1/13. erythema elevatum diutinum in a patient with human immunodeficiency virus.erythema elevatum diutinum (EED) is a chronic cutaneous leukocytoclastic vasculitis. This rare disease is characterized by red, brownish-purple, and yellow papules, plaques, and nodules distributed symmetrically about the extremities. There have been recent reports of the disease in association with infection with the human immunodeficiency virus (hiv). We describe the case of a 51-year-old man with hiv who presented with EED, which was successfully treated with dapsone.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/13. erythema annulare centrifugum-type psoriasis: a particular variant of acute-eruptive psoriasis.A case of erythema annulare centrifugum-type psoriasis in a 58-year-old white woman with a history of chronic plaque psoriasis is described. Initial failure of antipsoriatic treatments and an untypical histology complicated the diagnosis. After several trials acitretin at a low maintenance dose combined with oral fish oil and topical calcitriol led to sustained long-term remission. The spectrum of clinical differentiation of this rare disease, the histological characteristics and its nosological classification are discussed. It is suggested that this dermatosis represents a variant of acute psoriasis, rather than a variant of pustular psoriasis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/13. A case of hereditary angioedema with recurrent arthritis, erythema marginatum-like rash and chest pain.Hereditary angioedema (HAE) results from a congenital deficiency of C1 inhibitor and is characterized by submucosal and subcutaneous edema of skin, larynx and abdomen. Occasional reports have appeared linking HAE with autoimmune diseases. We report a case of HAE presenting recurrent nondeforming polyarthritis, erythema marginatum-like rash and chest pain. There were no significant radiographic joint changes. serologic tests for rheumatologic and autoimmune diseases were negative. After danazol treatment, physical examination and laboratory findings were normal over five years. We suggest that pediatricians should be aware of this rare disease and treat patients accordingly.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/13. erythema annulare centrifugum with autoimmune hepatitis.erythema annulare centrifugum (EAC) is a rare disease entity characterized by dense perivascular lymphocytic infiltrates in dermis. It has been associated with a few conditions, though its etiology is largely unknown. To our knowledge there has been no reported association with autoimmune hepatitis described earlier. This child also was positive for hepatitis C virus antibodies, though HCV rna was negative. We should keep in mind the false positivity of hepatitis c antibodies before deciding on its therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/13. Actinomycotic suppurative thyroiditis in a child.AIM: To present a rare case of actinomycotic suppurative thyroiditis in an infant with provision of the etiology, pathogenesis, clinical findings and treatment of this rare disease. DESIGN: A report of an 18-month-old female infant who presented with fever, erythema, induration and tenderness of the neck. The patient had the diagnosis of acute suppurative thyroiditis after a series of laboratory evaluation. RESULT: She was treated successfully with surgical debridement and intravenous penicillin g. CONCLUSION: Although rare, actinomyces spp. should be considered in the etiology of acute suppurative thyroiditis. Because of its fastidious nature the probability of positive culture is low, thus, the microbiology laboratory should be called in advance to make preparations before culture and transport.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/13. Cutaneous reactive angiomatosis occurring in erythema ab igne can cause atypia in endothelial cells: potential mimic of malignant vascular neoplasm.erythema ab igne (EAI), an old and rare disease, is an erythematous, often pigmented, reticular, macular dermatosis that occurs at the site of repeated exposure to moderate heat. Reported herein is an unusual case of EAI occurring in a 33-year-old woman with a very broad lesion of reticular erythema and pigmentation on the lower extremities. The patient frequently put her lower extremities close to a heater in the wintertime to alleviate chill. The lesion started a decade ago, and it gradually became conspicuous. Microscopic findings showed a proliferation of small blood vessels in a thickened papillary dermis, not as typical as seen in EAI, but as seen in cutaneous reactive angiomatosis. They were arranged as small lobules and associated with hyalinization, edema and delicate fibroplasia. Many vessels were lined by plump endothelial cells, some of which had enlarged hyperchromatic nuclei. Many of these cells were multinucleated. Similar-appearing cells were associated with concentric foci of hyalinization without vascular lumina. A few atypical mitoses were observed. The lesion became much less conspicuous after the patient started avoiding close exposure to a heater, without any other special treatments. The aforementioned changes may be confused with malignant vascular neoplasm because of unusual cytological atypia and atypical mitoses in the endothelial cells.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/13. Treatment of scleromyxoedema with hydroxychloroquine.BACKGROUND: Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis. patients AND methods: Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG lambda paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p.o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter. RESULTS: Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61 , 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months. CONCLUSION: hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/13. adult-onset acute rheumatic fever: possible resurgence in southern taiwan.BACKGROUND: By the late 1980s, acute rheumatic fever (ARF) had become a rare disease in taiwan. The low prevalence rate in this area is attributed to a better economic status, which has led to improved public health and adequate medical services. OBJECTIVES: The increasing number of patients with adult-onset ARF in the united states described in the literature prompted us to evaluate the cases diagnosed in our medical center. methods: A retrospective chart review was performed for patients with arthritis from July 1988 to October 2004. To be included, patients had to meet revised Jones criteria. RESULTS: Three adult patients with ARF have been diagnosed since June 2001, with no childhood ARF being diagnosed. All cases presented with migratory polyarthritis, whereas 1 had erythema marginatum and transient carditis. These patients responded well to treatment with antibiotics and nonsteroidal antiinflammatory drugs. CONCLUSIONS: Clinicians must provide careful assessment and treatment to patients presenting with acute pharyngitis. A possible resurgence of ARF can be eradicated by primary prevention of streptococcal pharyngitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/13. Recurring localized scarlatiniform scaled erythema Fereol-Besnier.Recurring scarlatiniform scaled erythema of Fereol-Besnier is a rare disease characterized by recurrent episodes of a prodromal phase with general malaise, head and muscle aches, gastrointestinal complaints and fever followed by an erythematous rash leading to extensive desquamation of the involved skin. It exists in a generalized and localized variant, the latter mainly involving the hands and feet. Its cause is unknown, although it has been speculated that a hyperergic reaction to infectious agents or medications may be etiopathologically involved. A typical case of the localized variant of this obscure disease is described and the common literature is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/13. erythema elevatum diutinum--a solitary lesion in a patient with rheumatoid arthritis.erythema elevatum diutinum is a rare disease of unknown aetiology. It is usually symmetrical with multiple lesions. An association with rheumatoid has previously been reported. We report a case of atypical erythema elevatum diutinum affecting the right elbow of a female patient with seropositive rheumatoid arthritis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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