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3.
Z Rheumatol ; 80(2): 189-193, 2021 Mar.
Article in German | MEDLINE | ID: mdl-33236165

ABSTRACT

Subacute cutaneous lupus erythematosus (SCLE) is a subtype of cutaneous lupus erythematosus characterized by high photosensitivity, the occurrence of annular or papulosquamous skin lesions located in body regions exposed to UV light, the presence of anti-Ro/SS­A antibodies, and mild systemic involvement, such as arthralgia and myalgia. Similar to other subtypes of cutaneous lupus erythematosus, certain trigger factors exist for the development of SCLE, such as exposure to UV light, cigarette smoking and drugs. Rheumatic diseases, such as dermatomyositis, have been known as paraneoplastic syndromes for a long time. In recent years, there has been an accumulation of publications on the association of SCLE with malignant diseases. This article reports the case of a 78-year-old female patient who was diagnosed with the concomitant development of SCLE and gastric carcinoma. In all older patients with SCLE, patients with widespread skin affection outside the UV-exposed body areas or patients with B­symptoms, the presence of a paraneoplastic SCLE should be considered and appropriate diagnostic steps should be initiated to screen for an associated neoplastic disease.


Subject(s)
Lupus Erythematosus, Cutaneous , Paraneoplastic Syndromes , Rheumatic Diseases , Aged , Autoantibodies , Female , Humans , Lupus Erythematosus, Cutaneous/diagnosis , Paraneoplastic Syndromes/diagnosis , Skin
4.
Internist (Berl) ; 61(6): 621-625, 2020 Jun.
Article in German | MEDLINE | ID: mdl-32367302

ABSTRACT

A patient with diffuse angiokeratomas of the lower abdomen and genital region was diagnosed with Fabry disease on the basis of genetic testing. Fabry disease is an X-linked lysosomal storage disease that can affect several organ systems including the heart or kidneys, resulting in reduced median survival. Pathogenetically, Fabry disease leads to a deficiency of the lysosomal enzyme α­galactosidase A (α-GAL A). Treatment options include lifelong enzyme replacement therapy or chaperone therapy.


Subject(s)
Enzyme Replacement Therapy , Fabry Disease/diagnosis , Fabry Disease/therapy , Abdomen , Adult , Angiokeratoma/pathology , Fabry Disease/genetics , Genitalia , Glycosphingolipids/blood , Humans , Male , Skin Neoplasms/pathology , alpha-Galactosidase/blood
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