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• W3116149776 abstract "To the Editor: Acute hemorrhagic edema of young children, also termed cockade purpura with edema or Finkelstein-Seidlmayer disease, is a rare small-vessel leukocytoclastic vasculitis. It characteristically occurs after a febrile illness or a vaccination and is sometimes considered a variant of Henoch-Schönlein purpura.1Ting T.V. Diagnosis and management of cutaneous vasculitis in children.Pediatr Clin North Am. 2014; 61: 321-346Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar,2Lava S.A.G. Milani G.P. Fossali E.F. Simonetti G.D. Agostoni C. Bianchetti M.G. Cutaneous manifestations of small-vessel leukocytoclastic vasculitides in childhood.Clin Rev Allergy Immunol. 2017; 53: 439-451Crossref PubMed Scopus (24) Google Scholar The diagnosis is made clinically in children who are not ill appearing and who present with erythematous annular skin lesions and diffuse nonpitting and often tender body edema with a predilection for cheeks, ears, and extremities (Fig 1). Petechiae, purpuric lesions, or ecchymoses also sometimes occur. It is widely held that this vasculitis typically affects children aged 4 weeks to 23 months, is skin limited, remits within 3 weeks, and does not recur.1Ting T.V. Diagnosis and management of cutaneous vasculitis in children.Pediatr Clin North Am. 2014; 61: 321-346Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar, 2Lava S.A.G. Milani G.P. Fossali E.F. Simonetti G.D. Agostoni C. Bianchetti M.G. Cutaneous manifestations of small-vessel leukocytoclastic vasculitides in childhood.Clin Rev Allergy Immunol. 2017; 53: 439-451Crossref PubMed Scopus (24) Google Scholar, 3Fiore E. Rizzi M. Ragazzi M. et al.Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review.J Am Acad Dermatol. 2008; 59: 684-695Abstract Full Text Full Text PDF PubMed Scopus (92) Google Scholar To investigate the characteristics of uncommon features and the prevalence of recurrences, we used the Acute Hemorrhagic Edema Bibliographic Database. This database includes 270 original reports addressing 458 individually documented cases (313 male and 145 female patients) published between 1970 and 2019.4Rinoldi P.O. Milani G.P. Bianchetti M.G. Ferrarini A. Ramelli G.P. Lava S.A.G. Acute hemorrhagic edema of young children: open questions and perspectives.Int J Dermatol Skin Care. 2019; 1: 63-67Crossref Google Scholar,5Pellanda G. Lava S.A.G. Milani G.P. Bianchetti M.G. Ferrarini A. Vanoni F. Immune deposits in skin vessels of patients with acute hemorrhagic edema of young children: a systematic literature review.Pediatr Dermatol. 2020; 37: 120-123Crossref PubMed Scopus (6) Google Scholar We analyzed the following uncommon features: (1) systemic features such as articular (joint pain with or without swelling), abdominal (such as pain, vomiting, diarrhea, intestinal bleeding, and intussusception), or kidney involvement (proteinuria or hematuria); (2) eruptions such as blistering lesions, Köbner phenomenon, or extensive skin necrosis; (3) production of tears partially composed of blood (ie, hemolacria); (4) compartment syndrome of the extremities; and (5) positive family history (acute hemorrhagic edema or another vasculitis in a first-degree relative of a patient). Uncommon features were detected in 109 of the 458 cases (Table I): a systemic involvement in 66 cases (61%), uncommon eruptions in 24 (22%), hemolacria in 7 (6.4%), compartment syndrome in 3 (3.3%), and a positive family history in 9 (8.2%). Patients with articular or kidney involvement were significantly older than those without uncommon features. The disease duration was less than or equal to 3 weeks in 107 of the 109 cases with uncommon features. The diagnosis was supported by a skin biopsy in 39 (36%) of the 108 cases with uncommon features (including 21 of the 22 cases with blistering eruptions and the patient with extensive skin necrosis).Table IPatients affected by acute hemorrhagic edema without and with uncommon featuresNSex (male:female patients)Age∗Median and interquartile range (≥10 cases) or individual values (<10 cases). (months)Without uncommon features349229:12011 (8-18)With uncommon features10984:2513†P < .05 versus cases without uncommon features (Kruskal-Wallis test with Dunn posttest). (9-19) Systemic features‡One systemic feature in 59 and 2 in 7 cases.6651:1515†P < .05 versus cases without uncommon features (Kruskal-Wallis test with Dunn posttest). (10-21)Articular involvement3425:919†P < .05 versus cases without uncommon features (Kruskal-Wallis test with Dunn posttest). (12-23)Abdominal involvement2620:611 (9-18)Kidney involvement1311:217†P < .05 versus cases without uncommon features (Kruskal-Wallis test with Dunn posttest). (13-19) Uncommon eruptions§One uncommon eruption in 22 and 2 in 2 cases.2418:69 (7-15)Blistering lesions2218:49 (6-16)Köbner phenomenon32:15, 11, 27Extensive skin necrosis10:17 Hemolacria74:35, 6, 7, 10, 11, 12, 13 Compartment syndrome33:011, 19, 25 Positive family history98:1Birth (N = 4), 8, 11, 12, 42Information not available in 1 case.∗ Median and interquartile range (≥10 cases) or individual values (<10 cases).† P < .05 versus cases without uncommon features (Kruskal-Wallis test with Dunn posttest).‡ One systemic feature in 59 and 2 in 7 cases.§ One uncommon eruption in 22 and 2 in 2 cases.|| Information not available in 1 case. Open table in a new tab Nine acute hemorrhagic edema cases occurred within 5 families. In 3 families, 1 case each of acute hemorrhagic edema and Henoch-Schönlein purpura was observed. In the remaining families, 2 and 4 family members were affected by acute hemorrhagic edema. Recurrences (reappearance of eruption after a recovery for ≥4 weeks) were observed in 21 (4.5%) of the 458 cases (10 in cases without and 11 in cases with uncommon features; P < .01; Fisher's exact test). This analysis confirms the excellent prognosis of acute hemorrhagic edema: it normally resolves spontaneously within 3 weeks without recurrences and is skin limited in approximately 85% of cases. On the other hand, this analysis provides new insights into the condition: one-fifth of patients present with a systemic involvement, uncommon eruptions, or a compartment syndrome. Furthermore, the family history is sometimes positive. None disclosed." @default.
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• W3116149776 title "Acute hemorrhagic edema: Uncommon features" @default.
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