36: Sweet Syndrome Flashcards
What are the possible outcomes of lesions in a patient with Sweet syndrome?
Lesions may resolve spontaneously or after treatment.
What clinical variant is represented by erythematous-based pustules in Sweet syndrome?
This represents pustular dermatosis.
What clinical variant is represented by lesions that appear as tiny pustules on red papules in Sweet syndrome?
This represents pustular dermatosis.
What diagnostic step is crucial for lesions that mimic necrotizing fasciitis in Sweet syndrome?
A biopsy is crucial to confirm the absence of infectious etiology and identify neutrophilic infiltration.
What is the expected outcome of lesions that coalesce into irregular plaques in Sweet syndrome?
The lesions resolve without scarring, either spontaneously or after treatment.
In what age group is concurrent sterile osteomyelitis reported in Sweet syndrome?
This complication is reported in children.
What phenomenon do lesions at sites of prior biopsies and venipuncture represent in Sweet syndrome?
This represents cutaneous pathergy, a form of skin hypersensitivity.
What is the phenomenon called when new skin lesions appear at the site of a prior healed skin disease in Sweet syndrome?
This is called Wolf’s isotropic response.
What percentage of patients with Sweet syndrome experience recurrent episodes, and what might this indicate in malignancy-associated cases?
1/3 to 2/3 of patients experience recurrent episodes. In malignancy-associated cases, recurrence may signal the return of malignancy.
What variant is characterized by lesions localized to the dorsal hands in Sweet syndrome, and what treatment is effective?
This is the neutrophilic dermatosis of the dorsal hands (pustular vasculitis of the dorsal hands). Systemic corticosteroids and/or dapsone therapy are effective.
What diagnostic step is necessary for erythematous, tender dermal nodules on the legs mimicking erythema nodosum in Sweet syndrome?
Evaluation of one or more new dermal nodules is necessary to establish the correct diagnosis.
What variant is characterized by erythematous, warm lesions mimicking necrotizing fasciitis in Sweet syndrome, and what is absent?
This is the necrotizing variant, characterized by the absence of infectious etiology.
What type of Sweet syndrome is characterized by bullous lesions mimicking pyoderma gangrenosum?
This is malignancy-associated Sweet syndrome.
What variant is associated with a history of obesity and relapsing widespread giant lesions in Sweet syndrome?
This is the giant cellulitis-like variant, associated with obesity and malignancy.
What cardiac involvements are associated with Sweet syndrome in children?
In children, cardiac involvement includes post-inflammatory elastolysis and Takayasu arteritis.
What cardiac involvements are associated with Sweet syndrome in adults?
In adults, it includes coronary artery occlusion.
What central nervous system involvements are associated with Sweet syndrome?
Central nervous system involvements include Neuro-Sweet disease, optic nerve involvement, and bilateral endogenous endophthalmitis.
What complications can arise from Sweet syndrome?
Complications can include secondary infection and recurrence of cancer in malignancy-associated cases.
What diseases may be associated with Sweet syndrome?
Associated diseases include Behçet disease, cancer, erythema nodosum, infections, IBD, and others.
What is the significance of the histiocytoid variant of Sweet syndrome?
The histiocytoid variant is associated with medications and other conditions such as autoimmune diseases.
What is the relationship between Sweet syndrome and leukemia cutis?
Leukemia cutis can occur concurrently with Sweet syndrome, characterized by abnormal neutrophils.
What associated condition might develop in a patient with Sweet syndrome and a history of AML treated with all-trans-retinoic acid?
The patient might develop differentiation syndrome.
What associated conditions should be considered in a patient with Sweet syndrome and a history of thyroid disease?
Associated conditions include IBD, sarcoidosis, and rheumatoid arthritis.
What is the unifying characteristic of Sweet syndrome and concurrent pyoderma gangrenosum?
Both conditions involve an inflammatory infiltrate of mature polymorphonuclear leukocytes.
What is the unifying characteristic of Sweet syndrome and concurrent subcorneal pustular dermatosis?
Both conditions involve an inflammatory infiltrate of mature polymorphonuclear leukocytes.
What age group is reported for concurrent Takayasu arteritis in Sweet syndrome?
