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VEXAS Syndrome: Clues to Avoid Vexation
Deepika Kumar1, Gauri Panse1, Christine J Ko1
1Yale University, New Haven, Connecticut, USA.
VEXAS syndrome, a condition with varied symptoms, frequently presents with skin issues. This review highlights key dermatologic and dermatopathologic signs for diagnosing Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome.
Area of Science:
- Rheumatology
- Dermatology
- Genetics
Background:
- VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an emerging autoinflammatory disorder.
- Cutaneous manifestations are reported in the majority of individuals with VEXAS syndrome.
Purpose of the Study:
- To review the diverse clinical and dermatopathologic findings associated with VEXAS syndrome.
- To provide guidance on recognizing VEXAS syndrome based on skin manifestations.
Main Methods:
- Literature review of VEXAS syndrome cases with a focus on dermatologic and dermatopathologic features.
- Synthesis of clinical presentation, histopathology, and diagnostic criteria.
Main Results:
- VEXAS syndrome presents with a wide spectrum of skin findings.
- Dermatologic clues are crucial for early identification and diagnosis.
Conclusions:
- Dermatologists play a key role in the diagnosis of VEXAS syndrome.
- Recognition of specific cutaneous and dermatopathologic findings can aid in timely diagnosis and management.
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