Background

This rare disorder may be closely related to Sweet's syndrome. It is characterized by a neutrophilic dermatosis. Possible systemic associations included bowel disorders and some infections. Prednisone and dapsone appeared to be helpful, but recurrences were common.

OUTLINE

Gross Appearance and Clinical Variants
Histopathological Features and Variants
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands: a report of 7 cases and review of the literature. DiCaudo DJ, Connolly SM. Department of Dermatology, Mayo Clinic Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259, USA.	Arch Dermatol. 2002 Mar;138(3):361-5. Abstract quote   BACKGROUND: Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands is a recently described disorder, which may clinically resemble a localized variant of Sweet syndrome. OBJECTIVES: To describe the clinical and histopathologic characteristics of this rare disorder; to compare and contrast these features with those of Sweet syndrome; and to investigate possible associations with systemic diseases. OBSERVATIONS: Seven women were referred for pustular or ulcerative plaques and nodules on the dorsal hands. In most patients, the initial diagnosis was cutaneous infection, but antibiotic therapy was ineffective. Skin biopsy specimens showed dense dermal neutrophilic infiltrates with leukocytoclasis and fibrinoid vascular necrosis. Cutaneous cultures yielded negative findings in all cases. Prednisone and dapsone appeared to be helpful, but recurrences were common. Minocycline hydrochloride was of uncertain benefit. Among the 7 patients, possible systemic associations included bowel disorders and a urinary tract infection. CONCLUSIONS: Neutrophilic dermatosis of the dorsal hands may be closely related to Sweet syndrome but frequently shows the histologic pattern of leukocytoclastic vasculitis. Recognition of this disorder is important, because it may be misdiagnosed as a localized cutaneous infection. Additional studies are needed to investigate further the possible associations with internal diseases, especially bowel disorders.
Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Department of Dermatology, University of Pennsylvania, Philadelphia, USA.	J Am Acad Dermatol. 2000 Nov;43(5 Pt 1):870-4. Abstract quote   An entity termed "pustular vasculitis of the hands" was recently described. Patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers, which were characterized histologically by a dense neutrophilic infiltrate and leukocytoclastic vasculitis. We describe patients with a similar clinical presentation, but who lacked vasculitis on biopsy findings. We describe 3 otherwise asymptomatic patients with hemorrhagic bullae, plaques, and pustules solely on the dorsal hands. Biopsy specimens showed a neutrophilic infiltrate and leukocytoclasis, but no necrotizing vasculitis, and were reminiscent of Sweet's neutrophilic dermatoses. In our patients, corticosteroids or dapsone led to clearing of the lesions, and small maintenance doses of dapsone prevented their recurrence. Our 3 patients had clinical lesions similar to those termed pustular vasculitis of the hands, but which lacked leukocytoclastic vasculitis on biopsy findings. Because of histologic findings and a therapeutic response more characteristic of Sweet's syndrome, we propose the term neutrophilic dermatosis of the dorsal hands. In addition, low-dose dapsone is proposed as a possible first-line therapy in this condition, especially in those with recurrent disease.
Pustular vasculitis of the hands. Hall AP, Goudge RJ, Ireton HJ, Burrell LM. Department of Dermatology, Austin and Repatriation Medical Centre, Heidelberg, Victoria, Australia.	Australas J Dermatol. 1999 Nov;40(4):204-7. Abstract quote   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.
Pustular vasculitis of the hands. Strutton G, Weedon D, Robertson I. Department of Pathology, Princess Alexandra Hospital, Woolloongabba, Brisbane, Australia.	J Am Acad Dermatol. 1995 Feb;32(2 Pt 1):192-8. Abstract quote   BACKGROUND: Several patients were observed with a peculiar cutaneous eruption limited to the dorsa of the hands and fingers. Clinically the lesions had some resemblance to those seen in Sweet's syndrome, but biopsy specimens showed severe leukocytoclastic vasculitis. OBJECTIVE: Our purpose was to characterize this eruption clinically and histologically and compare it with previously described diseases. METHODS: Six patients observed since 1977 are described. Skin biopsy specimens were obtained. RESULTS: In six women (age, 41 to 79 years) a symmetric eruption of papules and plaques limited to the dorsa of the radial sides of the hands and first three digits developed. The lesions resembled those of Sweet's syndrome and were associated with fever, sterile culture, blood neutrophil leukocytosis, nonresponse to antibiotic therapy, and rapid response to prednisone. Biopsy specimens showed a severe leukocytoclastic vasculitis. CONCLUSION: These patients appear to have a distinct entity that we have termed pustular vasculitis of the hands.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL	Neutrophilic dermatosis

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
SWEETS SYNDROME

 

TREATMENT	CHARACTERIZATION
GENERAL	Dapsone and corticosteroids

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DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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