Primary anetoderma as a possible preclinical cutaneous clue of Behçet's disease: evidence suggesting early...

Anetoderma is a rare elastolytic dermatosis characterized by focal loss of dermal elastic fibers leading to localized flaccid, atrophic, or herniated skin lesions. Although it may occur as a primary disorder or secondary to inflammatory dermatoses, infections, or trauma, immune-mediated vascular injury and dysregulation of extracellular matrix remodeling are increasingly recognized as key pathogenetic mechanisms. Behçet's disease (BD) is a multisystem inflammatory vasculitis driven by neutrophil hyperactivation and endothelial dysfunction, but elastolytic dermatoses have not been included among its classical cutaneous manifestations. We report a 27-year-old man who developed multiple asymptomatic atrophic lesions on the trunk and upper back. Histopathologic examination revealed preserved epidermal architecture and marked loss of elastic fibers in the papillary and mid dermis on Verhoeff-Van Gieson staining, consistent with primary anetoderma. Approximately 3 years later, the patient developed recurrent oral aphthosis, genital ulcers, papulopustular lesions, and a positive pathergy test, fulfilling the International Criteria for Behçet's Disease. The temporal sequence observed in our patient raises the hypothesis that primary anetoderma may represent a rare early cutaneous manifestation or a preclinical marker of microvascular inflammation in BD. Neutrophil-driven proteolysis and increased matrix metalloproteinase (MMP) activity may contribute to focal dermal elastolysis. Further observations are needed to clarify whether this association reflects a pathogenetic link or a coincidental finding.