DRESS – A Deathly Syndrome: Case Report from a Tertiary Care Centre in South India
John Masilamani Nachimuthu, Johnson Vijayakumar Paulraj,
Dinesh Kumar Suganandam, Sheeba Rani Paul
College of Nursing, CMC, Vellore.
*Corresponding Author Email: johnmasilamani87@gmail.com
ABSTRACT:
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare, severe, and potentially life-threatening adverse drug reaction characterized by fever, rash, eosinophilia, and multi-organ involvement. DRESS syndrome has a rare occurrence with an estimated incidence ranging from 1:1000 to 1:10,000 drug exposures. It can manifest in a wide range of symptoms, which often appear one to eight weeks after starting the trigger medication. Most of the diagnostic criteria are replaced by the validated RegiSCAR scoring system. The complexities of managing this condition include the importance of early recognition, withdrawal of the culprit drug, and the use of systemic corticosteroids. This case study underscores the critical role of interdisciplinary collaboration among clinicians, allergists, dermatologists, and nurses in achieving a favorable outcome for the patient. Furthermore, it emphasizes the need for heightened awareness among healthcare providers regarding the potential for DRESS syndrome when encountering patients with drug-induced skin eruptions and systemic symptoms.
KEYWORDS: DRESS Syndrome, Corticosteroid, Esinophilia.
INTRODUCTION:
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare and potentially life-threatening condition that can occur as a severe adverse reaction to certain medications. The term DRESS syndrome was first introduced by Bocquet et al. to denote a serious condition that can occur in response to drug exposure, characterized primarily by a significant skin reaction.
This designation highlights the need for careful monitoring and consideration in the context of medication use. Later, the term rash is replaced with reaction because of the varied involvement of the skin. This syndrome is characterized by a variety of symptoms that can affect multiple organs in the body. The other names for DRESS syndrome are:
· Drug-induced pseudo lymphoma
· Anticonvulsant hypersensitivity syndrome
· Drug-induced hypersensitivity syndrome
· Severe cutaneous adverse reaction (SCAR)
· Phenytoin hypersensitivity syndrome
· Drug-induced delayed multiorgan hypersensitivity syndrome
· Drug Rash with Eosinophilia and Systemic Symptoms.1–4
Epidemiology:
DRESS syndrome has a rare occurrence with an estimated incidence ranging from 1:1000 to 1:10,000 drug exposures.5 Since the incidence of DRESS depends on the type of medication and the immune status of each patient, the actual number can vary. In most cases, it remains undiagnosed or untreated. DRESS syndrome can affect not only hematological but also multiple solid organ impairments. The mortality due to DRESS syndrome varies from 10% to 20%.6 The prevalence of DRESS syndrome is higher among the African population.7
Cacoub et al. (2011) in their systematic review reported that a total of 172 cases were associated with 44 drugs. The most commonly reported drug associated with DRESS was Carbamazepine. The other drugs that are commonly associated are anticonvulsants, sulfonamides, nonsteroidal anti-inflammatory drugs, beta-lactam antibiotics, and antiretrovirals. However, in 10–20% of cases, the causative drug cannot be identified.8-10
Pathophysiology:
DRESS, or Drug Reaction with Eosinophilia and Systemic Symptoms, is recognized as an immune-mediated reaction. This condition manifests in a select group of patients and is characterized by the presence of eosinophilia. Additionally, DRESS represents a distinct modification of the lymphocytic response, highlighting its complex immunological underpinnings. It is a type IV hypersensitivity reaction. (Figure 1)11
Figure 1. Pathophysiology of DRESS syndrome retrieved from Ganeshanandan & Lucas(2021)11
Symptoms of DRESS Syndrome:
DRESS Syndrome can manifest in a wide range of symptoms, which often appear one to eight weeks after starting the trigger medication. Common signs and symptoms include:
1. High fever
2. Skin rash, often described as a morbilliform rash (resembling measles)
3. Erythroderma
4. Mucosal involvement
5. Systemic involvement causes inflammation of the lungs, heart, kidneys, liver, stomach, pancreas, colon, thyroid, muscle, and lymph nodes.
