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purpura; retiform purpura

A condition characterized by hemorrhage into the skin &/or mucus membranes, resulting in patches of purplish discoloration, petechiae or ecchymosis. Retiform purpura describes the netlike configuration of leakage from the skin vasculature. [3] Etiology: 1) primary cutaneous disorders - non palpable - trauma including domestic violence, sexual abuse - solar purpura - steroid purpura - capillaritis (pigmented purpura) - livedoid vasculitis 2) systemic diseases a) non palpable - clotting disorders - thrombocytopenia (including ITP) - abnormal platelet function - coagulation disorder - vitamin K deficiency - hemophilia - hematologic malignancy - vascular fragility - amyloidosis - Ehlers-Danlos syndrome - scurvy (vitamin C deficiency) - thrombi - disseminated intravascular coagulation (DIC) - monoclonal cryoglobulinemia - thrombotic thrombocytopenic purpura - antiphospholipid antibody syndrome - thrombophilia - myeloproliferative disorders - warfarin reaction - emboli - cholesterol - fat - immune complex - Gardner-Diamond syndrome (autoerythrocyte sensitization) - Waldenstrom's macroglobulinemia b) palpable purpura - vasculitis [3] - polyarteritis nodosa - leukocytoclastic vasculitis - Henoch-Schonlein purpura - eosinophilic granulomatosis with polyangiitis - Wegener's granulomatosis* - microscopic polyangiitis* - systemic lupus erythematosus - rheumatoid arthritis* - dermatomyositis* - mixed cryoglobulinemia - scleroderma [3] - emboli - acute meningiococcemia - disseminated gonococcal infection - rickettsial - rocky mountain spotted fever - epidemic typhus - ecthyma gangrenosum - fat emboli, septic emboli, cholesterol emboli - marantic endocrditis [3] - infectious (also see emboli) - hepatitis - atypical measles - echovirus 9 - TORCH group - Staphylococcus - Pseudomonas sepsis - bacterial endocarditis - HIV1 infection - angioinvasive fungal infection 3) pharmacologic causes (hypersensitivity): a) allopurinol b) ampicillin c) aspirin d) glucocorticoids e) warfarin (skin necrosis) f) heparin (skin necrosis, rare) 7) quinidine g) levamisole-contaminated cocaine - tender purpura on ears is prominent 4) miscellaneous causes a) mechanical increases in capillary pressure - cough - seizures - labor of pregnancy, miscarriage b) actinic purpura (senile purpura) c) orthostatic purpura (occurs in lower extremities after prolonged standing d) purpura simplex e) factitious or self-inflicted f) necrotizing fasciitis g) calciphylaxis h) intravascular procedure i) idiopathic * uncertain that purpura is palpable (from [3]) Epidemiology: more common in women than men Pathology: 1) extravasation of erythrocytes from cutaneous vessels into the surrounding dermis 2) with palpable purpura, extravasation occurs from inflamed & damaged vessels superficial to mid-dermal vessels Clinical manifestations: 1) non-pruritic, non-blanching red macules [3] 2) hypersensitivity purpura - appearance is variable - may or may not be palpable - may be bullous - symmetric in distribution - generally involves proximal extremities, legs & buttocks - pruritus may be present - no generalized bleeding - appears & regresses in crops 3) thrombocytopenic purpura - petechiae - superficial ecchymoses - may occur anywhere on body, but dependent areas & constriction or pressure points especially at risk - generalized bleeding from mucosa 4) ecchymoses suggest coagulation defect or senile purpura 5) palpable purpura suggests inflammation or occlusion of dermal vessels - small-vessel vasculitis - hypercoagulable state 6) scurvy - petechiae around hair follicles - ecchymoses & large subcutaneous hematomas - frequently saddle distribution involving thighs & buttocks - lassitude - generalized bleeding - painful periosteal hemorrhage in children * images [7] Laboratory: 1) complete blood count (CBC) & peripheral smear 2) anemia workup if indicated a) serum iron, TIBC, ferritin b) serum vitamin B12 c) serum folate 3) erythrocyte sedimentation rate (ESR) 4) urinalysis: hematuria with or without proteinuria may be sign of vasculitis 5) serum chemistries a) serum creatinine b) serum creatine kinase c) liver function tests 6) disseminated intravascular coagulation (DIC) panel a) prothrombin time (PT) b) activated partial thromboplastin time (aPTT) c) plasma fibrinogen d) D-dimer 7) anti-platelet antibodies 8) anti-nuclear antibodies (ANA) 9) rheumatoid factor (RF) 10) cryoglobulins 11) hepatitis panel 12) serum protein electrophoresis (SPE) 13) immunofixation electrophoresis (IFE) 14) capillary fragility (Rumpel-Leede) test 14) skin biopsy 15) bone marrow biopsy Radiology: 1) chest radiograph: infiltrates may be sign of vasculitis 2) electrocardiogram a) pericarditis b) coronary vasculitis c) consider transesophageal echocardiogram if suspecting embolic cause Differential diagnosis: - cutaneous T-cell lymphoma Management: 1) general - 50% of cases idiopathic & self-limited - drugs are most frequent identifiable cause - stop offending agent(s) - prednisone 1-2 mg/kg/day for 4-6 weeks with slow taper is the treatment of choice while investigating etiology 2) specific therapies - low potency topical glucocorticoid for benign capillaritis - antihistamines for urticarial vasculitis - non-steroidal anti-inflammatory agents - ascorbic acid & vitamin K - danocrine 200 mg PO TID-QID - immunosuppressive agents - plasmapheresis - alpha-interferon - platelet transfusions are only transiently helpful 3) consider dermatology consult for palpable purpura [3]

Related

ecchymosis petechiae

Specific

actinic purpura; solar purpura; senile purpura inflammatory purpura palpable purpura pinch purpura, periorbital purpura post transfusion purpura progressive pigmentary dermatosis; Schamberg's purpura; pigmented purpuric dermatosis; purpura annularis telangiectodes of Majocchi; cutaneous T-cell lymphoid dyscrasia; capillaritis (CTLD) purpura simplex retiform purpura

General

skin lesion sign/symptom hemorrhage (bleeding)

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 305
  2. Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 913-916
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
  4. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 326
  5. Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: an overview. Int J Dermatol. 2004 Jul;43(7):482-8. PMID: 15230884
  6. Wysong A, Venkatesan P. An approach to the patient with retiform purpura. Dermatol Ther. 2011 Mar-Apr;24(2):151-72 PMID: 21410606
  7. DermNet NZ. Purpura (images) http://dermnetnz.org/vascular/purpura.html