This complication is reported in children.
What age group is reported for concurrent coronary artery occlusion in Sweet syndrome?
This complication is reported in adults.
What is the likely underlying cause of concurrent bilateral endogenous endophthalmitis in Sweet syndrome?
The likely underlying cause is a nontuberculous mycobacterial infection.
What organ involvement might concurrent parvovirus B19 infection precede in Sweet syndrome?
This might precede spleen involvement.
What is the likely cause of concurrent systemic inflammatory response syndrome (SIRS) in Sweet syndrome?
The likely cause is Sweet syndrome-associated pulmonary involvement.
What is the unifying characteristic of Sweet syndrome and concurrent erythema elevatum diutinum?
Both conditions involve an inflammatory infiltrate of mature polymorphonuclear leukocytes.
What are the key features of neuro-Sweet disease?
Neuro-Sweet disease can present with self-remitting and reversible parkinsonism.
What are the two hypotheses explaining the phenomenon of concurrent leukemia cutis in Sweet syndrome?
The two hypotheses are ‘secondary’ leukemia cutis and ‘primary’ leukemia cutis.
What are the potential cardiac complications associated with Sweet syndrome in children?
Potential complications include post-inflammatory elastolysis and Takayasu arteritis.
What neurological manifestations can occur in patients with Sweet syndrome?
Neurological manifestations include Neuro-Sweet disease and optic nerve involvement.
How does oral involvement in Sweet syndrome differ between classical and hematologic disorder-associated forms?
In classical Sweet syndrome, oral involvement is uncommon, but it may occur frequently in hematologic disorders.
What are the associated conditions that may occur concurrently with Sweet syndrome?
Associated conditions include Behçet disease, cancer, erythema nodosum, and others.
What is the significance of ‘secondary’ and ‘primary’ leukemia cutis in relation to Sweet syndrome?
‘Secondary’ leukemia cutis refers to circulating immature myeloid precursor cells recruited to the skin due to inflammatory stimuli.
What is the typical age range for the onset of classical Sweet syndrome?
The onset typically occurs between 30 to 60 years of age.
What are the common solid tumors associated with Sweet syndrome?
Common solid tumors include those of the genitourinary organs, breast, and gastrointestinal tract.
What laboratory findings are typically associated with malignancy-associated Sweet syndrome?
Typical findings may include anemia, neutropenia, and abnormal platelet counts.
What cytokine is most frequently associated with drug-induced Sweet syndrome?
G-CSF (Granulocyte Colony-Stimulating Factor) is the cytokine most frequently associated.
What is the significance of detecting Sweet syndrome in the first 6 weeks of life?
It may herald a serious underlying disorder.
What laboratory tests should be considered for a patient with Sweet syndrome and elevated ESR and hepatic serum enzymes?
CBC with leukocyte differential, acute phase reactants, serum chemistries, and urinalysis should be considered.
What might be the underlying cause of lesions localized to sun-exposed areas in Sweet syndrome?
The lesions may be due to photodrug-associated Sweet syndrome or photosensitivity.
What does elevated IL-1, IL-8, and TNF-α in lesional skin suggest about the pathogenesis of Sweet syndrome?
These cytokines suggest an inflammatory response involving neutrophil activation.
What is the likely pathogenesis when febrile upper respiratory tract infection precedes skin lesions in Sweet syndrome?
It suggests bacterial, viral, or tumor antigens as triggers.
What laboratory tests should be performed for a patient with malignancy-associated Sweet syndrome who has anemia and abnormal platelet counts?
CBC with leukocyte differential and platelet count, as well as acute phase reactants, should be performed.
What is the likely mechanism for photodistributed lesions after TMP-SMX intake in Sweet syndrome?
The lesions are likely due to a photodrug-associated Sweet syndrome.
What do elevated G-CSF levels indicate about disease activity in Sweet syndrome?
Elevated G-CSF levels are closely associated with active Sweet syndrome.
What are the key diagnostic criteria for classical Sweet syndrome?
Key criteria include being most common in women and onset typically between 30-60 years of age.
What cytokines are involved in the pathogenesis of Sweet syndrome?
