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Livedoid Vasculopathy Further Evidence for Procoagulant Pathogenesis

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Livedoid Vasculopathy Further Evidence for Procoagulant Pathogenesis STUDY Livedoid Vasculopathy Further Evidence for Procoagulant Pathogenesis Bethany R. Hairston, MD; Mark D. P. Davis, MD; Mark R. Pittelkow, MD; Iftikhar Ahmed, MD Objective: To further characterize the clinical and patho- ments were decreased in 20 (74.1%) of 27, and factor V logic features, disease associations, and laboratory ab- Leiden mutation (heterozygous) was noted in 2 (22.2%) normalities of livedoid vasculopathy. of 9, decreased activity for protein C or protein S in 2 (13.3%) of 15, prothrombin G20210A gene mutation Design: Retrospective study of patients identified from in 1 (8.3%) of 12, and lupus anticoagulant in 5 (17.9%) our institutional database from January 1, 1990, to De- of 28. Anticardiolipin antibodies were present in 8 (28.6%) cember 31, 2000. of 28 patients, and elevated homocysteine levels in 3 (14.3%) of 21. Intraluminal thrombosis was observed Setting: Tertiary care institution. in 44 (97.8%) of 45 skin biopsy specimens. Direct im- munofluorescence disclosed multiple vascular conju- Patients: Forty-five patients with biopsy-proved live- gates in 31 (86.1%) of 36 biopsy specimens. doid vasculopathy. Conclusions: Livedoid vasculopathy was predomi- Main Outcome Measures: Clinical presentation, his- nantly bilateral, affected the lower extremities, and was topathologic diagnosis, results of testing for coagula- associated with ulceration and atrophie blanche. Histo- tion abnormalities, and assessment of vascular status. logic evidence of intraluminal thrombosis was observed in almost all biopsy specimens reviewed. Laboratory test- Results: Thirty-two patients (71.1%) were female (mean ing revealed numerous heterogeneous coagulation ab- age, 45 years; age range, 10-85 years). Bilateral lower ex- normalities, providing further evidence of procoagulant tremity disease occurred in 36 patients (80.0%), ulcer- mechanisms. ation in 31 (68.9%), and atrophie blanche in 32 (71.1%). In patients tested, transcutaneous oximetry measure- Arch Dermatol. 2006;142:1413-1418 IVEDOID VASCULOPATHY IS A izing vasculitis,”1 “livedo vasculitis,” and “li- chronic, recurrent, painful vedoid vasculitis.”4 The pathogenesis is not cutaneous disease with dis- fully understood. Variable nomenclature tinctive clinical features. The and the multifactorial nature of cutaneous initial clinical appearance ulcerations complicate classification of the Lmay include a primary lesion of focal pur- disease. It has been described as idio- pura progressing to shallow ulcerations. Atrophic, stellate, ivory to white, scarlike See also pages 1466 plaques stippled with telangiectasia and and 1481 surrounded by hyperpigmentation, known as “atrophie blanche,” are commonly iden- pathic,5 associated with immune complex– tified (Figure 1). Histologic features in- associated diseases,4 and a result of dermal clude hyalinizing vascular changes of the blood vessel occlusion.6 Laboratory test subintimal layer of dermal blood vessels, result abnormalities have been reported in typically with minimal inflammation 1 patients with the disease, including defec- (Figure 2). tive release of tissue plasminogen activa- tor,7 elevated fibrinopeptide A,8 antithrom- CME course available at bin III deficiency,9 and numerous other Author Affiliations: www.archdermatol.com thrombophilic abnormalities that are fur- Departments of Dermatology ther described in this article. The purpose (Drs Hairston, Davis, Pittelkow, and Ahmed) and Laboratory Originally described as atrophie blanche of our review was to further characterize the 2 Medicine and Pathology en plaque by Milian in 1929, synonyms for clinical features, disease associations, and (Dr Ahmed), Mayo Clinic, this disease have included “livedo reticu- laboratory test result abnormalities, includ- Rochester, Minn. laris with ulcerations,”3 “segmental hyalin- ing coagulation study results, in patients (REPRINTED) ARCH DERMATOL/ VOL 142, NOV 2006 WWW.ARCHDERMATOL.COM 1413 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Figure 2. Endothelial proliferation, hyalinized vascular change, and thrombosis of upper reticular dermal blood vessels (hematoxylin-eosin, original magnification ϫ200). Figure 1. Atrophic, stellate, ivory to white, scarlike plaques with central ulceration and surrounding hyperpigmentation. This patient had similar changes bilaterally on the legs, medial and lateral malleoli, and dorsal surfaces of the feet, without edema, other medical disease, or abnormal became available during the past decade; therefore, the period laboratory test results. from 1990 to 2000 was chosen for this retrospective review. Vascular status was assessed in the