CUTANEOUS NECROTIZING VENULITIS - SEQUENTIAL-ANALYSIS OF MORPHOLOGICAL ALTERATIONS OCCURRING AFTER MAST-CELL DEGRANULATION IN A PATIENT WITH A UNIQUE SYNDROME

  • 1 January 1978
    • journal article
    • research article
    • Vol. 32  (1) , 46-58
Abstract
An unusual patient, with dermal nodules, flexion contractures of the fingers and toes, cold-induced urticaria, dermographism and serum hypocomplementemia, had necrotizing cutaneous venulitis underling the spontaneous lesions. Since necrotizing cutaneous venulitis could be experimentaly induced by the physical stimuli of cold or trauma, the time-course of histopathological events was documented in the skin of this patient. The histopathological alterations were studied in 1 .mu.m thick, Epon-embedded skin biopsy specimens over an interval of 6 days. The early massive degranulation of the mast cells was followed by the sequential infiltration of neutrophilic, eosinophilic and basophilic polymorphonuclear leukocytes, by the development of venular endothelial cell necrosis and by the deposition of fibrin. The persistent serum hypocomplementemia involved the classic activating and amplification pathways. It seems possible that the unusual combination of pathobiological processes involving the mast cells and the complement system in this patient has created a unique syndrome, in which venules are damaged and the sheaths of the extensor tendons of the hands and feet become affected in time.