Amicrobial pustulosis of the folds - A cutaneous manifestation associated with connective tissue disease

Amicrobial pustulosis (AP) is a recently defined entity associated with con nective tissue diseases. Few cases have appeared in the literature. We repo rt a case of AP coexisting with a systemic lupus erythematosus-scleroderma overlap syndrome and marked photosensitivity. The patient presented promine nt pustular skin lesions and a few discoid lupus ones. No significant diffe rences in the inflammatory infiltrate were found between the two clinical v ariants. The infiltrate consisted mainly of CD4+ lymphocytes and many neutr ophils. CD1a+ dendritic cells were few in both epidermis and dermis. AP int roduces a potential source of diagnostic confusion, but increasing experien ce of this syndrome will improve the awareness and diagnostic potential amo ng dermatologists.