Ultrasound features of Primary Cutaneous CD 4 + Small / Medium - Sized T - Cell

are associated with slightly elevated AFRs; class ii, in which constitutional symptoms are also present, with fever and marked elevation of the AFRs; and class iii, in which the cutaneous form progresses to systemic forms of the disease. We classified our patient in the second subgroup. In cutaneous PAN, patients with marked elevation of AFRs are more likely to present inflammatory plaques and edema of the lower limbs. Considering this association as a more severe form of the disease, it has been proposed that the initial management approach should be more aggressive to avoid progression to systemic forms. Although the use of TNF inhibitors is not uncommon in the systemic forms of PAN, we have found very few reports in the literature in which this treatment has been required to control cutaneous forms (Table 1). In conclusion, we have presented a case of cutaneous PAN associated with a systemic inflammatory response and refractory to conventional treatments. It is essential to suspect this disease in patients with nodules beneath plaques showing centrifugal spread, even if histology does not initially support the diagnosis, particularly if there is an associated intense inflammatory reaction that could hide the vasculitis. Patients with cutaneous PAN with very high AFRs will often require management similar to the systemic forms, and TNF inhibitors are an option.