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| ARTICLE |
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| Year : 2018 | Volume
: 19
| Issue : 1 | Page : 31-37 |
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Sweet syndrome
Sasikala R Umesh1, Amalorpavamari Lucas2, Selva T Chacko2, Aruna S Rajan3
1 Reader, College of Nursing, CMC, Vellore, India
2 Professor, College of Nursing, CMC, Vellore, India
3 Charge Nurse CMC, Vellore, India
| Date of Web Publication | 11-Jun-2020 |
Correspondence Address:
 Source of Support: None, Conflict of Interest: None
Sweet Syndrome also known as Acute Febrile Neutrophilic Dermatosis is a skin disorder characterized by abrupt onset of tender, red-to-purple papules and nodules that coalesce to form plaques on the skin. Sweet Syndrome is uncommon but not rare. In general, Sweet Syndrome responds dramatically to oral corticosteroids and may improve or resolve with treatment of the underlying condition. Dermatosis generally resolves but may persist indefinitely in some patients and can be difficult to manage because of pain and skin breakdown. Recurrence of the syndrome is frequently encountered. This article outlines the disease process and its management. A case report is presented focusing on the nursing management of a patient with Sweet Syndrome using nursing process.
Keywords: Sweet Syndrome, dermatosis, case report
How to cite this article:
Umesh SR, Lucas A, Chacko ST, Rajan AS. Sweet syndrome. Indian J Cont Nsg Edn 2018;19:31-7 |
| Introduction | |  |
Sweet Syndrome (SS) was first described by Robert Douglas Sweet in the year 1964 and is also known as Acute Febrile Neutrophillic Dermatosis. It is a skin disorder characterized by the accumulation of neutrophils in the dermis of skin (National Organization of Rare Diseases, 2015). SS ranges from classic Sweet disease, which occurs in young women after a mild respiratory illness to a more aggressive neutrophilic process which may be associated with other inflammatory diseases or malignancy. Fever and leukocytosis frequently accompany the cutaneous lesions. In addition inflammatory involvement in the eyes, musculoskeletal system and internal organs may be present. Because this condition can be associated with many other diseases including malignancy, the patients overall prognosis depends on the underlying cause (Merola, Callen, & O’Ofori, 2018).
SS affects no more than 3 people per 10,000 (Zamanian & Ameri, 2007). According to some ofthese studies, SS could affect as few as 0.27 people per 10,000 in some countries (treating figures from 1990 in Scotland as representative of the UK), or up to 3 people per 10,000 in others (Iran). It is more common in countries where infection is poorly controlled as it can be triggered by infections (Ginarte & Toribio, 2011; Zamanian & Ameri, 2007).
| Epidemiology | | |
Although individuals between the ages of 30 and 60 most frequently develop classical SS, infants, children and older adults may also be affected. The age of onset tends to be older especially among patients with malignancy-associated SS. It is more common in females than males in a ratio of 3:1. Most cases have occurred between the ages of 30 and 50 years, however, reported age groups range from 2 to 75 years (Vashisht & Holmes, 2018).
| Pathogenesis | | |
The pathogenesis of SS is not well understood. Factors theorized to contribute to the development of SS include hypersensitivity reaction, cytokine dysregulation, and genetic susceptibility (Merola et al. 2018).
- Hypersensitivity Reaction
An immune reaction to bacterial, viral, tumor or other antigens could influence the development of SS through stimulating the production of cytokines that promote neutrophil activation and infiltration.
- Cytokine Dysregulation
Certain cytokines and chemokines may contribute to the initiation and propagation of the inflammatory response in SS.
- Genetic Susceptibility
Abnormalities in chromosome 3q (long arm) has been reported in association with SS.
| Classification | | |
According to Merola et al. (2018) SS is classified as follows
1. Classical Sweet Syndrome (CSS)
It is also referred to as idiopathic SS. It is triggered by infections, inflammatory bowel disease, pregnancy, primary immunodeficiencies, and autoimmune conditions (Behçet’s disease, sarcoidosis, autoimmune thyroid disease, connective tissue disorders including systemic lupus erythematosus and dermatomyositis).
