We encounter in the literature various terms used to refer to conditions that are characterized clinically by predominantly acral acute lesions and histologically by an inflammatory infiltrate predominantly composed of polymorphonuclear cells and which are often associated with fever and/or are preceded by an infectious process. These conditions are currently included in what is known as the spectrum of neutrophilic dermatoses.

Since the initial description of acute neutrophilic dermatosis by Douglas Robert Sweet in 1964 and the subsequent description of the spectrum of neutrophilic dermatoses, the nomenclature has changed as knowledge of these conditions has progressed and new clinical manifestations have been described.

Sweet referred to acute neutrophilic dermatosis as a condition characterized by fever; neutrophilic leukocytes; painful plaques on the face, neck, and limbs; and the presence of abundant inflammatory infiltrate in the dermis composed fundamentally of polymorphonuclear cells with no evidence of leukocytoclastic vasculitis. The presence of vasculitis as an epiphenomenon secondary to the inflammatory process was later accepted, especially in cases in which biopsy is performed late.

As for predominantly acral neutrophilic dermatoses, in 1995 Strutton et al. introduced the term pustular vasculitis of the dorsal hands to refer to skin lesions characterized clinically by pustules on a purpuric base and histologically by leukocytoclastic vasculitis.1 Later, in the year 2000, Galaria et al. reported 3 cases that were clinically similar to those reported earlier but lacked associated vasculitis; they introduced the term neutrophilic dermatosis of the dorsal hands.2 In 2004, Weenig et al. excluded the word “dorsal” from the term because they observed that many patients also had lesions on the palms and lateral regions of the hands; they called these conditions neutrophilic dermatosis of the hands.3 More recently, the term acral Sweet syndrome has been used to describe similar conditions.4

Before and around the same time that the neutrophilic dermatoses were being described, there were reports of other conditions that today could well be included in this spectrum. Examples include pustular bacterids of the hands and feet, reported by Andrews,5 and acute generalized pustular bacterid.6 Both conditions are characterized by the appearance of predominantly acral lesions after an infection, most frequently of the pharynx or tonsils.

We can therefore conclude that these various designations refer to similar conditions that today are probably better classified within the spectrum of neutrophilic dermatoses, with the peculiarity of acral predominance. Like the other neutrophilic dermatoses, these conditions are characterized by an intense neutrophilic infiltrate and can be associated (or not) with the presence of vasculitis. Moreover, they take different clinical forms—pustules, vesicles, or plaques—depending on the location of the infiltrate. Associated in their most classic form with febrile or infectious processes, these conditions can also occur in association with inflammatory diseases (such as inflammatory bowel disease) or neoplastic diseases; therefore, to rule out these processes, it is essential to always carry out an adequate medical history and physical examination and request whatever additional tests are appropriate in each case. It is necessary to be familiar with these terms because they continue to appear even in recent literature, even though many of them could probably now be considered obsolete.