Cytokines involved include G-CSF, GM-CSF, IFN-γ, IL-1, IL-3, IL-6, and IL-8.
What laboratory findings are typically associated with malignancy-associated Sweet syndrome?
Findings include peripheral leukocytosis, anemia, neutropenia, and elevated ESR.
What is the significance of G-CSF in the context of drug-induced Sweet syndrome?
G-CSF is the most frequently observed agent associated with drug-induced Sweet syndrome.
What are the signs of Sweet syndrome?
Presence of hematuria and proteinuria.
What tests may be performed in cases with CNS involvement?
Thyroid function tests and CSF analysis may also be performed.
What is the significance of G-CSF in Drug-Induced Sweet Syndrome?
G-CSF is the most frequently observed agent associated with drug-induced Sweet syndrome. Its elevation is often linked to the clinical presentation of Sweet syndrome in patients who have undergone treatment with certain medications, indicating a potential immune response or neutrophil activation related to the drug exposure.
What is the histological characteristic of Histiocytoid Sweet syndrome?
Histiocytoid Sweet syndrome is characterized by an inflammatory dermatosis with histiocytes internally arranged between dermal collagen bundles.
What are the clinical features of Classical Sweet syndrome?
Classical Sweet syndrome may present with spontaneous resolution without any therapeutic intervention, while some patients may have persistent lesions for weeks to months.
How does Behçet disease differ from Sweet syndrome in terms of HLA typing?
Behçet disease is associated with HLA 51, whereas Sweet syndrome is associated with HLA 54, making HLA typing an important differentiator between the two conditions.
What are the potential outcomes for patients with Drug-induced Sweet syndrome after discontinuation of the associated medication?
Patients with Drug-induced Sweet syndrome may experience spontaneous resolution of symptoms after discontinuation of the associated medication, although some may have persistent lesions for weeks to months.
What are the common extraneous sites affected by neutrophilic inflammation in Sweet syndrome?
Common extraneous sites affected by neutrophilic inflammation in Sweet syndrome include the aorta, bones, intestines, liver, lungs, and muscles.
What diagnostic step is necessary to differentiate Sweet syndrome from erythema nodosum?
A biopsy is necessary to differentiate Sweet syndrome from erythema nodosum.
What histologic variant might lesions in Sweet syndrome mimic if they resemble methotrexate-induced rheumatoid papules?
This might represent the histiocytoid variant.
What is the histological feature of the histiocytoid variant in Sweet syndrome?
This is the histiocytoid variant, often associated with malignancy or drug-induced cases.
What are the key histological features that differentiate Histiocytoid Sweet syndrome from other inflammatory dermatoses?
Histiocytoid Sweet syndrome is characterized by:
- Dermal infiltrate composed of immature granulocytes that are histiocytic mononuclear cells.
- Small cells resembling neutrophils.
- Staining for CD15, CD43, CD35 (LCA), CD68, HAM56, lysozyme, and MAC 387.
- Histopathological arrangement of histiocytes between dermal collagen bundles.
How does the clinical course of Classical Sweet syndrome differ from Malignancy-associated Sweet syndrome?
The clinical course of these two types of Sweet syndrome varies significantly:
1. Classical Sweet syndrome:
- Some patients experience spontaneous resolution without any therapeutic intervention.
- Others may have persistent lesions lasting for weeks to months.
2. Malignancy-associated Sweet syndrome:
- Successful management of the underlying cancer can lead to clearing of related dermatosis.
- The presence of malignancy may complicate the course, requiring careful monitoring.
What are the important differential diagnoses to consider when evaluating a patient with suspected Sweet syndrome?
Key differential diagnoses for Sweet syndrome include:
- Behçet disease: Differentiated by HLA typing (Behçet: HLA 51, Sweet: HLA 54).
- Familial Mediterranean fever: Requires biopsy to differentiate from giant cellulitis-like Sweet syndrome.
- Leukemia cutis: Associated with abnormal neutrophils.
- Drug-induced Sweet syndrome: Consider medications that may trigger symptoms.
- Other inflammatory dermatoses: Such as interstitial granulomatous dermatitis with arthritis and methotrexate-induced rheumatoid papules.
