Small vessel vasculitis, cutaneous L95.5

• Classification according to vessel size and type of inflammation: This classification classifies vasculitis according to objectifiable histopathological criteria which take into account the size of the vessels (small, medium and large) and the type and composition of the infiltrate (e.g. the important criterion of leukocytoclasia). "Small vessels" are capillaries, arterioles and venules; "medium-sized vessels" refers to medium-sized arterial and venous vessels and "large vessels" to the aorta and its direct outlets or their branches (e.g. A. temporalis). Even in this primarily histomorphological classification, there are blurs and overlaps, especially when clinical and histological entities are used synonymously. For example, leukocytoclastic vasculitis (LcV) is based on several clinical entities. The common feature of this group is the exclusively histologically detectable pathological substrate, namely vasculitis with the decay of neutrophilic leukocytes, leukocytoclasia. Therefore, this classification is ultimately not satisfactory from a clinical point of view.
• Histopathological and immunological classification and assignment of clinical entities (following Sundkötter). It has been shown that the occurrence of IgA-containing immune complexes (detection by DIF) is of considerable prognostic (rather unfavourable prognosis) and therapeutic importance.
• Cutaneous small vessel vasculitis (CSVV):
• LcV, largely associated with IgA:
  • Purpura Schönlein-Henoch
  • Infantile, acute hemorrhagic edema (age < 2 years)
• LcV (non-IgA-associated)
  • LcV (non-IgA-associated) - with / without systemic involvementUrticarial vasculitis
    :
    • Hypocomplementary urticarial vasculitis.
    • Normocomplementary urticarial vasculitis
    • Serum disease
    • Vasculitis with essential cryoglobulinemia
• Other (special forms):
  • Granuloma eosinophilicum faciei
  • Erythema elevatum diutinum
  • Septic vasculitis
  • Purpura fulminans
  • LcV with eosinophilia (entity in question)
  • LcV in collagenosis (SLE, scleroderma).

S. Vasculitis