Sweet Syndrome



Acute febrile neutrophilic dermatosis (Sweets Syndrome) is a skin condition characterized by fever and inflamed or blistered skin and mucosal lesions. Neutrophilic dermatoses are autoinflammatory conditions that can be associated with systemic disease.



Rare Disorder:

 - Fever + sudden onset of a rash

 - Rash: multiple tender, red or bluish-red bumps or lesions.

 - Occurs on the arms, legs, trunk, face or neck.

Other areas:

	- Musculoskeletal system such as inflammation of the joints (arthritis)

	- The eyes such as inflammation of the conjunctiva or the membrane that lines the eyes (conjunctivitis)

	- Internal organs

Most common extra cutaneous involvement:

Eyes

	- Conjunctivitis

	- Episcleritis



Signs and Symptoms:

	- Fever

	- Fatigue

	- Headaches

	- A general feeling of ill health (malaise)

	- Muscle pain (myalgia)

	- Inflammation and pain of the joints (arthritis and arthralgia)

	- Fever can precede the development of skin symptoms by days or weeks.



- Papules are solid, raised lesions; nodules are slightly larger and may extend deeper into the skin.

- Sudden onset of tender or painful bumps (nodules or papules) on the arms, legs, face or neck.

- They may also occur on the thighs and trunk.



Causes:

- Idiopathic Sweet syndrome - this is also known as classical Sweet syndrome.

- Underlying cancer (malignancy), lymphoma, breast CA

- Hematologic cancer such as certain types of leukemia

- Cancers of the genitourinary and gastrointestinal tracts.

- Drug induced: GCSF stimulating factor

- Upper respiratory infection, gastrointestinal infection, or influenza-like illness can precede skin lesions by one to three weeks.



DDX:

- Pyoderma gangrenosum

- Erythema multiforme

- Dermatomyositis

- CANDLE syndrome

- Allergic contact dermatitis

- Cellulitis

- Erythema nodosum

- Acral erythema

- Bacterial sepsis

- Neutrophilic rheumatoid arthritis

- Panniculitis

- Leukemia cutis

- Leukocytoclastic vasculitis

- Schnitzler’s syndrome

- Lupus

- Leukemia cutis



Diagnosis:

- Biopsy

- Microscopic examination of small samples of skin tissue may reveal the infiltrate of neutrophils in the dermis.

- CBC may also show neutrophils in the blood (neutrophilia).



Treatment:

- Systemic corticosteroids



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