Hemothorax Associated with Henoch-Schonlein Purpura

DERMATOLOGICA SINICA xxx (2016) 1e3

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Dermatologica Sinica

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CASE REPORT Hemothorax associated with HenocheSchonlein€ purpura

* Peng Hu 1, , Yun Guan 2, Li Mei Song 2, Fang Yuan Lu 2, Xun Xia 1

1 Department of Pediatrics, The First Afﬁliated Hospital of Anhui Medical University, Hefei, People's Republic of China 2 Department of Pediatrics, Hoten Area Hospital, Hoten, People's Republic of China article info abstract

Article history: Hemothorax is an extremely rare complication of HenocheSchonlein€ purpura. In this report, we Received: Mar 18, 2016 encountered a 9-year-old girl who suffered from nonthrombocytopenic purpura on her eyelids, buttocks, Revised: May 30, 2016 and lower limbs with hypertension, heme-positive stools, high serum immunoglobulin A, and low serum Accepted: Jun 13, 2016 complement component 3. Her skin biopsy revealed leukocytoclastic vasculitis with immunoglobulin A deposition. In addition, physical examination showed dullness on percussion and quiet breath sounds on Keywords: the left chest. Chest X-ray showed a large pleural effusion on the left hemithorax. Computed tomography hemothorax e € of her thorax revealed massive left-sided hemothorax with mediastinal shift to the right side. Most Henoch Schonlein purpura ﬂ immunoglobulin A critically, pleural uid evacuated by the chest tube was bloody. The patient was diagnosed as having e € methylprednisolone Henoch Schonlein purpura in combination with hemothorax, and treated with pulse methylpredniso- pleural ﬂuid lone followed by oral corticosteroids plus cyclophosphamide. Fortunately, we achieved excellent out- comes and the patient recovered gradually within a week. Copyright © 2016, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction Case Report

HenocheSchonlein€ purpura (HSP) is a common vasculitis of A 9-year-old girl was admitted to our department with a 10-day childhood, characterized by nonthrombocytopenic purpura, history of low-grade fever, cough, and purpuric rash on her abdominal pain, and joint swelling.1 A survey on the epidemio- eyelids, buttocks, and lower limbs (Figure 1). On admission, her logical characteristics of childhood HSP in Taiwan yielded an annual blood pressure was 112/70 mmHg, heart rate was 105 beats/min, incidence of 12.9 per 100,000 children under the age of 17 years.2 axillary temperature was 38.0C, respiratory rate was 33 breaths/ Although HSP is probably self-limiting in the majority of cases, min, and oxygen saturation was 92%. Physical examination the disease can also be associated with multiple organ lesions, such showed dullness on percussion and quiet breath sounds on the as nephritis, intussusception, enterobrosis, pancreatitis, hema- left chest. Complete blood count revealed mild anemia (hemo- tencephalon, and hemothorax. Among them, hemothorax is an globin 95g/L) and thrombocythemia (platelet 513 Â 109/L). Other extremely rare complication of HSP. To date, only one published blood chemistries were fibrin degradation products 92.2 mg/mL, case has been recorded in China, based on a systematic search of D-dimers 68.4 mg/mL, immunoglobulin A (IgA) 3.64 g/L, com- the literature in MEDLINE using the terms “HenocheSchonlein€ plement component (C)3 0.86 g/L, and C reactive protein purpura” and “hemothorax”.3 In the present report, we describe an 20.91 mg/L. She had microscopic hematuresis (46 erythrocytes/ unusual case of hemothorax associated with HSP. mL), massive proteinuria (7.77 g/d), and heme-positive stools. Antinuclear antibody and antineutrophil cytoplasmic antibody (ANCA) were negative. Skin biopsy revealed leukocytoclastic vasculitis with IgA deposition. Chest X-ray showed a large pleural effusion on the left hemithorax. Computed tomography of her Conflicts of interests: The authors declare that they have no financial or non-financial conflicts of interest related to the subject matter or materials dis- thorax revealed massive left-sided hemothorax with mediastinal cussed in this article. shift to the right side (Figure 2). A thoracocentesis was performed * Corresponding author. Department of Pediatrics, The First Affiliated Hospital of for the re-expansion of left lung, and the pleural fluid evacuated Anhui Medical University, Number 218 Jixi Road, Hefei 230022, People's Republic of by the chest tube was bloody (erythrocyte count 3.15 Â 109/L). China. E-mail address: [email protected] (P. Hu). The patient was diagnosed as having HSP in combination with http://dx.doi.org/10.1016/j.dsi.2016.06.003 1027-8117/Copyright © 2016, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).

Figure 2 (A) Chest X-ray shows a large pleural effusion in left hemithorax; (B) computed tomography of thorax reveals massive left-sided hemothorax with mediastinal shift to the right side.