2. Malignancy Associated Sweet Syndrome (MASS)
It can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose SS related hematologic dyscrasia or solid tumor was previously undiscovered. MASS is most commonly related to Acute Myelogenous Leukemia.
3. Drug-induced Sweet Syndrome
Multiple drugs may contribute to SS. Granulocyte Colony Stimulating Factor is the most common contributory medication. SS usually develops about two weeks after drug exposure in patients who lack a prior history of exposure to the inciting drug. Recurrence of the syndrome usually develops after re-exposure to the inciting drug.
| Clinical Manifestations | | |
Clinical manifestations include oral cavity, cutaneous, and extracutaneous manifestations
Oral cavity
Patient may present with oral ulcers particularly on the buccal mucosa or tongue. Additional oral findings may include bullae, vesicles, gingival hyperplasia, necrotizing ulcerative periodontitis, and tongue swelling (see [Figure 1]). | Figure 1: Oral cavity manifestations of Sweet Syndrome (Source: Oakley, 2015)
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Cutaneous
Tender, edematous inflamed papules, plaques, and nodules may be seen on skin (see [Figure 2]). | Figure 2: Cutaneous manifestations of Sweet Syndrome (Source: Oakley, 2015)
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Extracutaneous
Ocular inflammation, encephalitis, aseptic meningitis, myocarditis, aortitis and aortic stenosis, alveolitis, pleural effusions, hepatitis, hepatomegaly, neutrophilic inflammation of intestines, splenomegaly, glomerulonephritis, and sterile osteomyelitis are other manifestations that could be associated with SS. Other commonly associated symptoms include fever, arthralgias, malaise, headache, and myalgia.The diagnostic criteria is outlined in [Table 1]. | Table 1: Diagnostic criteria for classical Sweet Syndrome versus Drug-induced Sweet Syndrome
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| Diagnosis | | |
Clinical assessment
A rapid onset of skin lesions and the presence of associated symptoms (e.g., painful skin lesions, fever, & malaise) in association with a recent infection, malignancy, pregnancy or inflammatory bowel disease raises clinical suspicion for the diagnosis.
| Laboratory Studies | | |
Biopsy
It is a major diagnostic criterion for SS. The characteristic features in the biopsy specimen include
- prominent edema in the superficial dermis
- dense infiltrate of neutrophils in the upper and mid- dermis with sparing of the epidermis leukocytoclasis (damage caused by nuclear debris from infiltrating neutrophils)
- endothelial swelling
- absence of vasculitis
Serologic and other tests
Complete Blood Count with platelets and differential, complete metabolic panel, ESR, CRP, urinalysis and pregnancy test in women of childbearing age are the blood tests done to find associated infection and inflammatory responses.
Evaluation for malignancy
As there is a clinical suspicion for an underlying malignancy, patient has to be evaluated for the type of malignancy. The presence of both major and two minor clinical findings have been proposed as criteria for diagnosis (see [Table 2]) (Von den Driesch, 1994).
| Treatment | | |
According to Freedberg and Einsen (2003) management of SS includes systemic therapy, topical application, and treating the underlying causes.
1. Systemic Therapy
Corticosteroids are the first-line therapy and therapeutic gold standard for SS. Prednisolone is extremely and rapidly effective in doses of 1 mg/kg/day and will result in a dramatic clinical response in 24-48 hours. For severe cases, pulse systemic steroids is considered monthly either with intravenous Methyl Prednisolone or oral Dexamethasone daily for five consecutive days. Immunosuppressants like Dapsone and Colchicines may be effective as first-line treatment in mild cases or as an adjunctive treatment when steroid-sparing agents are necessary. In severe or refractory disease Cyclosporine A is given at 5-10 mg/kg per day. Infliximab 5 mg/kg/day at weeks 0, 2, and 6 or Etanercept 50 mg twice weekly may be considered as rescue therapy in patients failing the above treatments. Other effective agents that may be used alone or in combination with other medications when needed for refractory cases are Minocycline, Clofazamine, Thalidomide, Mycophenolatemofetil and SSKI(Saturated Solution of Potassium Iodide).
