fibrin and Angiomatosis

Reactive cutaneous vascular proliferation or angiomatosis is associated with various conditions, but is rarely seen secondary to vascular occlusion. We report an unusual case of a 79-year-old female who presented with 8 month history of purpuric facial plaques, with painful crusted ulceration of the nose, later developing similar eruptions on hands, thighs and trunk. Biopsies showed marked angioproliferation, with intravascular (IV) hyaline deposits (PAS+, fibrin+/-; IgM+, fibrinogen+, and C3+), associated with endothelial hyperplasia (Factor VIII+, Vimentin+). Immunofluorescence showed IV IgM, fibrinogen, and granular C3 deposits within vessel walls. Initially, extensive investigations only showed minimal monoclonal gammopathy of undetermined significance (MGUS) and repeatedly negative cryoglobulins. After a 3-year follow-up, the patient developed chronic lymphocytic leukemia (B-CLL). This case illustrates a difficult diagnostic challenge. Although this condition resembles other forms of reactive angiomatosis, it shows distinct features and should be considered in the differential diagnosis of unusual vascular proliferations of the skin. The cutaneous lesions are also considered a potential marker for an underlying systemic condition, which may require prolonged clinical follow-up. We believe this condition to be related to other rare cutaneous vascular proliferations associated with plasma cell and lymphoproliferative disorders. Furthermore, we suggest a common pathogenetic pathway resulting from the IV immunoglobulin deposits causing vascular injury, finally leading to the angiomatosis.

Topics: Aged; Angiomatosis; Biomarkers; Complement C3; Factor VIII; Female; Fibrin; Fibrinogen; Fluorescent Antibody Technique, Direct; Humans; Immunoglobulin M; Immunoglobulins; Immunohistochemistry; Leukemia, Lymphocytic, Chronic, B-Cell; Monoclonal Gammopathy of Undetermined Significance; Skin; Skin Diseases, Vascular

Topics: Angiomatosis; Argon; Autopsy; Basement Membrane; Erythrocytes; Fibrin; Fluorescein Angiography; Humans; Laser Therapy; Lasers; Leukocytes; Lipid Metabolism; Macrophages; Male; Microscopy, Electron; Middle Aged; Regional Blood Flow; Retina; Retinal Artery; Retinal Pigments; Retinal Vessels; Syndrome; Thrombosis; Time Factors

Topics: Angiomatosis; Anticoagulants; Arm; Biopsy; Blindness; Blood Coagulation Disorders; Capillaries; Chronic Disease; Factor V; Factor VIII; Female; Fibrin; Fibrinolysin; Humans; Leg Dermatoses; Middle Aged; Pain; Prednisolone; Skin Manifestations; Skin Ulcer; Thrombosis

Topics: Africa; Africa, Southern; Angiomatosis; Blood Transfusion; Epistaxis; Fibrin; Fibrin Foam; Genes; Genetics, Medical; Hemostatics; Humans; Iron; Nasal Septum; Skin Transplantation; Telangiectasis