Retiform purpura

Retiform purpura
Retiform purpura
Specialty	Dermatology
Symptoms	persistent dark red to dark purple hemorrhagic skin patches or plaques
Causes	vessel wall damage (vasculitis/depositional disease/angioinvasion by organism) or vessel lumen occlusion (thrombotic or embolic disease)
Treatment	Underlying cause and would care including circulatory support

Last updated December 21, 2024

Retiform purpura is the result of total vascular blockage and damage to the skin's blood vessels. The skin then shows lesions, appearing due to intravascular issues where clots, proteins, or emboli block skin vessels. They can also result from direct harm to the vessel walls, as seen in conditions like vasculitis, calciphylaxis, and certain severe opportunistic infections.

Pathophysiology

Retiform purpura is a cutaneous morphology characterized by a branching (reticular), non-blanching (purpuric) patch, plaque, or lesion that develops when blood vessels supplying the skin become obstructed.^[2]^[3] This blockage leads to downstream cutaneous ischemia, or insufficient blood supply to the skin, causing purpura, necrosis, and potentially ulceration.^[3] The obstruction in the dermal and subcutaneous vasculature results in hemorrhage secondary to skin ischemia, which can further lead to skin necrosis and ulceration.^[2]^[3]^[4] This morphology can occur in a variety of disorders.^[5]

Signs and symptoms

Retiform purpura can present as non-raised large patches of purpura, with angulated or net-like border. They can present as painful dark red or purple patches or plaques with bordering branching and central purpura, necrosis, and/or ulceration. They can vary in size from small (1–2 cm) to large (>10 cm), and may be single or multiple, localized or disseminated. The branching may only be seen at the edge of one or two lesions but is still the clue to this diagnosis and a potentially serious underlying condition.^[2] Inflammatory retiform purpura consists of redness around the lesion with associated central necrosis caused by vasculitic or infectious processes. Noninflammatory retiform purpura refers to lesions with central necrosis with surrounding redness caused by the occlusive process. There can be significant overlap between these two classifications. Pain within lesions is common and may be severe.^{[ citation needed ]}

Associated disorders

There are several vasculopathy disorders that can present with retiform purpura.^[3] Some thrombotic and coagulopathic disorders are disseminated intravascular coagulation (DIC),^[6] hypercoagulable states (like antiphospholipid antibodies), thrombotic thrombocytopenic purpura (TTP), warfarin-induced skin necrosis, heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria. Some intravascular protein deposition diseases causing retiform purpura are cryoglobulinemia (type I), cryofibrinogenemia, and paraproteinemia. Some embolic disorders that may lead to retiform purpura include cholesterol emboli, septic emboli, atrial myxoma, or other manifestations like nonbacterial thrombotic endocarditis (marantic endocarditis, Libman-Sacks endocarditis) and hypereosinophilic syndrome. Some vessel wall pathology resulting in retiform purpura could be vasculitis, septic vasculitis, vasculitis related to autoimmune disease or primary systemic vasculitis, or cryoglobulinemic vasculitis (cryoglobulinemia types 2 and 3).^[3]

Other manifestation of retiform purpura can be seen in opportunistic infections, calciphylaxis, primary hyperoxaluria, livedoid vasculopathy, pyoderma gangrenosum, and vasculopathy or vasculitis due to levamisole-contaminated cocaine.^[4]

The onset of widespread purpura, known as purpura fulminans, is a dangerous condition associated with disseminated intravascular coagulation (DIC). Determining the root cause of DIC is crucial for treatment. In severe opportunistic infections, retiform purpura can also emerge. It is vital to quickly assess patients showing signs of purpura fulminans, especially if they exhibit symptoms of sepsis, serious sickness, or have weakened immune systems.^[6]

Patient assessment and management

A detailed history and physical examination are essential for patients with retiform purpura, as they can offer significant insights for diagnosis. Specifically, evaluating the skin's color and the spread of purpura is crucial. Skin biopsies of retiform purpura can be instrumental in diagnosing suspected retiform purpura, specifically a punch or an excisional wedge biopsy. If infection is suspected, a tissue culture with initial lab workup.^{[ citation needed ]}

Because the disease manifests in several ways, it is crucial to obtain the underlying etiology when determining treatment plans.^{[ citation needed ]}

Related Research Articles

Disseminated intravascular coagulation (DIC) is a condition in which blood clots form throughout the body, blocking small blood vessels. Symptoms may include chest pain, shortness of breath, leg pain, problems speaking, or problems moving parts of the body. As clotting factors and platelets are used up, bleeding may occur. This may include blood in the urine, blood in the stool, or bleeding into the skin. Complications may include organ failure.