2. Topical Application
Topical and/or intralesional corticosteroids may be effective as either monotherapy or adjuvant therapy. High- potency topical steroids (e.g. Clobetasol propionate 0.05%) and Intralesional glucocorticoids (e.g., Triamcinolone Acetonide 3.0-10 mg) may be efficient for treating localized lesions
3. Treating the Underlying Cause
Successful therapy of the underlying disorder may promote resolution of SS and prevent recurrences.
| Case Report | | |
Mrs. S, a 74 year old lady presented with sore throat and coryza for the past three weeks followed by pain, swelling, and redness over the outer aspect of the right thigh and distal end of fingers of the left hand. Mrs. S was previously diagnosed with diffuse large B cell lymphoma and was treated with chemotherapy. On examination she had pallor, bilateral pitting edema, and carbuncle like features over the outer aspect of the right thigh just above the knee. She underwent debridement, which flared the lesions further leading to bullae eruptions. She was transferred to ICU for respiratory acidosis and hypotension management and was received back to the ward. Skin biopsy showed neutrophilic dermatosis and she was started on Thalidomide 100 mg once daily. During her stay in the hospital, she developed multiple skin lesions and oral lesions. Tab. Prednisolone dose was increased from 35 mg to 50 mg/day. Meanwhile she developed further new skin lesions with local infection and her blood culture grew Candida. She was treated with IV Levofloxacin 750 mg /od and Flucanazole100 mg / od for 14 days. The treatment options and prognosis were explained to the family members, who opted for supportive care. Skin care, pain management, nutritional support, and infection prevention was targeted in providing care.
| Nursing Management | | |
Nursing care of Mrs. S is discussed using nursing process approach (Gulanick & Myers, 2014).
1. Nursing Diagnosis: Impaired skin and mucosal integrity (oral mucosa) related to ruptured bullae and denuded areas of the skin
Expected Outcome: Improved skin and mucosal integrity as evidenced by satisfactory wound healing
Interventions
Mrs. S was assessed for the skin and mucosal integrity and monitored for the development of new lesions daily. Invasive lines were secured using bandages in order to prevent developing new blisters from usage of adhesive tape. Position was changed every 2 hours based on the location of the skin lesions. Bed cradle and sterile linen were used to prevent skin irritation and infection. Debridase ointment was applied over the slough to dissolve damaged tissue. Potassium Permanganate compress and medicated bath with 20% Chlorhexidine scrub were provided to reduce bacterial population of the wound surface.
After bath, topical application such as Flutibact (antiseptic) and 0.05% Tenovate (steroid) were applied twice daily. Duoderm, hydrocolloid dressing was done for right elbow ulcer. Inorder to promote wound healing 20% of serum albumin was administered. Oral hygiene was provided four times a day using normal saline and soda bicarbonate mouth wash followed by coconut oil swish. Candid mouth paint (1%) (antifungal) and 0.1% Elocon (topical steroid) were applied on the oral lesion to reduce inflammation and itching.
Evaluation: Though the old ulcers healed, Mrs. S continued to have new ulcers at multiple sites requiring continuous care.
2. Nursing Diagnosis: Acute pain related to denuded skin, oral lesions and infected ulcers
Expected Outcome: Pain is minimized as evidenced by verbalization of reduction of pain less than 2 on the numerical pain rating scale
Interventions
Mrs. S was assessed for the characteristics of pain. She was positioned comfortably with extra pillows to relieve pressure at the sites of blisters and open wounds. Clean, wrinkle free bed with soft mattress and pillow were provided to avoid excessive irritation. Fentanyl 50 mcg patch was applied every 72 hours. Inj. Tramadol 50 mg was administered half an hour before skin and oral care. Zytee gel and Mucaine gel (local anesthetic agents) were applied to oral ulcers half an hour before every meal.
