Pigmented purpura and cutaneous vascular occlusion syndromes

Lamadrid-Zertuche, Ana Cecilia; Garza-Rodríguez, Verónica; Ocampo-Candiani, Jorge de Jesús

doi:10.1590/abd1806-4841.20187459

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Review • An. Bras. Dermatol. 93 (3) • May-Jun 2018 • https://doi.org/10.1590/abd1806-4841.20187459 copy

Pigmented purpura and cutaneous vascular occlusion syndromes^* * Work conducted at the Dermatology Department, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Nuevo León, México.

Authorship SCIMAGO INSTITUTIONS RANKINGS

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

Size	Morphology	Characteristics
≤ 4mm = petechiae	Retiform	Inflammatory
5 to 9mm = macule	Non-retiform	Non-inflammatory
≥ 1cm = ecchymosis	Livedo reticularis
	Livedo racemosa

	Pathophysiology	Topography
Livedo reticularis	Low blood flow due to low output state.	Lower extremities
Livedo racemosa	Irregular blood flow due to mechanical obstruction.	Trunk and lower extremities
Retiform purpura	Purpura and necrosis due to venous occlusion.	Variable

	Clinical appearance	Topography	Histology
Schamberg's Purpura	Purpuric macules forming large plaques that acquire a brownish color described as "Cayenne pepper grains"	Lower limbs (pretibial)	Perivascular lymphocytic infiltrate, with erythrocyte extravasation and hemosiderin deposit
Eczematid-like purpura of Doucas and Katepanakis	Eczematous changes with lichenification	Lower limbs
Purpura annularis telangiectodes of Majocchi	Symmetric annular brown-red macules, with a clear atrophic center	Lower limbs
Geugerot and Blum's Disease	lichenified orange-red or purpuric plaques	Legs, thighs, trunk, and occasionally arms
Lichen aureus	Purpuric macules with orange or golden lichenoid papules	Lower limbs	Same characteristics as lichenoid band-like dermal lymphocyte infiltrate
Linear capillaritis	Similar to lichen aureus with linear pattern	Lower limbs	Same as other pigmented purpuras

Cryoglobulinemias	Type I	Type II	Type III
Antibody type	Monoclonal IgM or IgG	Polyclonal IgG and monoclonal IgM, positive RF	Polyclonal IgG and IgM, RF positive
Vascular occlusive mechanism	Hyperviscosity	Immune complex	Immune complex
Comorbidities	Hematological neoplasm (Type B lym- phoproliferative disorders: Waldenstrom's macroglobulinemia, multiple myeloma, monoclonal gammopathy)	Hepatitis C or B, HIV, autoimmune diseases	Autoimmune diseases (Sjogren syndrome, SLE, rheumatoid arthritis), hepatitis C, idiopathic.

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E-mail: revista@sbd.org.br

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[1] Mailing address: Verónica Garza-Rodríguez. Email: verogarzardz@gmail.com

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Pigmented purpura and cutaneous vascular occlusion syndromes* * Work conducted at the Dermatology Department, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Nuevo León, México.

Abstract:

Pigmented purpura and cutaneous vascular occlusion syndromes^* * Work conducted at the Dermatology Department, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Nuevo León, México.