noninvasive vascular labo- with biopsy-proved livedoid vasculopathy whose condi- ratory at our institution. Continuous-wave Doppler ultraso- nography, a useful method for detection and diagnosis of deep tions were evaluated at the Mayo Clinic, Rochester, Minn, 10 from January 1, 1990, to December 31, 2000. venous insufficiency, was used to evaluate 6 vein qualities: patency, spontaneity, phase, augmentation, competency, and pulsatility. The continuous-wave Doppler ultrasonographic data METHODS were supplemented with information from exercise strain- gauge outflow plethysmography, which adds quantitative in- 11 INCLUSION AND EXCLUSION CRITERIA formation about the severity of venous insufficiency. Arte- rial investigations included transcutaneous oximetry (TcPO2), which measures oxygen tension for determining the degree of This retrospective review was approved by the Mayo Founda- 12 tion Institutional Review Board. The medical record database ischemia in poorly perfused skin. The TcPO2 measurements at our institution was searched using the master index diag- were graded as normal, mild to moderately reduced, or se- noses of “livedo or livedoid plus vasculitis or vasculopathy,” verely reduced. “segmental hyalinizing vasculitis or vasculopathy,” and “atro- Biopsy specimens (stained with hematoxylin-eosin) from all phie blanche.” of the patients in this series were reviewed. Direct immuno- Inclusion criteria were as follows: (1) painful, stellate, shal- fluorescence testing was performed using conjugate antise- low ulcerations or crusted erosions on the legs, ankles, or feet; rum to IgG, IgM, IgA, complement component 3, and fibrin. a history of such lesions healing with atrophie blanche; or pres- ence of atrophie blanche; and (2) histologic features of the dis- RESULTS ease as previously defined,1 including endothelial prolifera- tion and hyalinized degeneration of the subintimal layer of The study group consisted of 32 female and 13 male dermal blood vessel walls. Both requirements were fulfilled in 45 patients. Clinical data and biopsy specimens were available patients. Their age range was 10 to 85 years (mean age, for all patients. 45 years). Exclusion criteria were as follows: (1) no skin biopsy speci- men had been obtained and (2) another cause for the leg ul- CLINICAL FEATURES ceration had been elucidated. Examples include primary in- flammatory vasculitis (eg, polyarteritis nodosa), pyoderma When patients were first seen, the duration of symp- gangrenosum, sarcoidosis, factitial dermatitis, conditions in- toms ranged from less than 1 year to 45 years (mean, 6.3 duced by drugs (eg, hydroxyurea), cryoglobulinemia, and pri- years). Atrophie blanche was described in 32 patients mary antiphospholipid antibody syndrome. (71.1%). Ulcers were noted in 31 patients (68.9%), 25 (55.5%) of whom also had atrophie blanche. Among the ABSTRACTION OF DATA 13 patients without classically described atrophie blanche, shallow stellate ulcerations were noted in 6, crusted ero- Information abstracted from patient medical records included sions with history of ulceration in 3, and crusted ero- age at onset of disease, sex, clinical manifestations, relevant medi- sions with purpuric papules or plaques in 4. Edema was cal history, histopathologic diagnosis, and results of labora- tory and noninvasive vascular testing. Laboratory test result ab- present in 8 patients (17.8%), and the disease was bilat- normalities were analyzed with emphasis on the presence of eral in 36 (80.0%). The lesions involved the lower ex- an inherited or acquired coagulation abnormality as measured tremities in all patients, with most having lesions in a com- and reported by the coagulation testing laboratory at our in- bination of the following locations: foot, 28 patients stitution. This extensive testing for coagulation abnormalities (62.2%); ankle, 30 (66.7%); and leg, 36 (80.0%). (REPRINTED) ARCH DERMATOL/ VOL 142, NOV 2006 WWW.ARCHDERMATOL.COM 1414 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Table 1. Associated Medical Diseases in Patients Table 2. Results of Laboratory Testing in Patients With Livedoid Vasculopathy With Livedoid Vasculopathy No. of No. of No. of Disease Patients Patients Patients With Test Tested Abnormal Results Venous insufficiency 4 Deep venous thrombosis 3 Factor V Leiden gene mutation 92 Connective tissue disease 6 (heterozygous) Rheumatoid arthritis 3 Protein C or protein S 15 2 Scleroderma 1 Prothrombin G20210A gene mutation 12 1 Mixed connective tissue disease 1 Homocysteine 21 3 Undifferentiated connective tissue disease 1 Anticardiolipin antibody 28 8 Solid organ carcinoma 3 IgG alone 1 Colon 1 IgM alone 5 Pancreas 1 IgG plus IgM 2 Prostate gland 1 Lupus anticoagulant 28 5 Hematologic malignancy 2 B-cell lymphoproliferative disease 1 Multiple myeloma
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