1/14. erdheim-chester disease: a case report.A 42-year-old man with erdheim-chester disease (EC) is presented. This is the first case of this disease reported in korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/14. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
3/14. A sporadic case of progressive mucinous histiocytosis.Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. immunohistochemistry showed positive labelling with alpha-1 antitrypsin, factor xiiia and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
4/14. erdheim-chester disease: two cases of orbital involvement.erdheim-chester disease (ECD) is an increasingly recognised form of fibro-inflammatory process characterised by xanthomatous histiocytes containing large amounts of ingested lipid, plasma cells, macrophages and Touton-type giant cells. Ophthalmic involvement in ECD has been reported in only 22 cases. We describe two patients, one presenting with diabetes insipidus and subsequently developing orbital pseudotumours and retroperitoneal fibrosis, the other presenting with exophthalmos and diplopia. The first patient was treated with cladribine and subsequently developed sudden onset of bilateral blindness while the second required radiation therapy for the retro-orbital process and developed radiation retinopathy. These cases typify the variable presentation and course in patients with ECD.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
5/14. Conjunctival necrobiotic granuloma in xeroderma pigmentosum.PURPOSE: We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum. methods: A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass. RESULTS: The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis. CONCLUSION: Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
6/14. Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis.Multicentric reticulohistiocytosis (MRH) is a rare disorder with proliferating histiocytes that develop into multinucleated giant cells with "ground-glass" cytoplasm. The disease presents with a rapidly destructive, sometimes permanently debilitating, polyarthritis and a papulonodular eruption, generally of the face and hands. We present 3 cases of MRH in which the initial clinical diagnosis was thought to be dermatomyositis (DM). The cutaneous findings in these cases included an erythematous, predominantly photodistributed macular and papular eruption clinically consistent with DM. However, skin biopsy specimens revealed the diagnosis of MRH. This previously unreported clinical similarity between MRH and DM is significant because the treatment options and future complications vary greatly between the 2 diseases. Thus, it is important to consider the possibility of multicentric reticulohistiocytosis when evaluating a patient with the clinical diagnosis of dermatomyositis.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
7/14. Multicentric reticulohistiocytosis presenting with destructive polyarthritis, laryngopharyngeal dysfunction, and a huge reticulohistiocytoma.Multicentric reticulohistiocytosis (MRH) is a rare multisystemic disease presenting with skin lesions and erosive polyarthritis and is often associated with malignancy. We describe a 60-year-old woman with diffuse papulonodular skin eruptions and progressive osteolytic bone damage over the bilateral hands, humeral head, and acromioclavicular joints within 2 years. Moreover, dysphagia and a hoarse voice occurred in this patient and an unusual huge mass-reticulohistiocytoma--developed over the left upper back. Tissue biopsy of the skin lesions, laryngeal nodules, and this large mass showed infiltration of numerous CD68( ) histiocytes and multinucleated giant cells with abundant eosinophilic ground-glass cytoplasm. Combination therapy with steroids and methotrexate improved her cutaneous, joint, and laryngopharyngeal symptoms. The large reticulohistiocytoma resolved with methylprednisolone pulse therapy. This polyarthritis, which can be confused with rheumatoid arthritis, can be diagnosed by careful immunohistochemical examination of biopsies. To prevent the irreversible disease process, early and aggressive therapy is necessary.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
8/14. Xanthoma disseminatum: a case with extensive mucous membrane involvement.A 37-year-old man had red or brownish papules on his face, chest, and groin, where they coalesced into plaques. Endoscopic examination revealed the presence of small xanthomatous papules on the mucous membrane of the stomach. A biopsy specimen revealed a dense infiltrate of histiocytes, foam cells, Touton giant cells, and inflammatory cells. The diagnosis of non-X histiocytosis was confirmed by immunohistochemical studies of the infiltrate.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
9/14. Orbital and eyelid involvement with erdheim-chester disease. A report of two cases.erdheim-chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with erdheim-chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
10/14. Disseminated dermal dendrocytomas. A new cutaneous fibrohistiocytic proliferative disorder?A 77-year-old white woman presented with 1 1/2-year history of progressively enlarging cutaneous papules, nodules, and plaques, some of which had spontaneously regressed. Her past medical history included untreated chronic lymphocytic leukemia for 10 years' duration. Multiple skin biopsy specimens revealed a diffuse superficial and deep dermal spindle-cell infiltrate accompanied by occasional foamy round cells and multinucleated giant cells. The spindle-shaped cells were focally arranged in a storiform pattern with prominent fibrous stroma. The spindle-shaped cells stained positively for numerous macrophage markers including CD45, factor xiiia, Leu M5, HLA-DR, CD4, and Leu M3, consistent with dermal dendrocytes. They were also positive for nonspecific esterase and acid phosphatase, which is typical of tissue macrophages. The spindle-shaped cells were negative for CD-1, S-100, and ATPase activity, thus excluding a Langerhans cell immunophenotype. Combining the clinical features, light microscopy, immunohistochemistry, and enzymatic analysis, this patient appears to represent a novel cutaneous fibrohistiocytic proliferative disorder that features large numbers of dermal dendrocytes.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
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