What is the therapeutic mainstay for Sweet syndrome?
The therapeutic mainstay for Sweet syndrome is systemic corticosteroids. They improve symptoms and resolve mucocutaneous lesions after initiation of therapy.
What are the potential side effects of potassium iodide used in the treatment of Sweet syndrome?
The potential side effects of potassium iodide include vasculitis and hypothyroidism.
Which drugs are considered as second-line systemic agents for Sweet syndrome?
The drugs considered as second-line systemic agents for Sweet syndrome include:
1. Indomethacin
2. Clofazimine
3. Cyclosporine
4. Dapsone
Cyclosporine and dapsone can be used in combination.
What laboratory evaluations are recommended for malignancy workup in Sweet syndrome?
Recommended laboratory evaluations for malignancy workup in Sweet syndrome include:
- CEA level
- CBC with leukocyte differential and platelet count (check every 6-12 months)
- Pap test in women
- Serum chemistries
- Stool guaiac slide test
- Urinalysis
- Urine culture.
What is the significance of CBC with leukocyte differential in the context of Sweet syndrome?
The CBC with leukocyte differential is significant as it helps monitor for peripheral leukocytosis with neutrophilia and elevated ESR, which are consistent laboratory findings in Sweet syndrome. It should be checked every 6-12 months due to the potential for dermatosis-related skin lesions to precede hematologic malignancy by as long as 11 years.
What are the first-line treatments for localized lesions in Sweet syndrome?
The first-line treatments for localized lesions in Sweet syndrome include:
- Topical corticosteroids (e.g., Clobetasol 0.05%)
- Intralesional corticosteroids (e.g., Triamcinolone acetonide) at a dose between 0.3 mg/cc to 10 mg/cc.
What is the first-line treatment for a patient with Sweet syndrome presenting with fever, tender erythematous skin lesions, and neutrophilia?
Systemic corticosteroids are the first-line treatment because they lead to prompt improvement of symptoms and resolution of mucocutaneous lesions.
What is the clinical significance of the initial appearance of dermatosis-related skin lesions in Sweet syndrome?
The initial appearance of dermatosis-related skin lesions in Sweet syndrome has been reported to precede the diagnosis of Sweet syndrome-associated hematologic malignancy by as long as 11 years.
What is the percentage of women affected by Sweet Syndrome in classical cases?
80% of patients with classical Sweet Syndrome are women.
What are the common clinical symptoms associated with Sweet Syndrome?
Common clinical symptoms include:
- Fever: 80-90%
- Musculoskeletal involvement: 12-56%
- Ocular involvement: 17-72%.
What laboratory finding is most commonly elevated in patients with Sweet Syndrome?
Elevated erythrocyte sedimentation rate is found in 90% of patients with Sweet Syndrome.
Which organ systems can be affected by Sweet Syndrome according to the extraneous manifestations?
Affected organ systems include:
- Central nervous system
- Ears
- Eyes
- Kidneys
- Intestines
- Liver
- Heart
- Lung
- Mouth
- Muscles
- Spleen.
What is the recurrence rate of Sweet Syndrome in classical cases?
The recurrence rate of Sweet Syndrome in classical cases is 30%.
How does the occurrence of ocular involvement in Sweet Syndrome compare between classical and hematologic malignancy forms?
Ocular involvement occurs in 17-72% of classical cases and 7% of hematologic malignancy cases, indicating a higher prevalence in classical forms.
What are the common laboratory findings in patients with Sweet Syndrome?
Common laboratory findings include:
1. Neutrophilia: 80% in classical cases
2. Elevated erythrocyte sedimentation rate: 90% in classical cases
3. Anemia: Infrequent in 82% of cases
4. Abnormal renal function: 11-50% of cases show abnormalities.
What are the extraneous manifestations of Sweet Syndrome in the lungs?
In the lungs, Sweet Syndrome can present with:
- Bronchi (main stem) showing red-bordered pustules with neutrophilic inflammation
- Pleural effusion showing abundant neutrophils without microorganisms
- Chest radiograph abnormalities indicating pulmonary tissue with neutrophilic inflammation.
