Background

This is a recently described disease with the characteristic findings of large ulcerative nodules on the dorsum of the hands. Under the microscope, the characteristic findings of a leukocytoclastic vasculitis are usually present.

OUTLINE

Epidemiology
Pathogenesis
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Neutrophilic dermatosis of the dorsal hands
INCIDENCE	Rare
SEX (M:F)	Women

 

PATHOGENESIS	CHARACTERIZATION
SWEET'S SYNDROME	May represent a localized variant of Sweet's

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
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.
Neutrophilic Dermatosis (Pustular Vasculitis) of the Dorsal Hands A Report of 7 Cases and Review of the Literature David J. DiCaudo, MD; Suzanne M. Connolly, MD	Arch Dermatol. 2002;138:361-365 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.
VARIANTS
MALE
Pustular vasculitis of the hands. Report of two men. Curco N, Pagerols X, Tarroch X, Vives P. Department of Dermatology, Hospital Mutua de Terrassa, Barcelona, Spain.	Dermatology 1998;196(3):346-7 Abstract quote

 

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
VARIANTS
WITHOUT VASCULITIS
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.

 

SPECIAL STAINS/IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
SPECIAL STAINS	Microbiological stains are negative

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Pyoderma gangrenosum
Sweet's Syndrome

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS
TREATMENT	Dapsone and prednisone

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Leukocytoclastic Vasculitis
Sweet's Syndrome
Vasculitis

Last Updated 3/29/2002