These symptoms can vary in severity and may worsen over time if the condition is not promptly recognized and treated.12
Common Investigations in DRESS:
The common investigations performed for a suspected DRESS include the following (13)
· Complete Blood counts
· Peripheral blood smear
· Liver Function Test
· Serum Creatinine
· Urinalysis
· Serology for viral hepatitis
Diagnostic Criteria:
Because of the variability in clinical presentations, the diagnosis of DRESS is usually delayed. The diagnostic criteria proposed by Bocquet et. al12 affirms that the dress syndrome is characterized by all three items.
1. Cutaneous drug eruption
2. Systemic manifestations that suggest involvement of the lung, heart, liver, kidney, muscle, and lymph nodes
3. Alterations in blood cell counts (elevated eosinophils, atypical lymphocytes)
These diagnostic criteria are replaced by the RegiSCAR DRESS validation criteria proposed by the RegiSCAR group. These criteria are based on clinical and laboratory findings with a scoring system, which facilitates the identification of negative/possible/probable/definitive cases of DRESS.14 These diagnostic criteria are presented in Table 1
Table 1. RegiSCAR scoring system
|
Criteria |
−1 |
0 |
+1 |
+2 |
Min |
Max |
|
Fever greater than or equal to 38.5 °C |
No |
Yes |
-1 |
0 |
||
|
Lymph node enlargement |
No/U |
Yes |
0 |
1 |
||
|
Eosinophilia |
|
|
|
|
0 |
2 |
|
Eosinophils |
700–1499/μL |
≥1500/μL |
|
|
||
|
Eosinophils, if leukocytes are <4,000 |
10–19.9% |
≥20% |
|
|
||
|
Atypical (or reactive) lymphocytes |
No/U |
Yes |
0 |
1 |
||
|
Skin Involvement |
|
|
|
|
-2 |
2 |
|
Extensive rash (>50% TBSA) |
No/U |
Yes |
|
|
||
|
Rash suggestive of DRESS |
No |
U |
Yes |
|
|
|
|
Biopsy suggestive of DRESS |
No |
Yes/U |
|
|
||
|
Organ Involvement |
|
|
|
|
0 |
2 |
|
Hepatic impairment |
No/U |
Yes |
|
|
||
|
Renal impairment |
No/U |
Yes |
|
|
||
|
Lung manifestations |
No/U |
Yes |
|
|
||
|
Muscle/Heart manifestations |
No/U |
Yes |
|
|
||
|
Pancreatic impairment |
No/U |
Yes |
|
|
||
|
Impairment of other organs |
No/U |
Yes |
|
|
||
|
Resolution in ≥15 days |
No/U |
Yes |
-1 |
0 |
||
|
Evaluation of other potential causes: |
|
|
|
|
0 |
1 |
|
ANA/ Blood cultures/ Serology for Hepatitis A/B/C/ Chlamydia/Mycoplasma pneumoniae/ Other serologies/PCR If none is positive and ≥3 of above negative |
Yes |
|
|
|||
|
Total Score |
|
|
|
|
-4 |
9 |
‘U’ is considered as unknown or unclassifiable. The final score is interpreted as follows
Figure 2: Interpretation of RegiSCAR Scoring System
Medical Management:
1. Following the diagnosis of DRESS syndrome, the definitive treatment involves the discontinuation of the culprit drug. It will be challenging if the medication is being taken for any underlying medical condition. Alternative medications may be prescribed under the close supervision of a healthcare provider. All patients with a confirmed diagnosis should be admitted and monitored for systemic involvement.
