[The 451(th) case: intermittent rash, fever and headache]

Area of Science:

• Rheumatology
• Neurology
• Dermatology

Background:

• Systemic vasculitis is a rare group of disorders characterized by inflammation of blood vessel walls.
• Diagnosis can be challenging due to diverse clinical presentations and overlapping symptoms with other conditions.

Observation:

• A 29-year-old woman presented with intermittent rashes, fever, headache, nausea, and vomiting.
• Physical examination revealed palpable purpura on extremities and trunk.
• Laboratory findings included elevated white blood cell count, platelet count, erythrocyte sedimentation rate, and C-reactive protein.

Findings:

• Cerebrospinal fluid analysis showed high intracranial pressure, but routine tests were normal.
• Skin biopsy confirmed systemic vasculitis with inflammatory infiltration of the muscular vessel wall.
• Antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative, complicating initial diagnosis.

Implications:

• This case underscores the importance of considering systemic vasculitis in patients with unexplained rashes, fever, and neurological symptoms.
• Combination therapy with corticosteroids and cyclophosphamide demonstrated efficacy in achieving rapid clinical improvement and remission.
• Early diagnosis and prompt treatment are crucial for favorable outcomes in systemic vasculitis.