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Eff88c923ae51b04c203b0aa7ad REVIEW 397 s Pigmented purpura and cutaneous vascular occlusion syndromes* Ana Cecilia Lamadrid-Zertuche1 Verónica Garza-Rodríguez1 Jorge de Jesús Ocampo-Candiani1 DOI: http://dx.doi.org/10.1590/abd1806-4841.20187459 Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes. Keywords: Antiphospholipid syndrome; Calciphylaxis; Myeloproliferative disorders; Purpura; Purpura fulminans; Vascular diseases INTRODUCTION Purpura is defined as a visible hemorrhage in the skin or TABLE 1: Purpura classification mucosa that is not evanescent upon pressure. Proper classification Size Morphology Characteristics provides a better patient approach due to the multiple diagnoses of purpura. Purpuras can be classified by size, morphology, pathophy- ≤ 4mm = petechiae Retiform Inflammatory siology, and other characteristics (Table 1). 5 to 9mm = macule Non-retiform Non-inflammatory Regarding morphology, retiform purpura should be diffe- ≥ 1cm = ecchymosis Livedo reticularis rentiated from livedo reticularis and livedo racemosa. These conditions Livedo racemosa have a similar morphological appearance, characterized as viola- Source: Piette WW, 2012.1 ceous macules in a net-like, arborized, or starry form but they differ in pathophysiology (Chart 1). The clinical course varies according to the etiology. Macular CHART 1: Livedo reticularis, livedo racemosa, and retiform purpu- purpura or non-palpable purpura heals faster and exhibits a col- ra characteristics or transition from red-blue to violaceous, green, yellow, or brown Pathophysiology Topography due to extravasation of erythrocytes and few inflammatory cells.1 Palpable purpura takes longer to heal due to the presence of inflam- Livedo reticularis Low blood flow due Lower extremities to low output state. matory cells and immune complex deposits that lead to vascular occlusion.1 Livedo racemosa Irregular blood flow Trunk and lower due to mechanical extremities Purpura has a long list of differential diagnoses, which obstruction. differ particularly in their pathophysiology. One way to approach Retiform purpura Purpura and necro- Variable purpura is by answering the question, “Is the patient bleeding?”, sis due to venous since some bleeding disorders require urgent treatment (Figure 1). occlusion. The purpose of this review is to describe some of the differential 16 diagnoses and their physiopathogenic mechanisms to provide a bet- Source: Wysong A, et al, 2011. Received 04 August 2017. Accepted 03 November 2017. * Work conducted at the Dermatology Department, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Nuevo León, México. Financial support: None. Conflict of interest: None. 1 Dermatology Department, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Nuevo León, México. MAILING ADDRESS: Verónica Garza-Rodríguez Email: [email protected] ©2018 by Anais Brasileiros de Dermatologia An Bras Dermatol. 2018;93(3):397-404. 398 Lamadrid-Zertuche AC, Garza-Rodríguez V, Ocampo-Candiani JJ Purpura Is the patient bleeding? Yes No Coagulation Pigmented Vascular occlusive Vessel wall Thrombocytosis Thrombocytopenia Vasculitis Asprted entities disorder purpura syndromes support problem Embolism Increased Myeloproliferative Small, medium (cholesterol, Emergency Other Congenital Acquired Senile purpura intravascular disorder and larg vessel crystal, fat, infective, myxoma) pressure NPH APS Dilution Hemophilia A, B warfarin necrosis Calcyphylaxis Scurvy Child abuse Purpura Von willebrand`s Myeloproliferative TTP (rare) Catheter Infection Amyloidosis fulminans (sepsis) disease disorders Trauma Factor v and Drugs (aspirin, Spider bite protein C Hepatic failure Valsalva TTP clopidogrel, ab- (Loxoceles) Ehrles-Danlos ciximab, NSAID, deficiency maneuver antibiotics, fibronolitics, etc. Connective tissue HIT Afibrinogenemia Renal failure Crioaglutination Drugs disease (APS) Infective organisms Gardner-Diamond Vitamin K (immunosupressed ITP syndrome deficiency patients, pyoderma gangrenosum, (psychogenic) Lucios, Leprosy, etc.) DIC Marantic endocarditis Procoagulant dilution Mondor disease Drugs NPH: nocturnal paroxysmal hemoglobinuria; TTP: thrombotic thrombocytopenic purpura; APS: antiphospholipid syndrome; HIT: heparin-induced thrombocytopenia; ITP: ldiopathic thrombocytopenic purpura; NSAID: non-steroidal anti-inflammatory drugs; DIC: disseminated intravascular coagulation FIGURE 1: Purpura: diagnostic algorithm ter patient approach and guide treatment. This article will discuss hyperactive substances such as textiles, colorants, and alcohol.2,3 