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Is this capillary malformation? Differential diagnosis and other dermal vascular lesions
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
Cutis marmorata telangiectatica congenita (CMTC) is a congenital multisystemic disorder with features including livedo racemosa, phlebectasias, and cutaneous and subcutaneous atrophy. The most important cutaneous manifestation of CMTC is livedo racemosa. This is a reticulate vascular eruption that should not be confused with a CM. While livedo racemosa has reticulate morphology, CM presents as a continuous telangiectatic patch or plaque. CMTC primarily involves the extremities and less commonly the trunk and face (Figure 86.9). It is associated with limb length and circumference discrepancy, glaucoma, intellectual disability, patent ductus arteriosus, and arterial stenosis.
Erythemas
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Nicola di Meo, Paola Corneli, Iris Zalaudek
Livedo reticularis presents as a regular unbroken network-like reddish-violaceous discoloration fading on warming of the skin or limb elevation (Figure 6.11A), while livedo racemosa consists of an irregular, broken (ramified) network-like, bluish-violaceous discoloration, which usually does not change with skin warming or limb elevation (Figure 6.12A).6 Livedo racemosa may also show a purpuric aspect (purpuric livedo), e.g., calciphylaxis (Figure 6.13A).6
Clinical presentations for chronic venous diseases
Published in Ken Myers, Paul Hannah, Marcus Cremonese, Lourens Bester, Phil Bekhor, Attilio Cavezzi, Marianne de Maeseneer, Greg Goodman, David Jenkins, Herman Lee, Adrian Lim, David Mitchell, Nick Morrison, Andrew Nicolaides, Hugo Partsch, Tony Penington, Neil Piller, Stefania Roberts, Greg Seeley, Paul Thibault, Steve Yelland, Manual of Venous and Lymphatic Diseases, 2017
Ken Myers, Paul Hannah, Marcus Cremonese, Lourens Bester, Phil Bekhor, Attilio Cavezzi, Marianne de Maeseneer, Greg Goodman, David Jenkins, Herman Lee, Adrian Lim, David Mitchell, Nick Morrison, Andrew Nicolaides, Hugo Partsch, Tony Penington, Neil Piller, Stefania Roberts, Greg Seeley, Paul Thibault, Steve Yelland
Secondary livedo reticularis is a pathologic variant associated with autoimmune diseases, or is veno-oclusive from hyperviscosity, cryoglobulins, drugs such as amantadine or infection such as hepatitis C or mycoplasma. It usually spreads beyond the legs to the buttocks and torso. It is necessary to differentiate the usually painless, symmetrical, unbroken vessel network of primary livedo reticularis from the frequently painful, irregular, asymmetrical and broken pattern observed in the secondary condition often associated with livedo racemosa.
A homozygote novel L451W mutation in CECR1 gene causes deficiency of adenosine deaminase 2 in a pediatric patient representing with chronic lymphoproliferation and cytopenia
Published in Pediatric Hematology and Oncology, 2019
Rabia Miray Kisla Ekinci, Sibel Balcı, Atil Bisgin, Ilgen Sasmaz, Goksel Leblebisatan, Faruk Incecik, Mustafa Yilmaz
Beside the classical vasculitic phenotype of DADA2, diversity of manifestations has been emphasized by several reports in the last 5 years. In a comprehensive review, skin manifestations including livedo reticularis, skin ulcers, subcutaneous rash, aphthous ulcers, and leukocytoclastic vasculitis, neurological signs such as stroke, polyneuropathy, and cranial nerve palsy were the leading findings in a substantial number of DADA2 patients. Besides, 4–16% of patients may have hematological manifestations in addition to other symptoms, however, only a minority of them suffer from severe anemia or pancytopenia as the first and only symptoms.9 Ben Ami et al. previously reported five DADA2 patients from four distinct families, representing predominantly hematological findings, of whom two had pure red cell aplasia resembling Diamond-Blackfan anemia, one had Coombs-positive hemolytic anemia with hypercellular bone marrow, one with a 6-year history of generalized lymphadenopathy, hepatosplenomegaly, and pancytopenia with dyserythropoietic changes in bone marrow. The remaining one had recurrent fever attacks accompanied by arthritis and splenomegaly.10 Our patient was not similar to those patients except for the absence of livedo racemosa, because she had both chronic anemia and hypocellular bone marrow resembling aplastic anemia, lymphoproliferation, and coombs positivity, on the counterpart, she did not have hemolysis or ANA positivity.
Clinical profiles and risk assessment in patients with antiphospholipid antibodies
Published in Expert Review of Clinical Immunology, 2019
Masaru Kato, Ryo Hisada, Tatsuya Atsumi
Livedo reticularis, particularly livedo racemosa, is the most common cutaneous manifestation of APS and develops in approximately one fourth of patients with APS but less occurs in SLE patients without aPL [24]. Small vessel thrombosis and inflammation at the dermis-subcutis junction is the common pathological feature of aPL associated livedo reticularis [36], consistent with aPL mediated pathophysiology as discussed above. Livedo reticularis in patients with APS has been shown by several cohort studies to be associated with thrombotic events and heart valve disease [37,38].
Antiphospholipid syndrome: the need for new international classification criteria
Published in Expert Review of Clinical Immunology, 2021
Georges El Hasbani, Ali T Taher, Savino Sciascia, Imad Uthman
Livedo racemosa, a similar dermatological manifestation to livedo reticularis, has also been reported in conjunction with aPL [66] although less prevalent than livedo reticularis [65]. Livedo racemosa occurs in the context of Sneddon’s syndrome which is characterized by cerebrovascular events in addition to livedo reticularis [67]. However, Sneddon’s syndrome can still be diagnosed in the absence of aPL [68].