Malignant atrophic papulosis (MAP) is a rare, obliterating vasculopathy affecting multiple systems, frequently with a poor prognosis. Although cutaneous lesions are often the initial presentation, systemic involvement...Malignant atrophic papulosis (MAP) is a rare, obliterating vasculopathy affecting multiple systems, frequently with a poor prognosis. Although cutaneous lesions are often the initial presentation, systemic involvement is also common, usually with a fatal outcome. Involvement of the genitalia is very rare. We describe a 45-year-old male patient with multisytemic manifestation of MAP accompanied by painful penile ulceration. The pathogenesis of MAP is not yet fully understood and effective treatment choices are limited. In our case, the combination of pentoxifylline and dipyridamole failed to provide a beneficial effect on the progression of the disease and the patient died due to intestinal and intrathoracic manifestation of MAP. In the present case, attention should be drawn to the following clinical course and therapeutic properties: (i) we describe the second patient in the literature diagnosed with MAPand painful penile ulceration; (ii) to our knowledge, this is the first reported case with oesophageal fistula due to MAP; (iii) we could not confirm the efficacy of pentoxifylline, the recently reported treatment modality, in our patient.展开更多
Background. Chronic urticaria is a common disease with an unclear pathogenesis, which may be resistant to therapy. Recent studies have focused primarily on a possible autoimmune basis. Aim. To investigate HLA class I ...Background. Chronic urticaria is a common disease with an unclear pathogenesis, which may be resistant to therapy. Recent studies have focused primarily on a possible autoimmune basis. Aim. To investigate HLA class I and II antigens in a Turkish population with chronic ordinary urticaria (COU; not physical, vasculitic or contact), and identify susceptible HLA antigens. Methods. HLA antigens were investigated in 55 patients diagnosed with COU, using a two-stage microdroplet lymphocytotoxicity test, with 104 healthy and genetically unrelated individuals evaluated as the control group. Results. HLA Bw4 and HLA DQ1 antigens were significantly higher in the study group (odds ratio (OR) = 2.93, 95%CI 1.47-5.85, P =0.003 and OR = 7.81, 95%CI 1.96-28.50, P = 0.001, respectively)whereas HLA-A24 antigen was higher in controls (OR= 0.36, 95%CI 0.15-0.86, P = 0.03). Conclusion. We propose that HLA-Bw4 and DQ1 antigens may be responsible for susceptibility to COU while HLA-A24 may have a protective role in the Turkish population.展开更多
Rowell s syndrome (RS) is a rare presentation of lupus erythe- matosus (LE) with erythema multiforme-like lesions associated with antinuclear, anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) posi...Rowell s syndrome (RS) is a rare presentation of lupus erythe- matosus (LE) with erythema multiforme-like lesions associated with antinuclear, anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64-year-old woman with LE and a 51-year-old woman with LE and Sj¨ ogren syndrome (SS) who had erythema multiforme-like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.展开更多
Background: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis ...Background: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement,whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8%of SLE, while 50%of HUVS patients are diagnosed with SLE. Observations and results: We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. Conclusions: SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE.As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.展开更多
文摘Malignant atrophic papulosis (MAP) is a rare, obliterating vasculopathy affecting multiple systems, frequently with a poor prognosis. Although cutaneous lesions are often the initial presentation, systemic involvement is also common, usually with a fatal outcome. Involvement of the genitalia is very rare. We describe a 45-year-old male patient with multisytemic manifestation of MAP accompanied by painful penile ulceration. The pathogenesis of MAP is not yet fully understood and effective treatment choices are limited. In our case, the combination of pentoxifylline and dipyridamole failed to provide a beneficial effect on the progression of the disease and the patient died due to intestinal and intrathoracic manifestation of MAP. In the present case, attention should be drawn to the following clinical course and therapeutic properties: (i) we describe the second patient in the literature diagnosed with MAPand painful penile ulceration; (ii) to our knowledge, this is the first reported case with oesophageal fistula due to MAP; (iii) we could not confirm the efficacy of pentoxifylline, the recently reported treatment modality, in our patient.
文摘Background. Chronic urticaria is a common disease with an unclear pathogenesis, which may be resistant to therapy. Recent studies have focused primarily on a possible autoimmune basis. Aim. To investigate HLA class I and II antigens in a Turkish population with chronic ordinary urticaria (COU; not physical, vasculitic or contact), and identify susceptible HLA antigens. Methods. HLA antigens were investigated in 55 patients diagnosed with COU, using a two-stage microdroplet lymphocytotoxicity test, with 104 healthy and genetically unrelated individuals evaluated as the control group. Results. HLA Bw4 and HLA DQ1 antigens were significantly higher in the study group (odds ratio (OR) = 2.93, 95%CI 1.47-5.85, P =0.003 and OR = 7.81, 95%CI 1.96-28.50, P = 0.001, respectively)whereas HLA-A24 antigen was higher in controls (OR= 0.36, 95%CI 0.15-0.86, P = 0.03). Conclusion. We propose that HLA-Bw4 and DQ1 antigens may be responsible for susceptibility to COU while HLA-A24 may have a protective role in the Turkish population.
文摘Rowell s syndrome (RS) is a rare presentation of lupus erythe- matosus (LE) with erythema multiforme-like lesions associated with antinuclear, anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64-year-old woman with LE and a 51-year-old woman with LE and Sj¨ ogren syndrome (SS) who had erythema multiforme-like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.
文摘Background: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement,whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8%of SLE, while 50%of HUVS patients are diagnosed with SLE. Observations and results: We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. Conclusions: SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE.As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.