2. Treatment includes administration of topical or systemic corticosteroids to reduce inflammation.15,16 Emollients and antihistamines are also prescribed to soothe the skin and control the allergic reaction. If symptom control is not achieved with steroids, drugs such as cyclosporine/ cyclophosphamide, rituximab, N-Acetyl cystine, immunoglobulins, and plasmapheresis can be attempted to manage specific organ complications.17
Nursing Management:
Nursing Management of patients with DRESS syndrome includes:
· Maintaining airway patency, optimizing breathing, and improving circulation.
· Frequent monitoring of vital signs
· Ensuring adequate fluid and electrolyte balance
· Providing optimal nutritional support
· Care of wounds and skin reactions
· Regulating the temperature
· Administration of drugs (corticosteroids, immunoglobulins, immunosuppressants)
· Monitoring, treating, and preventing infection
· Observing and initiating measures to prevent complications
· Educating the patient and family members on care
· Offering psychological support and reassurance
Prognosis:
The prognosis for individuals with DRESS Syndrome varies depending on the severity of the illness, early identification of the diagnosis, and effective management. While some people may recover fully with appropriate care, others may experience long-term complications, including organ damage. Patients with DRESS are highly prone to develop systemic autoimmune sequelae. The most common reported sequelae following DRESSS are autoimmune thyroiditis. Diabetes mellitus, autoimmune hemolytic anemia, alopecia, vitiligo, rheumatoid arthritis, systemic lupus erythematosus, and thrombocytopenic purpura are other autoimmune sequelae identified.17
Case report:
A 40-year-old woman was brought to the Emergency Department of a tertiary care centre in South India in a critical stage for further management. She was intubated in the previous hospital and was on high inotrope support. Upon receiving the patient, a quick initial assessment done revealed absent carotid pulse. Hence, Cardio Pulmonary Resuscitation was initiated, and a total of 24 cycles of CPR with 12 doses of Inj. Adrenaline 1mg was administered. Poor prognosis was explained to the patient's relatives. Despite all the life-saving measures tried, the monitor showed asystole, and the pupils were observed dilated and fixed. Death was declared. Her previous history of onset of the current illness was collected, and it revealed the following.
· She was apparently well till 10 days ago when she was presented to a local health center with complaints of intermittent episodes of fever, associated with vomiting of three episodes, and loose stools. Her blood investigations revealed an elevated level of C-reactive protein (59.7mg/L). She was treated symptomatically with medication (unknown) and was discharged on the fourth day.
· After discharge, she developed generalized itching, swelling, and jaundice. So, she presented it to the same hospital on the following day (5th day). Her investigations showed impaired liver function (SGOT-202U/L, SGPT-288U/L, ALP-450IU/L). She was discharged with oral antibiotics (Tab. Doxycycline), hepatoprotective drugs (Tab. Ursodeoxycholic acid, Tab. Liv 52 {Herbal supplement}), and proton pump inhibitors (Tab. Rabeprazole).
· On the tenth day of her illness, she started Ayurveda treatment in hopes of finding relief; however, she did not see any improvement.
· On day 11, she made the decision to seek medical assistance at a secondary care hospital in order to address the persistent symptoms she had been experiencing. On examination, her Temperature was 101°F, and her BP was measured as 90 / 60 mmHg. She had diffuse erythematous blanching maculopapular rash on the chest, back, thighs, legs, and had papular rash on the face with swelling of the lips, periorbital region, and cheeks with a few blisters on the left side of the trunk.
The following Differential Diagnoses were Considered:
· Drug-induced Liver injury
· Vasculitis,? Rickettsial fever,? Autoimmune disorder
· DRESS
She was admitted for further evaluation, and preliminary investigations showed leukocytosis with a significantly high level of eosinophils. There were transaminitis with elevated ALP and bilirubin. The USG findings were suggestive of acalculous cholecystitis. Her RegiSCAR score was 5, suggestive of probable DRESS. She started on broad-spectrum antibiotics. She developed respiratory distress on day 2 (13th day of illness) of admission. She was connected to non-invasive ventilation with supplemental oxygen. She was also given Inj. Hydrocortisone in view of a probable DRESS. Due to her hypotension, she was started on vasopressors. Further treatment options were discussed with relatives, and the patient was referred to a higher center for further treatment.
