Pustular vasculitis of the hands

Abstract

A case of pustular vasculitis of the hands with evidence of systemic involvement is described. A 64-year-old woman presented with a 2-day history of large, tense bullae arranged symmetrically over the dorsum of the three radial digits and extending on to the radial aspect of the dorsum of each hand. The bullae caused some discomfort and prevented normal use of her hands. There was no response to antibiotic therapy initiated prior to referral to hospital. Initial investigations revealed a raised white cell count with a neutrophilia, a raised erythrocyte sedimentation rate and a raised C-reactive protein. Abnormalities of liver function were detected. Aspirates from the bullae and blood cultures were sterile. The histology of debrided tissue demonstrated a florid neutrophilic dermal infiltrate with many blood vessels associated with prominent fibrin. A diagnosis of pustular vasculitis of the hands was made. The bullae were surgically debrided and treatment with oral corticosteroids was started. Two days after commencement of oral prednisolone, a crusted pustule appeared on her upper cutaneous lip. There was rapid resolution of both the vasculitis of the hands and the pustule on her upper lip following an increase in the dose of oral prednisolone. The patient was discharged on the seventeenth day following admission.

Skin Lesions of Sweet Syndrome and Its Dorsal Hand Variant Contain Vasculitis

“…38 Eighth, direct immunofluorescence studies of lesional skin were performed in 2 patients with NDDH. 2,40 The results of these studies in the patient described by Hall et al 40 and in patient 2 of DiCaudo and Connolly 2 were negative for immunoglobulins or complement. Hence, it is reasonable to postulate that the vasculitis observed in these patients with NDDH (and perhaps also in other patients with this condition) was a secondary leukocytoclastic vasculitis instead of a primary leukocytoclastic vasculitis.…”

“…Subsequently, Galaria et al38 proposed the term neutrophilic dermatosis of the dorsal hands for this condition.Presently, NDDH has been reported in 19 patients: 3 men 38,39 and 16 women 2,37,38,40. Skin lesions, morphologically similar to those of Sweet syndrome, are predominantly restricted to the dorsal hands; however, 8 (42%) of the 19 individuals also had lesions at other sites: oral mu-…”

“…Pustular vasculitis refers to a group of conditions that clinically are characterized by "pustular lesions that occur on purpuric bases" 36 and that microscopically "range from fully developed leukocytoclastic vasculitis to a neutrophilic vascular reaction [which is the preferred designation instead of the previously used Sweet-like vasculitis] with vessel changes such as those seen in Sweet syndrome." 36 38,40 to leukocytoclastic vasculitis of variable severity. 2,37,39,40 Similar to those of Sweet syndrome, lesions of NDDH rapidly resolve after initiation of therapy with systemic corticosteroids or dapsone or both.…”

“…36 38,40 to leukocytoclastic vasculitis of variable severity. 2,37,39,40 Similar to those of Sweet syndrome, lesions of NDDH rapidly resolve after initiation of therapy with systemic corticosteroids or dapsone or both. 2,37-40 Based on the efficacious response of patients with Sweet syndrome who have been treated with either potassium iodide or colchicine, it is reasonable to postulate that these agents would also be likely to provide prompt improvement of the skin lesions in patients with NDDH.…”

Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis

“…When the clinical lesions of the dermatosis are predominantly restricted to the dorsal hands, this localized, pustular variant of Sweet's syndrome has been referred to as either "neutrophilic dermatosis of the dorsal hands" or "pustular vasculitis of the dorsal hands" [3,149-154,401,402,408,420,427]. The morphology and response to treatment of the dermatosis-associated lesions reported as neutrophilic dermatosis of the dorsal hands are similar to those of Sweet's syndrome.…”