BACKGROUND: Isolated or predominantly hand involvement in Sweet's syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweet's syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy. METHODS: We report four cases of neutrophilic dermatosis affecting the hands and a review of the literature for similar cases. RESULTS: Of the four patients presented, two had associated carcinomas and one had myelodysplasia in transition to leukemia. The cutaneous symptoms preceded the finding of an occult malignancy. Thirty-two reported cases of neutrophilic dermatoses presenting on the hands showed clinicopathologic features similar to those in our series. Taken together, the mean age at diagnosis was 60.5 years, and 58% of the patients were female. Twenty-five per cent (nine patients) also had myelodysplasia or leukemia, 14% (five patients) ulcerative colitis, 6% (two patients) carcinoma, 6% (two patients) Crohn's disease, and 6% (two patients) seropositive arthritis. CONCLUSIONS: These cases illustrate the importance of recognizing that neutrophilic dermatoses may present uniquely or predominantly on the hands. This presentation is distinctive, and prompt diagnosis may prevent unnecessary medical or surgical therapy and may lead to the earlier diagnosis and treatment of an associated malignancy or other systemic disorder.
BACKGROUND: Isolated or predominantly hand involvement in Sweet's syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweet's syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy. METHODS: We report four cases of neutrophilic dermatosis affecting the hands and a review of the literature for similar cases. RESULTS: Of the four patients presented, two had associated carcinomas and one had myelodysplasia in transition to leukemia. The cutaneous symptoms preceded the finding of an occult malignancy. Thirty-two reported cases of neutrophilic dermatoses presenting on the hands showed clinicopathologic features similar to those in our series. Taken together, the mean age at diagnosis was 60.5 years, and 58% of the patients were female. Twenty-five per cent (nine patients) also had myelodysplasia or leukemia, 14% (five patients) ulcerative colitis, 6% (two patients) carcinoma, 6% (two patients) Crohn's disease, and 6% (two patients) seropositive arthritis. CONCLUSIONS: These cases illustrate the importance of recognizing that neutrophilic dermatoses may present uniquely or predominantly on the hands. This presentation is distinctive, and prompt diagnosis may prevent unnecessary medical or surgical therapy and may lead to the earlier diagnosis and treatment of an associated malignancy or other systemic disorder.