Atypical presentation of pediatric antiphospholipid syndrome
| Autor: | David G Cotter, Amin A. Hedayat, Harrison Shawa |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
Anetoderma LR livedo reticularis Case Report Dermatology Rapid plasma reagin SLE systemic lupus erythematosus DRVVT dilute Russell viper venom time Antiphospholipid syndrome medicine beta-2-glycoprotein Livedo reticularis Lupus anticoagulant Eccrine angiomatous hamartoma medicine.diagnostic_test business.industry anti-cardiolipin medicine.disease Malignant Atrophic Papulosis APS antiphospholipid syndrome EAH eccrine angiomatous hamartoma hypercoagulable eccrine angiomatous hamartoma RL1-803 medicine.symptom business antiphospholipid syndrome Purpura fulminans |
| Zdroj: | JAAD Case Reports JAAD Case Reports, Vol 16, Iss, Pp 50-52 (2021) |
| ISSN: | 2352-5126 |
| Popis: | Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by potentially life-threatening recurrent vascular thrombosis, miscarriage, and the presence of antiphospholipid antibodies, including anti-cardiolipin, anti-beta 2 glycoprotein, and lupus anticoagulant. It is additionally associated with false-positive rapid plasma reagin titer. APS can either be primary, presenting in the absence of another autoimmune disorder, or secondary, presenting with another autoimmune disorder. Data on the epidemiology of APS are limited, but 1 study found the annual incidence and prevalence to be 2.1 and 50 per 100,000, respectively.1 The epidemiology of APS is further limited in pediatric patients, as it is likely underdiagnosed due to a lack of pediatric-specific clinical criteria.2 Interestingly, children may have a higher rate of progression from primary to secondary APS.3,4 Cutaneous manifestations are common in APS, arising in 18% of cases, including livedo reticularis (LR), Raynaud phenomenon, atrophie blanche, anetoderma, urticaria, malignant atrophic papulosis, purpura fulminans, ulcerative skin lesions, pseudovasculitic lesions,5,6 and reactive angioendotheliomatosis.7 Although APS has been associated with various skin conditions, it has no established association with eccrine angiomatous hamartomas (EAHs). EAHs are rare, benign, acquired, or congenital neoplasms of eccrine and angiomatous structures, often within the middle or deep dermis, that usually present on the distal extremities with varied clinical features, including pain and hyperhidrosis.8,9 Herein we report a case of APS presenting as pain within a foot lesion, with changes consistent with EAH on pathology. |
| Databáze: | OpenAIRE |
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