During her stay in the Emergency Department, following are the nursing care measures initiated based on priority
Nursing Care:
1. Nursing diagnosis:
Ineffective airway clearance related to the inflamed airways & alveoli secondary to immune reaction.
Expected outcome:
Patient maintains a patent airway as evidenced by clear lung fields on auscultation and eTCo2 within normal range.
Interventions:
· Monitored the respiratory rate, rhythm, depth, eTCo2 and SaO2
· Ensured the ET tube was well secured with proper measurement
· Provided ventilation with 100% Fio2
· Frequent suctioning done with preoxygenation
· Administered intravenous steroids and nebulization
Evaluation:
Patent airway was maintained with advanced airway management.
2. Nursing Diagnosis:
Impaired gas exchange related to respiratory failure and inflamed airways & alveoli.
Expected Outcome:
Patient maintains optimal gas exchange as evidenced by Arterial Blood Gas values within the usual range.
Interventions:
· Monitored respiratory rate, rhythm, depth, and SaO2
· Auscultated her lungs for adventitious sounds such as wheeze & crackles
· Provided ventilation with 100% Fio2
· Monitored for signs and symptoms of hypoxia (cyanosis)
· Blood sample for gas analysis was taken
· Administered intravenous corticosteroids and bronchodilators through nebulization
Evaluation:
Patient initially maintained optimal gas exchange with advanced airway management, as evidenced by SaO2 between 95-97%.
3. Nursing diagnosis:
Decreased cardiac output related to inflammatory response of blood vessels.
Expected outcome:
Patient maintains optimal blood pressure and adequate urine output.
Interventions:
· Monitored her BP, heart rate and Sao2
· Administered Inotrope as ordered
· Administered intravenous fluids as prescribed
· Corrected Electrolyte and acid-base imbalances
· Urinary Catheterization was done, and urine output was closely monitored every 30 minutes
Evaluation:
Patient was able to maintain BP with the help of three different types of inotropes. Her urine output was minimal.
4. Nursing diagnosis:
Impaired skin integrity (maculopapular rash & blisters) related to the inflammatory reaction.
Expected outcome:
Patient maintains optimal functions of the skin.
Interventions:
· Assessed the condition of the skin thoroughly
· Monitored the temperature of the patient regularly
· Monitored the environmental temperature
· Secured the tubings and invasive lines to prevent damage due to pressure
· Appropriate PPEs were used by the staff members to prevent cross-infection
· Reverse barrier nursing was followed while handling the patient
· Administered intravenous fluids as prescribed 18
Evaluation:
Further damage to the skin integrity was prevented.
CONCLUSION:
DRESS Syndrome is a rare but serious drug-induced reaction that highlights the importance of careful monitoring and awareness of potential adverse effects when taking certain medications. Mainstay of treatment involves the cessation of the causative drug and supportive care interventions such as the use of corticosteroids, wound care, nutritional improvement, and thermoregulation. Early diagnosis and treatment are crucial for improving outcomes and minimizing the potential for long-term complications associated with this condition. A holistic nursing approach is crucial for managing these patients. This includes continual assessment and monitoring of vital signs and systemic functions, along with prompt interventions for life-threatening conditions. It also involves managing complex medical devices and coordinating with an interdisciplinary team to ensure patient stability and recovery.
CONFLICT OF INTEREST:
No conflict of interest.
ETHICAL CLEARANCE:
In this article, the patient’s identity is concealed. (No images of the patient are included.) Given that the patient data is sourced from a teaching institution, we responsibly utilize clinical information to enhance educational initiatives.
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Received on 12.03.2026 Revised on 14.04.2026 Accepted on 15.05.2026 Published on 27.07.2026 Available online from August 01, 2026 Asian J. Nursing Education and Research. 2026;16(3):189-194. DOI: 10.52711/2349-2996.2026.00038 ©A and V Publications All right reserved
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