Id- pigmented purpuras and some cutaneous vascular occlusion syn- iopathic capillaritis is the most common form, since most cases are dromes. Although vasculitis is part of the differential diagnoses, it not associated with a specific trigger.4 will not be discussed in this review. Epidemiological data are lacking. However, a 5-fold in- creased prevalence is noted in men compared to women. The con- Pigmented purpuras dition predominantly affects adults 40 to 60 years of age.2,5 Some Pigmented purpuras, also known as chronic pigmented variants predominate in children and young adults.3-5 purpuric dermatosis, purpura simplex, and capillaritis, among oth- The physiopathogenesis is unknown, but the condition is ers, encompasses five major clinical variants, including Schamberg’s believed to be due to a cutaneous hypersensitivity reaction that purpura, purpura annularis telangiectodes of Majocchi, pigment- causes capillary fragility and permeability, leading to erythrocyte ed purpuric lichenoid dermatitis of Gougerot and Blum, eczema- extravasation and hemosiderin deposits noted on biopsy.1,2,6 The tid-like purpura of Doucas and Kapetanakis, and lichen aureus.1-4 following three main pathogenic theories have been described ac- Each condition is associated with different triggers, which are diffi- cording to histological data: vascular fragility, humoral immunity, cult to establish in practice and do not appear to have therapeutic or and cellular immunity.2,3 prognostic implications.3,4 Pigmented purpuras present as petechiae or pigmented ma- Capillaritis is associated with many triggers, including ve- cules on distal lower extremities.1-4 These lesions can be generalized, nous hypertension, exercise, pregnancy, frail capillaries, drug tox- due to self-limiting viral infection, or localized. All variants have icity from acetaminophen, aspirin, hydralazine, and thiamine, and specific distinguishing clinical characteristics (Chart 2). An Bras Dermatol. 2018;93(3):397-404. Pigmented purpura and cutaneous vascular occlusion syndromes 399 CHART 2: Pigmented purpuras Clinical appearance Topography Histology Schamberg’s Purpura Purpuric macules forming large plaques Lower limbs Perivascular lymphocytic infiltrate, that acquire a brownish color described (pretibial) with erythrocyte extravasation and as “Cayenne pepper grains” hemosiderin deposit Eczematid-like purpura of Eczematous changes with lichenification Lower limbs Doucas and Katepanakis Purpura annularis telangiec- Symmetric annular brown-red macules, Lower limbs todes of Majocchi with a clear atrophic center Geugerot and Blum’s Disease lichenified orange-red or purpuric Legs, thighs, plaques trunk, and occa- sionally arms Lichen aureus Purpuric macules with orange or golden Lower limbs Same characteristics as lichenoid lichenoid papules band-like dermal lymphocyte infiltrate Linear capillaritis Similar to lichen aureus with linear Lower limbs Same as other pigmented purpuras pattern Source: Díaz Molina VL et al., 2009 2; Sardana K et al., 2004 3; Allevato MA, 2007 4; Karadag AS et al., 2013 5, and Hoesly FJ et al,, 2009. 7 Schamberg’s purpura disease affects adults between 40 and 60 years of age.2 The condition This condition is also known as progressive or chronic pur- is characterized by lichenified plaques with an orangish-red or pur- puric dermatosis. Schamberg’s purpura predominates in men in plish color on the anterior regions of lower extremities (lower legs their 50s and is associated with viral infections. 2 The condition typ- and thighs), trunk, and occasionally arms.2, 4,8 When single, a lesion ically involves the pretibial region and extends proximally, sparing can mimic Kaposi’s sarcoma.2,4 Some cases may be associated with the face, palmo-plantar regions. 3 Schamberg’s purpura typically mycosis fungoides.2,4 presents as purpuric macules forming large plaques that acquire a brownish color described as “Cayenne pepper grains”.2,3 The lesions Lichen aureus tend to be asymptomatic but are sometimes mildly pruritic.3 Also called purpuric lichen, lichen aureus is named for to its golden color.3 Affecting young adults between 20 and 30 years Eczematid-like Purpura of Doucas and Katepanakis of age, it is characterized by typically unilateral orange or golden Eczematid-like purpura or itching purpura is described as purpuric macules with lichenoid papules on the lower extremi- a variant of Schamberg’s purpura 3, 5 that typically affects men.5 The ties.2-4 The lesions are often chronic, lasting between 3 months and condition is characterized by
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