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage. Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their own are separate entities.

Purpura is a condition of red or purple discolored spots on the skin that do not blanch on applying pressure. The spots are caused by bleeding underneath the skin secondary to platelet disorders, vascular disorders, coagulation disorders, or other causes. They measure 3–10 mm, whereas petechiae measure less than 3 mm, and ecchymoses greater than 1 cm.

<span class="mw-page-title-main">Cryoglobulinemia</span> Presence of cold-sensitive antibodies in the blood

Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells.

A schistocyte or schizocyte is a fragmented part of a red blood cell. Schistocytes are typically irregularly shaped, jagged, and have two pointed ends.

Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by reduction in blood flow (ischemia) through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration (cyanosis). This can be a secondary effect of a condition that increases a person's risk of forming blood clots (thrombosis), including a wide array of pathological and nonpathological conditions. Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.

Protein C deficiency is a rare genetic trait that predisposes to thrombotic disease. It was first described in 1981. The disease belongs to a group of genetic disorders known as thrombophilias. Protein C deficiency is associated with an increased incidence of venous thromboembolism, whereas no association with arterial thrombotic disease has been found.

<span class="mw-page-title-main">Calciphylaxis</span> Painful, necrotic skin lesions associated with chronic kidney disease

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome characterized by painful skin lesions. The pathogenesis of calciphylaxis is unclear but believed to involve calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and eventual death of skin cells due to lack of blood flow. It is seen mostly in people with end-stage kidney disease but can occur in the earlier stages of chronic kidney disease and rarely in people with normally functioning kidneys. Calciphylaxis is a rare but serious disease, believed to affect 1-4% of all dialysis patients. It results in chronic non-healing wounds and indicates poor prognosis, with typical life expectancy of less than one year.

Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation.

Loxoscelism is a condition occasionally produced by the bite of the recluse spiders. The area becomes dusky and a shallow open sore forms as the skin around the bite dies (necrosis). It is the only proven type of necrotic arachnidism in humans. While there is no known therapy effective for loxoscelism, there has been research on antibiotics, surgical timing, hyperbaric oxygen, potential antivenoms and vaccines. Because of the number of diseases that may mimic loxoscelism, it is frequently misdiagnosed by physicians.

Rheumatoid vasculitis is a skin condition that is a typical feature of rheumatoid arthritis, presenting as peripheral vascular lesions that are localized purpura, cutaneous ulceration, and gangrene of the distal parts of the extremities.

Necrotizing vasculitis, also called systemic necrotizing vasculitis, is a general term for the inflammation of veins and arteries that develops into necrosis and narrows the vessels.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which a person's blood plasma is allowed to cool substantially, causing the (reversible) precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins.

<span class="mw-page-title-main">Cutaneous small-vessel vasculitis</span> Inflammation of small blood vessels, accompanied by skin bumps

Cutaneous small-vessel vasculitis (CSVV) is inflammation of small blood vessels, usually accompanied by small lumps beneath the skin. The condition is also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis,

Progressive vaccinia is a rare cutaneous condition caused by the vaccinia virus, characterized by painless but progressive necrosis and ulceration.

Livedoid vasculopathy (LV) is an uncommon thrombotic dermal vasculopathy that is characterized by excruciating, recurrent ulcers on the lower limbs. Livedo racemosa, along with painful ulceration in the distal regions of the lower extremities, is the characteristic clinical appearance. It heals to form porcelain-white, atrophic scars, also known as Atrophie blanche.

Microvascular occlusion refers to conditions that can present with retiform purpura. It has been suggested that phenylephrine may be a cause.

Levamisole induced necrosis syndrome (LINES) is a complication of adulterated cocaine recognized in 2011, caused by the use of levamisole as a cutting agent for cocaine. Spontaneous bruising of the earlobes is considered characteristic of this condition, but lesions can present anywhere on the body.

