Diagnosis: Acute febrile neutrophilic dermatosis (Sweet syndrome)
A 34-year-old female presents with tender, erythematous plaques on her face and arms, accompanied by fever and malaise for the past week. Initial laboratory evaluation reveals leukocytosis with neutrophilia, prompting further investigation into this acute dermatosis.
A 34-year-old female presents with a one-week history of tender, juicy red plaques on her face and arms, along with fever and malaise. On examination, the plaques are well-defined, erythematous, and measure up to 5 cm in diameter. The lesions are painful to palpation, with no associated vesicles or ulcers. The patient exhibits signs of systemic involvement, including a temperature of 38.5°C.Well-defined erythematous plaques: Present on the face and arms.Acute onset: Symptoms developed over the past week.Associated systemic symptoms: Fever and malaise.Leukocytosis: Laboratory findings show elevated white blood cell count with neutrophilia.Negative history for infection: No recent infections or travel history.
The patient reported that the lesions began abruptly after experiencing a mild upper respiratory infection. She has no significant past medical history but mentions a family history of autoimmune diseases. There are no known drug allergies, and she is not currently taking any medications. The patient denies recent travel or exposure to known infectious agents.Onset: Abrupt onset following mild upper respiratory infection.Triggers: No identifiable triggers, including medications or recent vaccinations.Past medical history: Unremarkable with no chronic conditions.Family history: Positive for autoimmune diseases.Social history: Non-smoker, no alcohol use, and no recent travel.
Acute / First-Line ManagementCorticosteroids: First-line treatment involves systemic corticosteroids, with prednisone starting at 0.5-1 mg/kg/day, tapered based on clinical response.Supportive care: Management of fever and pain with antipyretics and analgesics as needed.Workup and Diagnostic ConfirmationSkin biopsy: Histopathological examination typically reveals a dense neutrophilic infiltrate in the dermis.Laboratory tests: Complete blood count to assess leukocytosis and neutrophilia; inflammatory markers may be elevated.Exclusion of underlying conditions: Rule out associated malignancies or infections through thorough history and laboratory workup.Long-Term ManagementMaintenance therapy: Once acute symptoms resolve, consider tapering corticosteroids and monitoring for relapses.Second-line agents: In cases of recurrent or refractory disease, consider dapsone or colchicine as alternative treatments.
Pyoderma gangrenosum: Often presents with painful ulcers and may be associated with systemic diseases; biopsy shows neutrophilic infiltration.Dermatitis herpetiformis: Characterized by pruritic vesicles and papules, typically associated with gluten sensitivity; direct immunofluorescence can confirm diagnosis.Vasculitis: Presents with palpable purpura or ulcers; laboratory tests may show ANCA positivity or cryoglobulinemia.Infectious etiology: Bacterial or viral infections can mimic the presentation; cultures and serologies can help differentiate.Drug-induced skin reactions: Certain medications can cause similar dermatoses; thorough medication history is essential.Acute generalized exanthematous pustulosis: Characterized by pustular lesions; often associated with recent medication use.Subacute cutaneous lupus erythematosus: May present with erythematous plaques; associated with systemic symptoms and positive autoimmune markers.Granulomatosis with polyangiitis: Systemic vasculitis that may present with skin lesions; typically associated with respiratory symptoms and ANCA positivity.
High-Yield PearlsClassic presentation: Sweet syndrome typically presents with tender, erythematous plaques along with systemic symptoms like fever.Histopathology: A skin biopsy showing neutrophilic infiltration is key to diagnosis.Systemic corticosteroids: Prednisone is the first-line treatment; rapid improvement is often seen.Associated conditions: Sweet syndrome may be triggered by malignancies, infections, or autoimmune diseases; thorough workup is essential.Relapses: Monitor for potential recurrences after tapering corticosteroids; consider alternative therapies for refractory cases.Sweet syndrome is characterized by an acute onset of painful plaques and systemic symptoms, requiring prompt recognition and management to prevent complications.
Tags: Sweet syndrome, neutrophilic dermatosis