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Table of Contents
Year : 2023  |  Volume : 41  |  Issue : 1  |  Page : 54-55

Acrocyanosis and retiform purpura as the first manifestation of catastrophic antiphospholipid syndrome in a child: A case report

1 Department of Dermatology, MacKay Memorial Hospital, Taipei, Taiwan
2 Department of Allergy, Immunology and Rheumatology, Mackay Children's Hospital, Taipei, Taiwan
3 Department of Pediatric Cardiology, Mackay Children's Hospital, Taipei, Taiwan
4 Department of Dermatology, MacKay Memorial Hospital, Taipei; Department of Medicine, MacKay Medical College, New Taipei City, Taiwan

Date of Submission26-May-2022
Date of Decision23-Dec-2022
Date of Acceptance26-Dec-2022
Date of Web Publication20-Feb-2023

Correspondence Address:
Dr. Yu-Hung Wu
Department of Dermatology, MacKay Memorial Hospital, No. 92, Sec. 2, Zhongshan North Road, Taipei 10449
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.DS-D-22-00086

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How to cite this article:
Wong CK, Fang LC, Hung WL, Wu YH. Acrocyanosis and retiform purpura as the first manifestation of catastrophic antiphospholipid syndrome in a child: A case report. Dermatol Sin 2023;41:54-5

How to cite this URL:
Wong CK, Fang LC, Hung WL, Wu YH. Acrocyanosis and retiform purpura as the first manifestation of catastrophic antiphospholipid syndrome in a child: A case report. Dermatol Sin [serial online] 2023 [cited 2023 May 31];41:54-5. Available from: https://www.dermsinica.org/text.asp?2023/41/1/54/370022

Dear Editor,

Antiphospholipid syndrome (APS) is a multisystem autoimmune disorder characterized by the development of vascular thrombosis in the presence of antiphospholipid antibodies.[1] Catastrophic APS (CAPS) is a fulminant and fatal variant that occurs in <1% of patients with APS.[2] Cutaneous involvement as the first manifestation occurs in approximately 10% of patients.[2] Herein, we present an unusual pediatric case of newly diagnosed systemic lupus erythematosus (SLE) initially presenting with CAPS with acrocyanosis and retiform purpura on soles.

A 7-year-old boy without a history of any major disease sought medical help with a fever of up to 39°C for 2 days. He was experiencing acrocyanosis, tachycardia, dyspnea, decreased urine output, and headache. Laboratory investigations revealed hemoglobin of 8.5 g/dL, a high total leukocyte count (42,000/μL with 71% eosinophils), and elevated troponin I levels (1.599 ng/mL, normal range <0.5ng/mL). An electrocardiogram revealed diffuse ST inversion and a prolonged QTc interval. An echocardiogram revealed pericardial effusion. High levels of erythrocyte sedimentation rate (>140 mm/h) and C-reactive protein (1.057 mg/dL, normal range <0.79 mg/dL) were detected. Other chemical analyses including renal function test and urinalysis were normal. No pathogen in cerebrospinal fluid was detected in the smear and culture. Computed tomography scan of the brain was unremarkable. Physical examination revealed diffuse cyanosis of fingers and toes tips. His soles exhibited livedoid and purpuric changes with a prominence at the weight-bearing region [Figure 1]a and [Figure 1]b. No abnormal skin changes were observed on his trunk. Upon histopathological examination of an incised specimen from the toe, the epidermis and dermis appeared normal, and the inflammation was minimal. However, small thrombi were noted in some capillaries and venules [Figure 2]a, [Figure 2]b, [Figure 2]c. These focal intravascular thrombi tested positive on a periodic acid–Schiff stain with diastase [Figure 2]d, supporting a diagnosis of thrombotic vasculopathy. Direct immunofluorescence revealed consistent immunoglobulin (Ig) A, IgG, IgM, C3, and fibrinogen intravascular deposition in the blood vessels of the deep dermis. The direct antiglobulin test was positive, and laboratory tests revealed an antinuclear antibody (ANA) titer of 1:1280, decreased levels of protein S (42.2%, normal range 60%–130%) and protein C (51.9%, normal range 70%–140%), and the presence of anticardiolipin IgG (20.7 GPL/mL) and IgM (23.2 MPL/mL). Therefore, the patient met the diagnostic criteria for SLE and CAPS, including histopathological evidence of multiple small vessel occlusions, the presence of anticardiolipin antibodies, and multiorgan involvement over a very short period.
Figure 1: Clinical features. (a) Livedoid patches on sole and purpuric changes in toes and (b) cyanosis of toe tips