What is the clinical significance of musculoskeletal involvement in Sweet Syndrome?
Musculoskeletal involvement occurs in 12-56% of cases, which can lead to significant discomfort and may complicate the clinical picture, necessitating careful management of symptoms.
What are the conditions probably associated with Sweet Syndrome?
Conditions probably associated with Sweet Syndrome include:
- Cancer: Hematologic malignancies (most commonly acute myeloid leukemia) and solid tumors (most commonly carcinomas of the genitourinary organs, breast, and gastrointestinal tract).
- Infections: Most commonly of the upper respiratory tract (streptococcal) and gastrointestinal tract (salmonellosis and yersiniosis).
- Medications: Most commonly granulocyte colony-stimulating factor.
- Pregnancy.
- Vaccinations: Bacille Calmette-Guérin (BCG) and influenza.
What are some conditions possibly associated with Sweet Syndrome?
Conditions possibly associated with Sweet Syndrome include:
- Behçet disease
- Erythema nodosum
- Relapsing polychondritis
- Sarcoidosis
- Thyroid disease: Grave disease and Hashimoto thyroiditis.
- Antineutrophilic cytoplasmic antibody–associated vasculitis
- Ankylosing spondylitis
- Anti–factor Vll inhibitor
- Antiphospholipid syndrome
- Aplastic anemia
- Aortitis: Takayasu arteritis.
- Autoimmune disorders: Cholangitis, dermatomyositis, lupus erythematosus, pemphigus vulgaris, and Sjögren syndrome.
What are the first-line systemic treatments for Sweet Syndrome?
First-line systemic treatments for Sweet Syndrome include:
1. Prednisone: 1 mg/kg/day (usually ranging from 30-60 mg) as a single oral, morning dose. Taper dose to 10 mg/day; some patients may require 2-3 months of treatment or intravenous therapy.
2. Methylprednisolone sodium succinate: Intravenously administered up to 100 mg/day over 1 or more hours, daily for 3-5 days.
3. Potassium iodide: Administered orally as a 30 mg enteric-coated tablet, TID for a daily dose of 90 mg or as a saturated solution (1 gm of iodide per potassium iodide (SSI), beginning at a dose of 3 drops TID to 450 mg/day and increasing by 1 drop TID, typically to a final dose of 21 drops/day (1050 mg/30.3 drops/day or 1500 mg).
4. Dapsone: Administered as either monotherapy or in combination therapy.
What are some second-line systemic treatments for Sweet Syndrome?
Second-line systemic treatments for Sweet Syndrome include:
1. Indomethacin: Administered orally at a dose of 150 mg for 7 days, and then 100 mg/day for 14 days.
2. Clofazimine: Administered orally at a daily dose of 200 mg for 4 weeks, and then 100 mg/day for 4 weeks.
3. Cyclosporine: Administered as monotherapy or as a second-line agent after failure of first-line therapy or as a corticosteroid-sparing agent.
4. Dapsone: Administered as either monotherapy or in combination therapy.
What is the initial dose of potassium iodide (SSI)?
The initial dose is 3 drops TID to 450 mg/day.
How is the dose of potassium iodide (SSI) increased?
The dose is increased by 1 drop TID, typically to a final dose of 21 drops/day (1050 mg/30.3 drops/day or 1500 mg).
What are some second-line systemic treatments for Sweet Syndrome?
Second-line systemic treatments include Indomethacin, Clofazimine, Cyclosporine, and Dapsone.
What is the dosage regimen for Indomethacin?
Indomethacin is administered orally at a dose of 150 mg for 7 days, and then 100 mg/day for 14 days.
What is the dosage regimen for Clofazimine?
Clofazimine is administered orally at a daily dose of 200 mg for 4 weeks, and then 100 mg/day for 14 days.
What is the dosage regimen for Cyclosporine?
Cyclosporine is administered as monotherapy or as a second-line agent after failure of first-line therapy; initial oral dose ranges from 2 mg/kg/day to 4 mg/kg/day to 10 mg/kg/day.
What is the dosage regimen for Dapsone?
Dapsone is administered as either monotherapy or in combination therapy; initial oral dose ranges from 100 mg/day to 200 mg/day.
