Skip to main content

Sweet syndrome (Introduction, Types, Pathophysiology)

 

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Introduction

Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, was first described by Robert Douglas Sweet in 1964. It is a reactive process characterized by the sudden onset of tender, red-to-purple papules and nodules that often merge to form plaques, usually occurring on the upper extremities, face, or neck. This dermatologic condition is often associated with fever and elevated neutrophil levels in the blood (peripheral neutrophilia).


Sweet syndrome


Sweet syndrome is categorized into three types based on the underlying cause:

1. Classic or Idiopathic Sweet Syndrome

This is the most common form, predominantly affecting young women and often following a mild respiratory illness. It may also be associated with pregnancy, inflammatory bowel disease (IBD), vaccinations, other inflammatory conditions, and infections.

2. Malignancy-Associated Sweet Syndrome

This form occurs alongside an underlying malignancy, sometimes being the first indication of a hidden cancer. It can also present as a paraneoplastic syndrome or as part of an established cancer, most commonly linked with acute myelogenous leukemia and myelodysplastic syndrome.

3. Iatrogenic Sweet Syndrome

 This variant is triggered by certain medications, most notably granulocyte colony-stimulating factor (G-CSF). Other implicated agents include trimethoprim/sulfamethoxazole, minocycline, contraceptives like levonorgestrel/ethinyl estradiol, and all-trans retinoic acid (ATRA). Antineoplastic agents, biologics, and radiotherapy have also been linked to iatrogenic Sweet syndrome.


sweet syndrome


Pathophysiology of Sweet Syndrome

Sweet syndrome is a “neutrophil-mediated hypersensitivity reaction” triggered by factors such as infections, malignancies, drugs, or inflammation. Its hallmark is an abnormal activation of neutrophils, supported by the presence of neutrophilia and the condition’s response to treatments targeting neutrophil function.

Granulocyte colony-stimulating factor (G-CSF) plays a key role by prolonging neutrophil survival and increasing their activity. Elevated G-CSF levels correlate with disease severity, and even in cases with low neutrophil counts, lesions can develop due to heightened neutrophil function. All-trans retinoic acid (ATRA), used in acute promyelocytic leukemia, has also been linked to neutrophil dysfunction in drug-induced cases.

Cytokines, such as IL-1, IL-2, and IFN-γ, contribute to the disease, particularly in malignancy-related forms. Elevated IL-6 levels also track with disease activity, decreasing with corticosteroid treatment.

Genetically, HLA-B54 (in Japanese populations) and chromosome 3q abnormalities are associated with the condition. Mutations in FLT3 have also been linked to Sweet syndrome-like skin disease, and familial cases suggest a genetic predisposition.

Labels:

Comments

Post a Comment

Popular posts from this blog

Chronic neck pain

  Chronic Neck Pain: A Primary Care Approach Neck pain bahut hi common hai, aur ise hum teen main types mein divide karte hain: 1. Mechanical Neck Pain Ye sabse zyada commonly dekhi jaane wali neck pain hai jo primary care centre mein aati hai. Patients ko generally localized neck pain hota hai jo kisi aur jagah radiate nahi karta. Zyada tar ye pain neck ke center mein hota hai. Symptoms: Localized pain jo neurological deficits ke bina hota hai, jaise weakness ya numbness nahi hoti. Agar aap us jagah ko press karte ho jaha "ouch" feel hota hai, toh wohi area problematic hota hai. Etiology: Exact cause clear nahi hota, lekin myofascial ya musculoskeletal strain se related hota hai. Management: Ye pain time ke sath apne aap theek ho jaata hai, chahe aap koi intervention karein ya na karein. Common treatments mein NSAIDs (painkillers), physical therapy, aur massage include hain. Physical therapy helpful ho sakti hai taaki patient apni posture aur movement ko sudha
Post a Comment
Read more

GENE THERAPY SE DIKHAYI DENE LAGI BEHTAR VISION CONGENITAL BLINDNESS MEIN

  GENE THERAPY SE DIKHAYI DENE LAGI BEHTAR VISION CONGENITAL BLINDNESS MEIN   Ek naye gene therapy, ATSN-101, ne rare congenital blindness ke ek type, Leber congenital amaurosis (LCA) 1, se peedit bade aur bacchon mein roshni ko mehsoos karne aur vision ko behtar banane ke promising results dikhaye hain. Ye LCA, GUCY2D gene mein mutation ke kaaran hoti hai. “The Lancet”mein chhapi ek early-phase clinical trial mein 15 patients ko shaamil kiya gaya aur roshni ko mehsoos karne mein khaas sudhar dekha gaya. Is trial ke dauran, 12 mahine ke ilaj ke baad, jinhonne therapy ka sabse highest dose liya unmein nazar ke bareek tafseel (fine details) dekhne ki kshamata mein sudhar dikha. Example ke taur par, patients ne eye test chart par lagbhag 8 letters behtar dekhe, jo ETDRS chart ke lagbhag 2 lines ke barabar hai. Halanki nazar ki tez kshamata (acuity) mein sudhar chhote the, lekin roshni ko mehsoos karne ki kshamata, khaaskar retine ke rods aur cones mein, sabse khaas baat thi. Dr. A
Post a Comment
Read more

Sweet syndrome (Introduction, Types, Pathophysiology)

  Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) Introduction Sweet Syndrome (SS), ya acute febrile neutrophilic dermatosis, ko pehli baar Robert Douglas Sweet ne 1964 mein describe kiya tha. Ye ek reactive process hai jo achanak se tender, red-to-purple papules aur nodules ke saath hota hai, jo aksar plaques banate hain. Ye lesions zyadatar upper extremities, face, ya neck par hote hain. Is dermatologic condition ke saath fever aur elevated neutrophil levels (peripheral neutrophilia) bhi dekhe jaate hain. Sweet syndrome ko teen types mein categorize kiya gaya hai based on underlying cause: 1. Classic ya Idiopathic Sweet Syndrome Ye sabse common form hai, jo predominantly young women ko affect karta hai aur aksar mild respiratory illness ke baad hota hai. Iska association pregnancy, inflammatory bowel disease (IBD), vaccinations, inflammatory conditions, aur infections se bhi ho sakta hai. 2. Malignancy-Associated Sweet Syndrome   Ye form underlying malignancy ke
Post a Comment
Read more