Evaluation: The pain was minimized as evidenced by reduction in pain score to 2. Mrs. S cooperated well during the care. She also expressed her increased comfort level.
3. Nursing Diagnosis: Imbalanced nutrition less than body requirement related to hypermetabolism, non healing ulcers, loss of protein from dermal wounds, nausea and inability to take diet orally secondary to painful oral lesions
Expected Outcome: Nutritional status is improved as evidenced by stable weight, presence of wound healing and improved feeling of wellbeing
Interventions
Mrs. S’ nutritional status was assessed. On admission her BMI was 26 kg / m2. There was a decline in BMI to 24.2 kg / m2 in 2 weeks. Dietary recommendations was obtained and a 1800 kcal, 100 gm protein diet was planned and provided. To reduce nausea Inj. Emeset 8 mg/IV was administered as per order. Diet was provided according to her preference and tolerance as liquids, bland and soft solid diet. Zytee gel and Mucaine gel were applied to oral ulcers half an hour before meal. Oral hygiene was provided four times a day. Inj. Albumin 20% was transfused as her serum albumin level was 1.9 g /dl. Strict intake and output was maintained.
Evaluation: There was a decline in BMI in the 2 weeks of her hospitalization. Old wounds healed. Mrs. S’ oral intake increased over two weeks. Serum albumin level increased from 1.9 g / dl to 2.1g / dl.
4. Nursing Diagnosis: Impaired body image related to multiple skin lesions and hyperpigmentation
Expected Outcome: Positive body image is enhanced as evidenced by absence of negative comments about her appearance
Interventions
Good rapport with Mrs. S was developed by answering her questions. Her emotional status regarding change in the physical appearance was assessed. She was explained about the lesions, dryness, healing of the lesions and treatment. Meticulous skin care was provided for quick granulation. She was prevented from exposing the body unnecessarily.
Evaluation: The lesions on the lip healed completely which gave Mrs. S a positive feeling. However she developed new skin lesions as some of the existing lesions were healing.
5. Nursing Diagnosis: Risk for ineffective individual coping and family related to recurrence of new lesions and prolonged hospitalization
Expected outcome: Effective coping is enhanced as evidenced by following the given instructions, adhering to the treatment and ability to perform the activities of daily living
Interventions
Mrs. S and her family were encouraged to ventilate their feelings and identify their strengths. Their doubts regarding the disease and treatment were cleared. Non- judgmental attitude was exercised. Spiritual support and counselling was offered.
Evaluation: Mrs. S and her family members were depressed when she continued to develop new lesions. With adequate support and counselling their coping improved and she and her family chose palliative management.
6. Nursing Diagnosis: Risk for complications related to ruptured skin and mucosal membrane and prolonged restricted mobility
Expected outcome: Complications are prevented as evidenced by absence of the manifestations of respiratory distress, infection, deep vein thrombosis, and constipation
Interventions
Respiratory distress
Monitored her respiratory rate, rhythm and saturation. Instructed and assisted her to deep breathe and cough or “huff” every 1 -2 hours. Maintained adequate hydration by encouraging fluid intake upto 2000 ml in a day. Kept the head end elevated. Administered nebulization (Salbutamol 5 mg, Ipravent 0.5 mg) as prescibed. Provided chest physiotherapy and incentive spirometry exercises. Administered oxygen as per the order based on the saturation.
Evaluation: Mrs. S developed respiratory acidosis (ABG: PH: 7.2, PaCO2 : 55 mm Hg, PaO2: 55 mm Hg, HCo3: 20mEq/L). She was managed with non invasive ventilation in ICU. Later she developed respiratory distress and her oxygen requirement increased from 2 liters to 15 liters/min.
