A retrospective review of patient data found that older patients with systemic inflammation and neutrophilic dermatosis (ND) skin infiltrates may have vacuoles, enzyme E1, X-linked, autoinflammatory, somatic (VEXAS) syndrome. These findings were published as a research letter published in the Journal of the American Academy of Dermatology.
This multicenter, nationwide study evaluated clinical features of patients (N=59) with VEXAS included in the French VEXAS study group (NFVS) database.
Patients were median age 68 (interquartile range [IQR], 61.5-73) years when symptoms first appeared and nearly all (97%) were men.
The first symptom for more than half (63%) of patients was cutaneous involvement (pain: 35%, pruritus: 24%) and at the first consultation, the most common suspected cause of symptomology was Sweet syndrome (47%).
The review of lesion photographs (n=37) revealed that 97% of patients had round-shaped maculopapules and 32% had arcuate-shaped lesions, in which 89% had 10 or more lesions, 76% had lesions that were pink or red in color, 57% had both small (<1 cm) and larger (³1 cm) lesions, 85% had lesions on the trunk, 86% had lesions on the arms, 84% had lesions on the legs, 30% had lesions on the face.
The occurrence of lesions was generally characterized by periods of flare and remission among 81% of patients with a median of 6 flares per year that lasted a median of 9.8 days. However, lesions were permanent for 11% of patients.
Biopsy data were available for 43 patients. At biopsy, lesions were found to have infiltrates (89%) consisting of immature myeloid cells, mature neutrophils, lymphocytes, and histiocytes as well as leukocytoclasia (63%). These features led to a diagnosis of ND rich with immature myeloid cells among 77% of patients.
Immunostaining revealed CD68 and myeloperoxidase (MPO) expression in 88% of samples with myeloid precursors. Cytological tests classified the myeloid precursors as metamyelocytes (52%), myelocytes (42%), and neutrophils (26%).
The major limitations of this study were the retrospective design and small sample size.
On the basis of these trends, the investigators suspected that the spectrum of cutaneous lesions fit the criteria for VEXAS syndrome and advised that “VEXAS syndrome should be considered in patients ≥60 years with general symptoms, systemic inflammation, and with skin infiltrates showing ND with immature myeloid cells.”
Zakaine È, Rodrigues F, Papageorgiou L, et al. Clinical and pathological features of cutaneous manifestations in VEXAS syndrome: a multicenter retrospective study of 59 cases. J Am Acad Dermatol. 2022;S0190-9622(22)02980-2. doi:10.1016/j.jaad.2022.10.052