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Figure 2: Pathology of toe skin biopsy. (a) Normal epidermis and dermis with minimal inflammation. Small thrombi were present in venules in (b) upper dermis and (c) lower dermis close to eccrine glands. (d) Intravascular thrombi were positive on a periodic acid–Schiff stain with diastase (hematoxylin and eosin, original magnification, ×20 [a], ×400 [b], ×400 [c]; periodic acid–Schiff stain with diastase, ×400 [d])

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This patient received prednisolone (15 mg once daily), hydroxychloroquine (200 mg once daily), azathioprine (75 mg once daily), and aspirin (100 mg once daily). Leukocytosis with eosinophilia and skin condition improved within 1 week after initiation of therapy. Hemolytic anemia, ANA titer, and anticardiolipin antibodies returned to normal 6 months later. Subsequent myocardial perfusion scans and echocardiography revealed normal results in the follow-up period.

The prevalence of APS is 40–50/100,000 people.[3] APS has various manifestations, including deep vein thrombosis, stroke, transient ischemic attacks, autoimmune hemolytic anemia, thrombotic microangiopathic syndromes, myocardial infarctions, and valve abnormalities. Patients may present with cutaneous manifestations such as livedo reticularis/racemosa, cutaneous necrosis, skin ulceration, digital gangrene, Raynaud's phenomenon, and vasculitis-like eruptions.[4]

More than half of APS cases occur within the context of an autoimmune disorder. SLE occurs in 35% of patients with APS. The prognoses of patients with SLE who have APS are less favorable compared with those without APS because of APS-related thrombotic events.[5] The diagnosis of APS is made based on the clinical evidence of vascular thrombosis and the presence of one or more types of antiphospholipid antibodies, including anticardiolipin antibodies, β2 glycoprotein-I antibodies, and lupus anticoagulants.[1]

APS that progresses within 1 week and has disseminated thrombotic vasculopathy involving multiple small supplying vessels of three or more organs/tissues is referred to as CAPS.[2] In addition to antiphospholipid antibodies, CAPS is characterized by activation of the complement system, which causes microvascular thrombosis in multiple organs and subsequent tissue necrosis with multiorgan failure.[6] The peripheral blood eosinophilia in this patient was an uncommon presentation. Eosinophils regulate blood coagulation through actively providing a procoagulant phospholipid surface.[7] Transient eosinophilia might be a secondary phenomenon.

The treatment of CAPS is aimed at inhibiting the cytokine storm and thrombosis to prevent sequelae.[8] However, CAPS has a high mortality rate even with optimal management.[8] This patient's retiform purpuric changes on soles led to vasculopathy and the diagnosis of SLE-related CAPS. The unique initial presentations of SLE-related CAPS warrant clinical attention to ensure accurate diagnosis and treatment.

Ethical approval

This study was approved by the Institutional Review Board of Mackay Memorial Hospital (approved number: 22MMHIS061e, approval date: April 08, 2022).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient's guardian consent forms. the guardian has given the consent for the child's images and other clinical information to be reported in the journal. The guardian understands that the child's name and initial will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

Dr. Yu-Hung Wu, an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.

  References Top

Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003;12:530-4.  Back to cited text no. 1
Cervera R, Bucciarelli S, Plasín MA, Gómez-Puerta JA, Plaza J, Pons-Estel G, et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32:240-5.  Back to cited text no. 2
Durcan L, Petri M. Chapter 2 – Epidemiology of the Antiphospholipid Syndrome. In: Cervera R, Espinosa G, Khamashta M, editors. Handbook of Systemic Autoimmune Diseases. Vol. 12. The Netherlands: Elsevier; 2017. p. 17-30.  Back to cited text no. 3
Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46:1019-27.  Back to cited text no. 4
Pons-Estel GJ, Andreoli L, Scanzi F, Cervera R, Tincani A. The antiphospholipid syndrome in patients with systemic lupus erythematosus. J Autoimmun 2017;76:10-20.  Back to cited text no. 5
Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: A clinical review. J Nephropathol 2014;3:9-17.  Back to cited text no. 6
Moosbauer C, Morgenstern E, Cuvelier SL, Manukyan D, Bidzhekov K, Albrecht S, et al. Eosinophils are a major intravascular location for tissue factor storage and exposure. Blood 2007;109:995-1002.  Back to cited text no. 7
Cervera R. Update on the diagnosis, treatment, and prognosis of the catastrophic antiphospholipid syndrome. Curr Rheumatol Rep 2010;12:70-6.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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