purpura; retiform purpura
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Introduction
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
- primary cutaneous disorders
- non palpable
- trauma including domestic violence, sexual abuse
- solar purpura
- steroid purpura
- capillaritis (pigmented purpura)
- livedoid vasculitis
- non palpable
- systemic diseases
- non palpable
- clotting disorders
- thrombocytopenia (including ITP)
- abnormal platelet function
- coagulation disorder
- hematologic malignancy
- vascular fragility
- thrombi
- emboli
- cholesterol
- fat
- immune complex
- Gardner-Diamond syndrome (autoerythrocyte sensitization)
- Waldenstrom's macroglobulinemia
- clotting disorders
- palpable purpura
- vasculitis[3]
- emboli
- infectious (also see emboli)
- non palpable
- pharmacologic causes (hypersensitivity):
- quinidine
- levamisole-contaminated cocaine
- tender purpura on ears is prominent
- levamisole-contaminated cocaine
- miscellaneous causes
- mechanical increases in capillary pressure
- actinic purpura (senile purpura)
- orthostatic purpura (occurs in lower extremities after prolonged standing
- purpura simplex
- factitious or self-inflicted
- necrotizing fasciitis
- calciphylaxis
- intravascular procedure
- idiopathic
* uncertain that purpura is palpable (from[3])
Epidemiology
more common in women than men
Pathology
- extravasation of erythrocytes from cutaneous vessels into the surrounding dermis
- with palpable purpura, extravasation occurs from inflamed & damaged vessels superficial to mid-dermal vessels
Clinical manifestations
- non-pruritic, non-blanching red macules[3]
- hypersensitivity purpura
- thrombocytopenic purpura
- petechiae
- superficial ecchymoses
- may occur anywhere on body, but dependent areas & constriction or pressure points especially at risk
- generalized bleeding from mucosa
- ecchymoses suggest coagulation defect or senile purpura
- palpable purpura suggests inflammation or occlusion of dermal vessels
- 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
- complete blood count (CBC) & peripheral smear
- anemia workup if indicated
- erythrocyte sedimentation rate (ESR)
- urinalysis: hematuria with or without proteinuria may be sign of vasculitis
- serum chemistries
- disseminated intravascular coagulation (DIC) panel
- anti-platelet antibodies
- anti-nuclear antibodies (ANA)
- rheumatoid factor (RF)
- cryoglobulins
- hepatitis panel
- serum protein electrophoresis (SPE)
- immunofixation electrophoresis (IFE)
- capillary fragility (Rumpel-Leede) test
- skin biopsy
- bone marrow biopsy
Radiology
- chest radiograph: infiltrates may be sign of vasculitis
- electrocardiogram
- pericarditis
- coronary vasculitis
- consider transesophageal echocardiogram if suspecting embolic cause
Differential diagnosis
Management
- 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
- 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
- consider dermatology consult for palpable purpura[3]
More general terms
More specific terms
- 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
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 305
- ↑ Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 913-916
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 326
- ↑ Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: an overview. Int J Dermatol. 2004 Jul;43(7):482-8. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15230884
- ↑ Wysong A, Venkatesan P. An approach to the patient with retiform purpura. Dermatol Ther. 2011 Mar-Apr;24(2):151-72 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21410606
- ↑ 7.0 7.1 DermNet NZ. Purpura (images) http://dermnetnz.org/vascular/purpura.html