6 research outputs found
Epidemiology of pemphigus in Turkey: One-year prospective study of 220 cases
Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation). The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus. </p
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Recurrent angiolymphoid hyperplasia with eosinophilia during several pregnancies
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign vascular tumor. Although its etiology is not clearly known, infection, hormonal factors, and trauma are the suspected reasons. There are few cases considered to be related to pregnancy described in the literature. Granulomas are rarely seen in the histopathology and only four patients with granulomas have been described. Herein, we will present a 28-year-old woman who has been pregnant three times; in every pregnancy, she developed pink nodular lesions on the back of her ear. In the first two pregnancies, the lesions had appeared during the pregnancy and regressed completely in the postpartum period. In her third pregnancy, the condition emerged again and lasted 1.5 years after birth of her child. Histopathology exhibited ALHE with granulomas. Although the relationship between ALHE and pregnancy is already known, there are few cutaneous ALHE cases associated with pregnancy reported in the literature. This association is further supported by this case having recurrent ALHE during each pregnancy period. Also, the presence of naked granuloma in histopathology, unlike most of the cases, contributes to the current histopathological data
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Intravascular lymphoma presenting with paraneoplastic syndrome
Intravascular lymphomatosis (IVL) is a rare type of B-cell non-Hodgkin lymphoma (BCNHL), which can mimic many other diseases. Fever, neurological symptoms, and skin findings are the most frequent clinical findings. Intravascular lymphomatosis may be associated with genetic factors and infection with human immunodeficiency virus (HIV), human herpesvirus 8 (HHV-8), human T-lymphotropic virus 1 (HTLV1), Epstein-Barr virus (EBV), and hepatitis B virus (HBV). A 50-year-old man was hospitalized with recalcitrant hyponatremia of unknown cause. He had also telangiectatic, indurated, slightly erythematous plaques on his trunk for the last 10 days. His past medical history was unremarkable, although he was a carrier of hepatitis B. Multiple skin biopsies were performed and were considered to be diagnostic of IVL. The hyponatremia was unresponsive to water restriction and hypertonic solution support but it resolved with B cell directed chemotherapy. A final diagnosis was made as syndrome of inappropriate antidiuretic hormone (SIADH) in the setting of IVL. This case had a relatively early diagnosis with just 10-days of skin lesions. Intravascular lymphomatosis is a very rare disease and is usually difficult to diagnose. An even more uncommon presentation is IVL complicated by a paraneoplastic syndrome. There are prior reported cases of SIADH in the setting of IVL. However, this case underscores the importance of evaluating patients with SIADH for potential IVL
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Intravascular lymphoma presenting with paraneoplastic syndrome
Intravascular lymphomatosis (IVL) is a rare type of B-cell non-Hodgkin lymphoma (BCNHL), which can mimic many other diseases. Fever, neurological symptoms, and skin findings are the most frequent clinical findings. Intravascular lymphomatosis may be associated with genetic factors and infection with human immunodeficiency virus (HIV), human herpesvirus 8 (HHV-8), human T-lymphotropic virus 1 (HTLV1), Epstein-Barr virus (EBV), and hepatitis B virus (HBV). A 50-year-old man was hospitalized with recalcitrant hyponatremia of unknown cause. He had also telangiectatic, indurated, slightly erythematous plaques on his trunk for the last 10 days. His past medical history was unremarkable, although he was a carrier of hepatitis B. Multiple skin biopsies were performed and were considered to be diagnostic of IVL. The hyponatremia was unresponsive to water restriction and hypertonic solution support but it resolved with B cell directed chemotherapy. A final diagnosis was made as syndrome of inappropriate antidiuretic hormone (SIADH) in the setting of IVL. This case had a relatively early diagnosis with just 10-days of skin lesions. Intravascular lymphomatosis is a very rare disease and is usually difficult to diagnose. An even more uncommon presentation is IVL complicated by a paraneoplastic syndrome. There are prior reported cases of SIADH in the setting of IVL. However, this case underscores the importance of evaluating patients with SIADH for potential IVL