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A 73-year-old man with left parotid gland swelling over 2 months was referred to our hospital in March 201X. Purpura on the lower legs had been recurrent for >20 years. Biopsy of the parotid gland demonstrated diffuse infiltration of abnormal lymphocytes that were negative for CD10 and positive for CD19, CD20, and κ-chain. The Ki-67 positivity was <10%; lymphoepithelial lesions were observed. The patient was diagnosed with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Chromosome analysis revealed t (X;14) (p11.2;q32), and fluorescence in situ hybridization (FISH) of metaphase spreads showed three signals of the immunoglobulin heavy chain (IGH) gene on the derivative chromosomes X and 14, besides the normal chromosome 14. CT findings of parotid glands were suggestive of Sjogren syndrome, and biopsy of the purpura on the leg demonstrated leukocytoclastic vasculitis. In the literature, only seven patients with lymphoma and t (X;14) translocation have been reported. Of these, five patients had MALT lymphoma, one had nodal marginal zone lymphoma, and one had diffuse large B-cell lymphoma. In all patients, lymphoma evolved from previous autoimmune diseases. It is suggested that MALT lymphoma with the t (X;14) translocation forms a new entity of lymphoma.

A 62 year-old man had fever, splenomegaly, systemic lymphadenopathy and palpable petechiae. Laboratory studies showed leukocytosis (10,200/microliters) with plasma cells (23%) and polyclonal gammopathy. Southern blot analysis of peripheral blood mononuclear cells showed no rearrangement of immunoglobulin genes, indicating polyclonal plasmacytosis in peripheral blood. A diagnosis of Castleman's disease was made by the cervical lymph node biopsy, which showed onion-skin structures in the germinal centers and plasmacytosis in the interfollicular region. A skin biopsy of the purpura lesion disclosed leukocytoclastic vasculitis. This is an unusual case of Castleman's disease presenting plasmacytosis in peripheral blood and leukocytoclastic vasculitis.

Pathogenesis and treatment of hypersensitivity angiitis and Henoch-Schönlein purpura were summarized. Both diseases were included in the category of "leukocytoclastic vasculitis". Hypersensitivity angiitis was frequently associated with drug or infectious exposure and the involvement of venules and capillaries. Many patients with this disease have detectable autoantibodies to neutrophil cytoplasmic antigens (ANCA) typically reactive with myeloperoxidase. Henoch-Schönlein purpura was characterized by vasculitis in multiple organs such as the skin, joints, gastrointestinal tract and kidneys. Henoch-Schönlein purpura nephritis revealed marked deposition of IgA (mainly IgA1) and C3 in the glomerular mesangial areas and capillary walls by immunofluorescence. Combinations of intensive plasma exchange, steroids and cyclophosphamide were effective for patients with both diseases.

Vasculitis has a simple definition--primary inflammation of the blood vessels, often with necrosis and occlusive changes--but its clinical manifestations are diverse and complex. In the absence of pathognomonic clinical features and laboratory tests, the diagnosis of vasculitis relies heavily on the correct interpretation of histologic changes but these too may not be specific in any given case. A positive biopsy is always helpful whereas a negative biopsy does not exclude the disease under consideration because the vasculitis may be focal and segmental in distribution, as in polyarteritis nodosa. Pathologic and immunohistochemical characteristics of some of the major categories of systemic vasculitis were described; 1) polyarteritis nodosa; 2) Churg-Strauss syndrome; 3) hypersensitivity vasculitis; 4) Wegener's granulomatosis; 5) Kawasaki disease; 6) malignant rheumatoid arthritis; 7) systemic lupus erythematosus; 8) scleroderma; 9) polymyositis-dermatomyositis; 10) mixed connective tissue disease; 11) polyangiitis overlap syndrome; 12) giant cell arteritis; 13) Takayasu arteritis; 14) Behçet disease; 15) Buerger disease; and 16) inflammatory aortic aneurysm. Each of these major vasculitic syndromes has its own unique histopathologic features, but overlap still occurs.

Cutaneous vasculitis is classified into the vasculitis of which vascular lesions are confined to the skin tissues and the vasculitis of which cutaneous lesions are one aspect of systemic vasculitis. The symptoms vary depending on the size of the affected blood vessel, degree of inflammation and its course and include urticarial erythema, palpable purpura, livedo sign, papule, bulla, nodule and ulceration. In this article, several clinical and histopathological findings of cutaneous vasculitis such as urticarial vasculitis, vasculitis allergica cutis, polyarteritis nodosa cutanea are described.