Neutrophilic Dermatosis (Pustular Vasculitis) of the Dorsal Hands

Abstract

Background: Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands is a recently described disorder, which may clinically resemble a localized variant of Sweet syndrome.

The Relationship Between Neutrophilic Dermatosis of the Dorsal Hands and Sweet Syndrome

“…Cases originally described as Sweet syndrome lacked vasculitis, though vascular injury may occur secondary to a brisk neutrophilic infiltrate and the accompanying enzymatic and cytokine cascade. Leukocytoclastic vasculitis was present in 2 (22%) of 9 patients in our series and in 12 (28%) of 43 cases in the literature, 3,5 or 27% (14/52) in aggregate. This agrees with 2 histologic series that identified leukocytoclastic vasculitis or vasculitislike changes in 18% to 30% of cases of typical Sweet syndrome.…”

“…Others have since reported cases and offered insights into the categorization of this entity. [3][4][5] We report 9 new cases of NDDH, all with features of Sweet syndrome, including histologic features (brisk dermal neutrophilic infiltrate), laboratory findings (elevated inflammatory markers and neutrophilia), constitutional symptoms (fever, malaise), associated illnesses (hematologic malignancy, inflammatory bowel disease), and response to treatment with corticosteroids. We discuss these 9 cases in the context of the 43 cases previously reported.…”

“…Interestingly, 3 of our cases demonstrated pathergy, as noted in other series. 3 Much of the nosologic debate has been related to the presence of vasculitis in NDDH biopsy specimens. Cases originally described as Sweet syndrome lacked vasculitis, though vascular injury may occur secondary to a brisk neutrophilic infiltrate and the accompanying enzymatic and cytokine cascade.…”

Sweet Syndrome Associated with Celiac Disease

“…Oral prednisolone, dapsone, or minocycline led to resolution in six of seven patients in 1-9 weeks with 50% recurrence after up to 4 years. [23] Lesions may also spontaneously remit.…”

Neutrophilic dermatosis: a rare cause of an ischaemic digit

“…Galaria et al (2000) described similar cases, but as there was no vasculitis in several of these cases, the term NDDH was preferred over pustular vasculitis. DiCaudo et al (2002) described a case series of seven women. Five had lesions at sites other than the hands.…”