<span class="mw-page-title-main">Palpable purpura</span> Type of firm, hemorrhagic skin lesion, usually asymptomatic

Palpable purpura is characterized by firm, elevated hemorrhagic plaques or papules that can measure several centimeters in diameter. These are typically found on dependent surfaces, like the back of a recumbent patient or the lower legs. The center of a lesion may become ulcerative, pustular, vesicular, necrotic, or nodular. They tend to be asymptomatic, but when nodular or ulcerative, they can become tender. Palpable purpura is the most common cutaneous lesion among individuals with inflammatory vascular injury, whereas nonpalpable purpura typically indicates bleeding caused by a platelet or coagulation disorder.

References

↑ Georgesen, Corey; Fox, Lindy P.; Harp, Joanna (April 2020). "Retiform purpura: A diagnostic approach". Journal of the American Academy of Dermatology. 82 (4): 783–796. doi:10.1016/j.jaad.2019.07.112. PMID 31479689. S2CID 201831828.
1 2 3 Jones, Leah (November 2020). "Retiform Purpura". DermNet . Retrieved 25 October 2023.
1 2 3 4 5 Georgesen, C; Fox, LP; Harp, J (April 2020). "Retiform purpura: A diagnostic approach". Journal of the American Academy of Dermatology . 82 (4): 783–796. doi:10.1016/j.jaad.2019.07.112. PMID 31479689. S2CID 201831828 . Retrieved 25 October 2023.
1 2 Wysong, A; Venkatesan, P (March 2011). "An approach to the patient with retiform purpura". Dermatologic Therapy . 24 (2): 151–172. doi: 10.1111/j.1529-8019.2011.01392.x . PMID 21410606. S2CID 10099582 . Retrieved 25 October 2023– via Wiley.
↑ Tan, Cheng; Jing, Hong-Mei; Zhang, Li-Tao; Ma, Dong-Lai; Zhang, Ru-zhi (2018), Zhu, Wen-Yuan; Tan, Cheng; Zhang, Ru-zhi (eds.), "Cutaneous Vascular Diseases", Atlas of Skin Disorders: Challenging Presentations of Common to Rare Conditions, Singapore: Springer, pp. 189–196, doi:10.1007/978-981-10-8037-1_14, ISBN 978-981-10-8037-1 , retrieved 28 January 2024
1 2 Levi, Marcel (January 2004). "Current understanding of disseminated intravascular coagulation". British Journal of Haematology . 124 (5): 567–576. doi:10.1046/j.1365-2141.2003.04790.x. PMID 14871243. S2CID 12700686 . Retrieved 25 October 2023– via Wiley.

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[Review-Georgesen-1] Georgesen, Corey; Fox, Lindy P.; Harp, Joanna (April 2020). "Retiform purpura: A diagnostic approach". Journal of the American Academy of Dermatology. 82 (4): 783–796. doi:10.1016/j.jaad.2019.07.112. PMID 31479689. S2CID 201831828.

[:0-2] 1 2 3 Jones, Leah (November 2020). "Retiform Purpura". DermNet . Retrieved 25 October 2023.

[:1-3] 1 2 3 4 5 Georgesen, C; Fox, LP; Harp, J (April 2020). "Retiform purpura: A diagnostic approach". Journal of the American Academy of Dermatology . 82 (4): 783–796. doi:10.1016/j.jaad.2019.07.112. PMID 31479689. S2CID 201831828 . Retrieved 25 October 2023.

[:2-4] 1 2 Wysong, A; Venkatesan, P (March 2011). "An approach to the patient with retiform purpura". Dermatologic Therapy . 24 (2): 151–172. doi: 10.1111/j.1529-8019.2011.01392.x . PMID 21410606. S2CID 10099582 . Retrieved 25 October 2023– via Wiley.

[5] Tan, Cheng; Jing, Hong-Mei; Zhang, Li-Tao; Ma, Dong-Lai; Zhang, Ru-zhi (2018), Zhu, Wen-Yuan; Tan, Cheng; Zhang, Ru-zhi (eds.), "Cutaneous Vascular Diseases", Atlas of Skin Disorders: Challenging Presentations of Common to Rare Conditions, Singapore: Springer, pp. 189–196, doi:10.1007/978-981-10-8037-1_14, ISBN 978-981-10-8037-1 , retrieved 28 January 2024

[:3-6] 1 2 Levi, Marcel (January 2004). "Current understanding of disseminated intravascular coagulation". British Journal of Haematology . 124 (5): 567–576. doi:10.1046/j.1365-2141.2003.04790.x. PMID 14871243. S2CID 12700686 . Retrieved 25 October 2023– via Wiley.

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