Other Purpura Syndromes

Description / Epidemiology
  • Benign condition
  • F > M
  • Primary (in younger pts) or secondary
  • A/w autoimmune connective tissue disease (Sjögren > LE / RA): Anti-Ro (SSA) & anti-La (SSB) often present = ↑ risk of dvlp disease
  • Rarely, monoclonal gammopathy & very rarely, lymphoma or multiple myeloma
Pathogenesis
  • Presence of immune complexes IgG or IgA rheumatoid factor (RF) = highly soluble thus lesions appear and resolve quickly
Location
  • Lower extremities
Clinical features
  • Crops of petechiae / larger purpuric macules, +/- burning
  • +/- palpable purpura
  • Worse w/tight clothing, heat and prolonged standing
  • Labs: polyclonal hypergammaglobulinema, ↑ ESR
Pathology
  • Hemorrhage, perivascular lymphocytic infiltrate, leukocytoclastic vasculitisTreatment
Treatment
  • Avoid triggers (long standing, alcohol)
  • Aspirin / support stockings → exacerbate disease
  • Tx underlying causes
  • +/- Colchicine

Description / Epidemiology
  • Factitious disorder
  • F > M
  • A/w psychiatric diseases (depression, anxiety, hypochondriasis, sexual maladjustments, masochism, borderline personality disorder, obsessive-compulsive behavior)
Location
  • Any area (atypical locations)
Clinical features
  • Painful ecchymoses at site of trauma, variable sizes
  • Atypical morphology
  • No precipitating factor
Pathology
  • Edema, minimal perivascular infiltrate, extravasated RBC
Treatment
  • Difficult → avoid confronting pt
  • Address possibility of underlying psychiatric disease + refer for psychiatric tx

Description / Epidemiology
  • Superficial thrombophlebitis of s/c vessels in anterolateral thoracoabdominal wall
  • 3 F > 1 M
  • Paraneoplastic: 10% pts w/breast carcinoma
  • Other associations: RA, filariasis, pregnancy, OCP, IV drug use, IV catheters, non-breast cancer, hypercoagulable state
Pathogenesis
  • Thrombophlebitis due to: trauma, excessive physical activity, breast surgery, mastitis, breast abscess
Location
  • Trunk
Clinical features
  • Acute onset of chest pain → then, unilateral cord-like thrombosed vein
Pathology
  • Bx rarely needed.
  • Sclerosing endophlebitis w/ +/- complete occlusion by thrombus & inflammatory cells
Treatment
  • Benign & self-limited (except if underlying breast carcinoma): pain d/c in 10 days & cord d/c in weeks-months
  • Local heat, rest of arm, breast support → improve Sx