DIAGNOSIS OF SWEET SYNDROME
Sweet syndrome ka diagnosis symptoms aur tissue samples (biopsy) ke basis par hota hai. Is syndrome ki ek khaas baat yeh hai ki skin ke ghaav bina daag ke theek ho jaate hain, aur tissue samples mein blood vessel inflammation nahi hoti. Yeh isliye important hai kyunki yeh kisi aur serious bimari, jaise cancer, ka indication ho sakta hai, aur jaldi diagnosis aur treatment zaroori hai.
CLASSIC YA IDIOPATHIC SWEET SYNDROME:
Iska diagnosis karne ke liye "major criteria" aur kam se kam do "minor criteria" meet karni hoti hain.
Major Criteria:
1. Ekdum se dardnaak laal danay ya dher saare chhote bumps, kabhi kabhi blisters ya skin mein doosre badlav ke saath.
2. Biopsy dikhati hai ki white blood cells skin mein gather ho gaye hain, bina blood vessels mein inflammation ke.
Minor Criteria:
1. Koi underlying condition jaise infection, vaccination, inflammatory disease, cancer, ya pregnancy se sambandh.
2. Bukhaar 38°C (100.4°F) se zyada.
3. Lab results mein abnormalities jaise elevated ESR (inflammation marker), high C-reactive protein, ya increased white blood cells/neutrophils.
4. Steroid treatment se accha response.
DRUG-INDUCED SWEET SYNDROME:
Is type ko diagnose karne ke liye yeh paanchon criteria meet honi chahiye:
1. Ekdum se dardnaak laal bumps ya ghaav.
2. Biopsy dikhati hai white blood cells without blood vessel inflammation.
3. Bukhaar 38°C (100.4°F) se zyada.
4. Symptoms kisi particular dawa lene ke baad ya drug challenge ke baad shuru hote hain.
5. Symptoms dawa band karne ke baad ya steroid treatment se theek hote hain.
DIFFERENTIAL DIAGNOSIS
- Allergic Contact Dermatitis: Allergens ke exposure ke baad delayed hypersensitivity reaction hota hai.
- Cellulitis: Ek bacterial skin infection hoti hai jisme redness, swelling, aur garmi hoti hai, aur systemic symptoms bhi ho sakte hain.
- Behçet Disease: Oral aur genital ulcers ke saath hoti hai, skin lesions jaise erythema nodosum ya pseudofolliculitis ke saath.
- Herpes Simplex: Vesicular lesions commonly lips ya genitals par dikhte hain.
- Drug Eruptions: Drug-induced skin reactions kaafi diverse hoti hain, aur recent medication ka history hota hai.
- Erythema Multiforme: Targetoid lesions dikhte hain aur infections ya medications ke kaaran ho sakti hai.
- Erythema Nodosum: Painful, red nodules lower legs par dikhte hain, jo systemic conditions ke saath associated hoti hain.
- Pyoderma Gangrenosum: Rapidly badi hone wali, dardnaak ulcers, jisme neutrophilic infiltrate hota hai, aur systemic disease se associated ho sakti hai.
Yeh differential diagnoses Sweet syndrome ko doosri bimariyon se distinguish karne mein madadgar hain.
LABORATORY STUDIES
1. Complete Blood Cell (CBC) Count with Differential:
- Maqsad: Neutrophilia detect karna aur hematologic disorders ya malignancy ko screen karna.
- Findings: Neutrophilia typical hota hai; neutropenic patient mein neutrophilia ka absence Sweet syndrome ko rule out nahi karta. Anemia aur thrombocytopenia underlying malignancy ka indication de sakti hai.
- Follow-up: Agar CBC count mein koi abnormality ho to bone marrow biopsy ka suggestion diya ja sakta hai.
2. Erythrocyte Sedimentation Rate (ESR) aur C-Reactive Protein (CRP):
- Maqsad: Inflammation levels assess karna.
- Findings: Elevated ESR aur CRP aksar common hote hain lekin nonspecific hote hain.
3. Urinalysis:
- Maqsad: Renal involvement ka pata lagana.
- Findings: Proteinuria ya hematuria ho sakti hai.
4. Liver Functions Test:
- Maqsad: Liver function evaluate karna.
- Findings: Nonspecific elevation of hepatic enzymes ho sakti hai.
5. Antineutrophilic Cytoplasmic Antibodies (ANCAs):
- Maqsad: Autoimmune components investigate karna.
- Findings: Kuch patients mein present ho sakti hain, lekin consistently nahi hoti.
6. Cultures:
- Maqsad: Infections ko rule out karna.
- Findings: Bacteria, fungi, aur mycobacteria ke liye cultures obtain kiye jaane chahiye.
Imaging Studies:
1. Chest Radiograph:
- Maqsad: Pulmonary involvement evaluate karna agar symptoms ho.
- Findings: Pulmonary symptoms systemic corticosteroids se theek ho jaate hain.
2. Systemic Evaluation:
- Maqsad: Underlying malignancy ka investigation karna, especially agar ulcerative lesions, oral lesions, ya abnormal blood counts ho.
