Disseminated intravascular coagulation in Stevens-Johnson syndrome and toxic epidermal necrolysis - 07/11/20

Doi : 10.1016/j.jaad.2020.08.065

Chun-Bing Chen, MD ^a,^b,^c,^d,^e,^f,^g, Tsun-Hao Hsu, MD ^a,^b, Rosaline Chung-Yee Hui, MD, PhD ^a,^b, Chun-Wei Lu, MD ^a,^b,^c,^d,^f, Wei-Ti Chen, MD ^a,^b, Pin-Hsuan Chiang, MD ^a,^b, Chuang-Wei Wang, PhD ^a,^b,^c,^d,^e, Shiow-Shuh Chuang, MD, PhD ^h, Jui-Yung Yang, MD ^h, Shih-Yi Yang, MD ^h, Shu-Ying Chang, MD ^h, Yen-Chang Hsiao, MD ^h, Kuo-Chin Kao, MD ^i,^j, Han-Chung Hu, MD ^i,^j, Ting-Shu Wu, MD, PhD ^k, Chao-Wei Hsu, MD, PhD ^l, David Hui-Kang Ma, MD, PhD ^m, Shin-Yi Chen, MD ⁿ, Ya-Chung Tian, MD ^o, Chi-Yuan Cheng, MS ^p, Chi-Hua Chen, MS, PhD ^p, Min-Hui Chi, MD ^a,^b, Ming-Ying Wu, MD ^a,^b, Ren-Feng Liu, MD ^a,^b, Chi-Hui Wang, MD ^a,^b, Ya-Ching Chang, MD ^a,^b, Jing-Yi Lin, MD ^a,^b, Hsin-Chun Ho, MD ^a,^b, Yang Yu-Wei Lin ^a, Chee Jen Chang, PhD ^b,^q,^r, Yu-Jr Lin, BA ^b,^q,^r, Cheng-Lung Ku, PhD ^s,^t, Shuen-Iu Hung, PhD ^b,^e, Wen-Hung Chung, MD, PhD ^a,^b,^d,^e,^f,^g,^u,^v,^⁎
for the
Taiwan Severe Cutaneous Adverse Reaction Consortium
^a Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan
^b College of Medicine, Chang Gung University, Taoyuan, Taiwan
^c Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan
^d Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
^e Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
^f Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
^g Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China
^h Department of Plastic and Reconstructive Surgery, The Burn Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan
ⁱ Department of Thoracic Medicine, Chang Gung Memorial Hospital, Taoyuan, Taiwan
^j Clinical Informatics Department of Respiratory Therapy, College of Medicine, Chang Gung University, Taoyuan, Taiwan
^k Division of Infectious Diseases, Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kweishan, Taoyuan, Taiwan
^l Department of Hepatogastroenterology, Liver Research Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan
^m Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
ⁿ Department of Ophthalmology, Chang Gung Memorial Hospital, Keelung, Taiwan
^o Department of Nephrology, Kidney Research Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
^p Department of Pharmacy, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
^q Research Services Center for Health Information, Chang Gung University, Taoyuan, Taiwan
^r Clinical Informatics and Medical Statistics Research Center, Chang Gung University, Taoyuan, Taiwan
^s Laboratory of Human Immunology and Infectious Disease, Graduate Institute of Clinical Medical Sciences, Chang Gung University, Taoyuan, Taiwan
^t Department of Nephrology, Chang Gung Memorial Hospital, Taoyuan, Taiwan
^u Department of Dermatology, Beijing Tsinghua Chang Gung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China
^v School of Medicine, Shanghai Jiao Tong University, Shanghai, China

^∗Correspondence to: Wen-Hung Chung, MD, PhD, Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, No. 5, Fusing St, Taoyuan 333, Taiwan.Department of DermatologyDrug Hypersensitivity Clinical and Research CenterChang Gung Memorial HospitalNo. 5, Fusing StTaoyuan333Taiwan

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Abstract

Background

Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known.

Objective

This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN.

Methods

We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019.

Results

We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%).

Limitations

The study limitations include small sample size and a single hospital system.

Conclusion

Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.

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Key words : coagulopathy, disseminated intravascular coagulation, severe cutaneous adverse reactions, Stevens-Johnson syndrome, thrombocytopenia, toxic epidermal necrolysis

Abbreviations used : CI, OR, SJS, TEN

Plan

Patient characteristics

Complications and mortality associated with disseminated intravascular coagulation in SJS/TEN patients

Infectious pathogens identified among SJS/TEN patients with disseminated intravascular coagulation

Management of SJS/TEN patients with disseminated intravascular coagulation

Discussion

	Funding sources: Supported by research grants from the Ministry of Science and Technology, Taiwan (MOST 108-2314-B-182A-006-MY3 to CB Chen; (MOST 103-2321-B-182-001, MOST 104-2314-B-182A-148-MY3, MOST 104-2325-B-182A-006, MOST 105-2325-B-182A-007, MOST 106-2314-B-182A-037-MY3, MOST 106-2622-B-182A-003-CC2, MOST 107-2622-B-182A-001-CC2, MOST 108-2314-B-182A-104-MY3, MOST 108-2320-B-182A-023-MY3, MOST 108-2320-B-182A-024-MY2, MOST 109-2320-B-182A-008 -MY3 to WH Chung) and Chang Gung Memorial Hospital, Taiwan (CMRPG2H0081, CMRPG2J0221, CMRPG2J0222, NMRPG2J6012, NMRPG2J6013, CIRPG2I0011, CIRPG2I0012, CIRPG2I0013 to CB Chen; CIRPD1D0032 to CJ Chang; CIRPG3I0022, CIRPG3I0023, CIRPG3I0042, CIRPG3I0043, CLRPG3E0036, CLRPG3J0012, CMRPG3I0382, CORPG3J0322, CLRPG2E0053, CMRPG3D0363, CORPG3F0042~3, NCRPG3G0023, NCRPG3GS023, NMRPG3G6293, NMRPG3J6062, NMRPG3J6063, NMRPG3K0521, OMRPG3E0041 to WH Chung).
	Conflicts of interest: None disclosed.
	IRB approval status: Reviewed and approved by the IRB and the ethics committee of Chang Gung Memorial Hospital, in compliance with Taiwanese law.