Serum IgA is elevated in half the children, while complement C3 and C4 have been reported to be low in a few patients.5,6 In addition, this disease must be differentiated from other childhood vasculitides, such as systemic lupus erythematosus and ANCA- associated vasculitis. In the present report, we encountered a 9- year-old girl who suffered from nonthrombocytopenic purpura on her eyelids, buttocks, and lower limbs with heme-positive stools, high serum IgA, and low serum complement C3. Her skin biopsy revealed leukocytoclastic vasculitis with IgA deposition. Antinu- clear antibody and ANCA were negative. Therefore, the diagnosis of HSP in our patient can undoubtedly be made. Figure 1 Purpuric rash on eyelids, buttocks, and lower limbs. On admission, our patient exhibited cough, tachypnea, hypox- emia, and mild anemia, besides purpuric rash. Physical examina- hemothorax, and treated with pulse methylprednisolone (10 mg/ tion showed dullness on percussion and quiet breath sounds on the kg/d for 3 days) followed by oral corticosteroids (1 mg/kg/d) plus left chest. Chest X-ray showed a large pleural effusion on the left cyclophosphamide (500 mg/m2). Within a week, her skin lesions, hemithorax. Computed tomography of her thorax revealed massive renal involvement, and hemothorax resolved gradually, and she left-sided hemothorax with mediastinal shift to the right side. Most fl had normal oxygen saturation while breathing room air. critically, pleural uid evacuated by the chest tube was bloody (erythrocyte count 3.15 Â 109/L). Therefore, hemothorax secondary to HSP is the most preferable diagnosis in our patient according to Discussion available clinical symptoms and laboratory findings. Hemothorax is an extremely rare complication of HSP. To date, only one published In 1990, the American College of Rheumatology published diag- case has been recorded.3 Although the real pathogenesis is still nostic criteria for HSP. These included: (1) nonthrombocytopenic unknown, the hemothorax is likely due to an allergic diffuse purpura; (2) age 20 years or younger at onset of first symptoms of vasculitis, and may additionally be caused by an allergic reaction or the disease; (3) gastrointestinal involvement; and (4) histologic immune function disorder.7 IgA immune complex deposition, changes showing granulocytes in the walls of arterioles or venules.4 fragmentation, and the adhesion of a large number of white blood

Please cite this article in press as: Hu P, et al., Hemothorax associated with HenocheSchonlein€ purpura, Dermatologica Sinica (2016), http:// dx.doi.org/10.1016/j.dsi.2016.06.003 P. Hu et al. / Dermatologica Sinica xxx (2016) 1e3 3 cells may be the main causes of hemothorax, and they promote an References increase in the permeability of the pleural capillary network.8 € Standard treatment for hemothorax associated with HSP has not 1. Tizard EJ, Hamilton-Ayres MJ. HenocheSchonlein purpura. Arch Dis Child Educ Pract Ed 2008;93:1e8. been established. Corticosteroids are not recommended in patients 2. Yang YH, Hung CF, Hsu CR, et al. A nationwide survey on epidemiological with uncomplicated HSP, but pulse methylprednisolone followed characteristics of childhood HenocheSchonlein€ purpura in Taiwan. Rheuma- by oral corticosteroids plus cyclophosphamide is effective in tology (Oxford) 2005;44:618e22. € 9 3. Hammoudeh M, Qaddoumi NK. Pleural hemorrhage in HenocheSchonlein reversing internal organ lesions. A meta-analysis conducted by purpura. Clin Rheumatol 1993;12:538e9. 10 Rajagopala et al indicated that corticosteroids plus cyclophos- 4. Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology phamide could significantly decrease the mortality of pulmonary 1990 criteria for the classification of HenocheSchonlein€ purpura. Arthritis e hemorrhage associated with HSP from 100% to 27.6%. In this pa- Rheum 1990;33:1114 21. 5. Lau KK, Wyatt RJ, Moldoveanu Z, et al. Serum levels of galactose-deficient IgA tient, we followed suit. Fortunately, we achieved excellent out- in children with IgA nephropathy and HenocheSchonlein€ purpura. Pediatr comes and the patient recovered gradually within a week. We Nephrol 2007;22:2067e72. e € believe that the same treatment choice used for pulmonary hem- 6. Motoyama O, Iitaka K. Henoch Schonlein purpura with hypocomplementemia in children. Pediatr Int 2005;47:39e42. orrhage associated with HSP can be used for hemothorax. In 7. Ren X, Zhang W, Dang W, et al. A case of anaphylactoid purpura nephritis addition, plasmapheresis should be considered as an optional accompanied by pulmonary hemorrhage and review of the literature. Exp Ther Med 2013;5:1385e8. treatment, if no obvious therapeutic effects are obtained after e € 11 8. Krijnsen P, Halma C. Spots inside out. IgA vasculitis/Henoch Schonlein pur- multiple drug therapy. pura. Neth J Med 2014;72:32e6. 9. Hu P, Guan Y, Lu L. HenocheSchonlein€ purpura triggered by Mycoplasma Acknowledgments pneumoniae in a female infant. Kaohsiung J Med Sci 2015;31:163e4. 10. Rajagopala S, Shobha V, Devaraj U, et al. Pulmonary hemorrhage in HenocheSchonlein€ purpura: Case report and systematic review of the English The authors would like to gratefully acknowledge the most helpful literature. Semin Arthritis Rheum 2013;42:391e400. comments on this paper received from Professor Ling Lu and Pro- 11. Donghi D, Schanz U, Sahrbacher U, et al. Life-threatening or organ-impairing e € fi fi Henoch Schonlein purpura: Plasmapheresis may save lives and limit organ fessor Bo Hu, Department of Pediatrics, The rst Af liated Hospital damage. Dermatology 2009;219:167e70. of Anhui Medical University, Hefei, People's Republic of China.

Please cite this article in press as: Hu P, et al., Hemothorax associated with HenocheSchonlein€ purpura, Dermatologica Sinica (2016), http:// dx.doi.org/10.1016/j.dsi.2016.06.003