3. FDG Positron-Emission Tomography (PET):
- Maqsad: Myeloproliferative disorders aur solid tumors ka assessment.
- Findings: Early malignancies ko evaluate karne ke liye useful hoti hai.
Procedures:
1. Skin Biopsy:
- Maqsad: Diagnosis confirm karna.
- Findings: Reticular dermis mein dense neutrophilic infiltrate; massive papillary dermal edema hoti hai. True vasculitic changes absent hoti hain.
2. Bone Marrow Aspiration:
- Maqsad: Myelodysplastic disease detect karna.
- Indications: Agar CBC count mein abnormality ho ya atypical bullous/ulcerative Sweet syndrome ho.
3. Cancer Screening aur Inflammatory Bowel Disease ka Evaluation:
· Maqsad: Underlying causes ko identify karna, khaaskar bullous ya ulcerative lesions wale patients mein.
Histologic Findings:
Classic Pattern: Reticular dermis mein dense
neutrophilic infiltrate aur massive papillary dermal edema hota hai.
Leukocytoclastic nuclear debris present ho sakti hai. True vasculitic changes
absent hoti hain.
Epidermis:Usually spared hoti hai, lekin spongiosis aur subcorneal pustules dekhe ja sakte hain.
Subcutaneous Involvement: Rare hoti hai, lekin agar present ho, to reticular dermis mein extensive involvement hoti hai.
Variants:
1. Histiocytoid Sweet Syndrome:
· Features: Isme histiocytoid cells hoti hain, jo kabhi kabhi galti se histiocytes samjhe jaate hain.
· Differential: Leukemia cutis.
2. Doosre Variants: Lymphocytic, subcutaneous, aur cryptococcoid types.
TREATMENT & MANAGEMENT OF ACUTE FEBRILE NEUTROPHILIC
DERMATOSIS (SWEET SYNDROME)
Sweet syndrome ko generally oral corticosteroids se treat kiya jata hai, aur iska response kaafi accha hota hai. Agar treatment na mile, to yeh condition kuch hafton se mahino tak chal sakti hai, lekin aksar bina daag ke theek ho jati hai. Idiopathic aur malignancy-associated forms mein recurrence common hota hai. Kuch rare cases mein lesions waapas aa sakte hain ya lambey samay tak bane reh sakte hain. Malignancy-related cases mein zyada severe, treatment-resistant bullous ya ulcerative lesions ho sakte hain, jo kabhi kabhi atypical pyoderma gangrenosum jaise dikhte hain.
Medical Care:
1. Corticosteroids:
Systemic Corticosteroids: Prednisone primary treatment hota hai, jo jaldi kaam karta hai. Typical dose hoti hai 0.5-1 mg/kg/day. Severe cases mein 2 mg/kg/day do doses mein diya ja sakta hai. Pulmonary infiltrates bhi jaldi theek ho jate hain. Recurrence common hoti hai, isliye steroid ko dheere dheere kam karne ki salah di jati hai.
Topical Corticosteroids: High-potency options, jaise clobetasol propionate (0.05%) ya intralesional glucocorticoids (e.g., triamcinolone acetonide 3-10 mg/mL), localized lesions ke liye effective ho sakte hain.
2. Steroid-Sparing Agents:
Indomethacin, Colchicine, Potassium Iodide: Yeh kuch patients mein kaam aaye hain. Colchicine ko ek retrospective study mein pehle-line therapy ke roop mein effective paaya gaya.
Dapsone, Cyclosporine, Etretinate, Pentoxifylline, Clofazimine: Yeh agents kabhi kabar success ke saath use kiye gaye hain.
Other Agents: Doxycycline, metronidazole, isotretinoin, methotrexate, cyclophosphamide, chlorambucil, adalimumab, infliximab, IVIG, pulse doses of methylprednisolone, aur interferon-alfa ko kuch cases mein effective paya gaya hai.
3. Thalidomide: Yeh tab use hoti hai jab doosri treatments, jaise corticosteroids, metronidazole, dapsone, aur methotrexate, kaam na karein.
4. Biologic Agents:
- Etanercept: Kuch cases mein effective hota hai, khaaskar un patients mein jo rheumatoid arthritis se affected hain. Lekin, iska use karte waqt cancer ka risk dhyan mein rakhna padta hai.
- Anakinra aur Rituximab: Yeh refractory cases mein effective paaye gaye hain.
- Adalimumab: Recalcitrant cases mein yeh bhi effective hota hai.
CONSULTATIONS
· Dermatologist: Sweet syndrome ke diagnosis, evaluation, aur management ke liye, aur underlying causes ko identify karne ke liye dermatologist ki salah zaroori hoti hai.
· Internal Medicine Specialist: Triggering conditions ya underlying bimariyon ko assess karne ke liye internal medicine specialist ki madad zaroori hoti hai.
Is comprehensive approach ke zariye Sweet syndrome ko effectively manage kiya ja sakta hai, jisme sirf disease ko nahi, balki kisi bhi underlying condition ya triggers ko bhi address karna important hota hai.
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