC contributes to the disappearance of the elastic fibres and enhances the severity of the clinical course.- - - - - - - - - - ranking = 5keywords = skin disease (Clic here for more details about this article) |
5/19. Granulomatous slack skin disease--disease features and response to pentostatin.Granulomatous slack skin disease (GSSD) is a rare condition characterized clinically by redundant skin folds, which show a predilection towards flexural areas, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibres. The disease is often indolent, although rapid progression and transformation have been described. There is much debate as to whether this condition is a subset of mycosis fungoides or a separate disease entity in itself. We describe a case of GSSD with unique manifestations including granulomatous bone marrow involvement and hypercalcaemia. The patient has twice achieved a good response to pentostatin after failure of combination chemotherapy. This is the first report of the successful use of the purine analogue pentostatin in the management of GSSD.- - - - - - - - - - ranking = 5keywords = skin disease (Clic here for more details about this article) |
6/19. Palisaded neutrophilic and granulomatous dermatitis: an unusual cutaneous manifestation of immune-mediated disorders.OBJECTIVE: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption most often associated with rheumatoid arthritis and other immune-mediated diseases. We present 4 cases to familiarize rheumatologists with the clinical presentation and histopathology of PNGD. methods: We report 4 cases to illustrate the clinical and histologic spectrum of this rare skin disease found in rheumatologic patients. The disease pathogenesis and treatment options are discussed. RESULTS: All 4 patients presented with symmetric erythematous-to-violaceous papules and plaques that, upon biopsy, revealed a dermatitis composed of variable numbers of histiocytes and neutrophils. Some cases responded to topical corticosteroid treatment or to dapsone, whereas others resolved spontaneously. CONCLUSIONS: PNGD is a rare cutaneous finding in patients with a variety of immune-mediated systemic diseases, most often rheumatoid arthritis. It is a benign condition that may spontaneously remit or may respond favorably to topical corticosteroids or dapsone.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
7/19. Pituitary granuloma and pyoderma gangrenosum.pyoderma gangrenosum is a rare chronic and recurrent skin disease characterized by progressing lesions from papulopustules to large necrotic sterile ulcers. Its definite etiology remains unknown. In a 40-year-old woman with typical pyoderma gangrenosum an intrasellar mass with suprasellar extension was diagnosed and removed by transsphenoidal surgery. Histopathological features of the lesion were those of a nonspecific granulomatous hypophysitis. Five months postoperatively the patient experienced visual defects and hypopituitarism demonstrated by endocrine evaluation. Computerized tomography showed the recurrence of the intrasellar expanding mass. Extensive and repeated evaluation failed to find any evidence of sarcoidosis, tuberculosis or histiocytosis. Corticosteroid therapy was preferred to surgery and 80 mg daily prednisone produced a dramatic shrinkage of the pituitary pseudotumor. Long-term follow-up studies did not disclose any recurrence of the pituitary granulomatous process nor objective evidence of underlying disease even after steroid dosage has been tapered. The hypothesis of a pituitary localization of pyoderma gangrenosum is suggested by the similarity between the histopathologic findings of the two conditions and the excellent response to steroid therapy.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
8/19. Acral granulomatous dermatosis.A 17-year-old boy developed an acral granulomatous dermatosis which resembled clinically acrodermatitis continua of Hallopeau. The histology showed dermal and subcutaneous abscess formation with granulomas. The lesions responded to systemic corticosteroid therapy, with residual atrophy and contractures of the fingers.- - - - - - - - - - ranking = 4.1651939472341keywords = dermatosis (Clic here for more details about this article) |
9/19. antibodies to the borrelia burgdorferi flagellum in patients with scleroderma, granuloma annulare and porphyria cutanea tarda.It is generally accepted that cutaneous Lyme borreliosis comprises erythema chronicum migrans, lymphadenosis benigna cutis, and acrodermatitis chronica atrophicans. In recent years the tick-borne spirochete borrelia burgdorferi has been associated with a number of other cutaneous disorders. We therefore investigated sera from 175 patients with localized scleroderma (morphea) (n = 64), systemic sclerosis (n = 74), granuloma annulare (n = 16) and porphyria cutanea tarda (n = 21) with the new, highly sensitive and specific borrelia burgdorferi flagellum ELISA assay. As controls (n = 297) served normal healthy volunteers and patients with other skin diseases. It was found that the distribution of individual antibody values and the median antibody levels were identical in controls and in patients with scleroderma, granuloma annulare and porphyria cutanea tarda. These data do not support the hypothesis of an etiological association between borrelia burgdorferi infection and scleroderma, granuloma annulare or porphyria cutanea tarda.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
10/19. Linear granuloma annulare.granuloma annulare is a benign dermatosis with multiple clinical presentations and a common histologic picture, which is characterized by areas of degenerated collagen surrounded by palisading histiocytes. A case of granuloma annulare that presented as progressive, asymptomatic, symmetric truncal bands is described. The varied clinical presentations and possible pathogenetic mechanisms of granuloma annulare are reviewed.- - - - - - - - - - ranking = 0.83303878944682keywords = dermatosis (Clic here for more details about this article) |
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