- Disease of unknown etiology most commonly affecting adult women
- Associated with hematopoietic malignancies, adenocarcinoma and upper respiratory tract infections
- Patients present with tender violaceous nodules or plaques on the face, neck and upper extremities in conjunction with a flu-like illness.
- Dense, diffuse neutrophilic infiltrate involving the superficial and deep dermis
- Prominent papillary dermal edema, sometimes causing subepidermal vesiculation
- Intraepidermal spongiotic vesicles are sometimes seen.
- Although not considered a vasculitic disorder, late-stage lesions may show secondary leukocytoclastic vasculitis.