Infection
Stringent hand hygiene technique was followed. Vital signs were monitored two hourly to note for rise in temperature and pulse. Seperate equipment were used to provide care. Blood counts were monitored. Strict aseptic technique was followed while administering injections and handling invasive lines. Catheter care was provided every 4 hours. Administered antibiotics Inj. Piptaz 4.5 gm Q8H, Inj. Vancomycin 1gm od, Inj. Meropenam1gm Q8H, Inj. Levofloxacin 750 mg od, Tab. Colchin 0.5 mg od and Inj. Flucanazole 100mg od.
Local Infection
Meticulous skin and mouth care was provided. Strict aseptic technique was followed during skin care and oral care and reverse barrier nursing technique was employed. Bed linen was autoclaved. Visitors entry was restricted. Topical application were applied as per the order. Hibiscrub bath OD, Flutibact ointment BD, Tenovate BD, Candid mouth paint BD were continued.
Evaluation: Mrs. S developed systemic and local infection. Skin lesion grew proteus, klebsilla and pseudomonas. Her highest blood count was 23100/cc mm. Her blood culture grew candida and gram negative organism. She was treated with antimicrobials. She responded to antibiotics in the beginning of her hospitalization. Later Mrs. S succumbed to a gram negative sepsis.
Deep Vein Thrombosis
Homan’s sign was assessed daily. Adequate fluid intake was ensured. Passive exercises were encouraged. Physiotherapy was provided and she was assisted for ambulation. Encouraged and ensured adequate fluid intake.
Evaluation: Mrs. S did not develop Deep Vein Thrombosis.
Constipation
Assessed the bowel movement. Administered stool softener. Assisted in ambulation. Administered enema as per the order. Mrs. S developed constipation and she received Syr. Cremaffin 15ml hsod and Glycerine enema prn.
Evaluation: Mrs. S was able to pass stools with stool softeners and enemas.
Mrs. S further developed skin lesions and succumbed to sepsis. She passed away in the hospital ward.
| Conclusion | | |
SS is a benign condition. If left alone, the lesions will resolve spontaneously but may recur in upto 30% of patients. After initiation of treatment with systemic corticosteroids, there is a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions. The prognosis depends on the associated cause and response of the lesion to treatment. Infection is the leading cause of death in patients with blistering diseases. Protective isolation measures and standard precautions are warranted to improve outcomes in patients with Sweet Syndrome. An individualized and holistic approach that is based on knowledge regarding the disease is needed when caring for a patient with Sweet Syndrome.
Conflicts of Interest: The authors have declared no conflicts of interest.
| References | | |
| 1. | Freedberg, I., & Einsen, A. (2003). Fitzpatrick’s dermatology in general medicine. Tutzing: McGraw Hill.  |
| 2. | Ginarte, M. & Toribio, J. (2011) Autoimmune disorders current concepts and advances from bedside to mechanistic insights: Sweet Syndrome. London : Infotech Limited. |
| 3. | Gulanick, M., & Myers, J. L. (2014). Nursing care plans: Diagnoses, interventions, and outcomes. Philadelphia: Elsevier Health Sciences. |
| 4. | |
| 5. | |
| 6. | |
| 7. | Vashisht, P., & Holmes, H. (2018). Sweet Syndrome. In Stat Pearls: Treasure Island. |
| 8. | Von den Driesch, P. (1994). Sweet’s syndrome (Acute Febrile Neutrophilic Dermatosis). Journal of the American Academy of Dermatology, 31(4), 535-556. |
| 9. | Walker, D. C., & Cohen, P. R. (1996). Trimethoprim- sulfamethoxazole-associated acute febrile neutrophilic dermatosis: Case report and review of drug-induced Sweet’s syndrome. Journal of the American Academy of Dermatology, 34(5), 918-923. |
| 10. | Zamanian, A., & Ameri, A. (2007). Acute febrile neutrophilic dermatosis (Sweet’s syndrome): A study of 15 cases in Iran. International Journal of Dermatology, 46(6), 571-574. |
[Figure 1], [Figure 2]
[Table 1], [Table 2]
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