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Neutrophilic dermatosis of the dorsal hands versus pustular vasculitis
962 Letters
Neutrophilic dermatosis of the dorsal hands versus pustular vasculitis To the Editor: We read with interest the article by Galaria et al,1 reporting 3 cases of a localized form of Sweet’s syndrome on the dorsal hands, similar to the cases of pustular vasculitis of the hands reported by Strutton et al2 but without histopathologic vasculitis. Two years ago, we saw a patient with clinical and histologic findings similar to those described by the authors. A 61-year-old man had a 48-hour history of bullous lesions on the dorsal hands. The lesions began as erythematous papules, which rapidly progressed to large hemorrhagic bullous lesions with pustules and a purple-red border (Fig 1). There was intense edema surrounding the lesions, which were remarkably limited to the dorsa of both his hands and fingers. The lesions were painful but not accompanied by adenopathy, fever, or malaise. Medical his-
J AM ACAD DERMATOL JUNE 2002
tory was significant for adenocarcinoma of the colon 3 years before. Laboratory investigations revealed an elevated white blood cell count of 14.8 with neutrophilia (11.8 ⫻ 109/L), erythrocyte sedimentation rate of 81 mm/h, and fibrinogen level of 991 mg/dL. Cultures of specimens from the lesions for bacteria and viruses were all negative. The biopsy specimen revealed a partially necrotic epidermis with marked exocytosis of neutrophils. There was a prominent papillary dermal edema with presence of some neutrophils and a dense neutrophilic infiltrate with leukocytoclasis in the reticular dermis. Very scattered and focal images of fibrinoid necrosis can be observed in isolated dermal vessels but not a true leukocytoclastic vasculitis. No signs of recidivism or metastases of colon carcinoma were detected. The patient had been treated with antibiotics without any response. We started oral administration of prednisone (45 mg/d), and the lesions cleared rapidly without scarring. Seventeen months later, the patient had a new flare, less intense than before, which showed a dramatic response to prednisone. The cases published by Galaria et al1 and Strutton et al2 and our case all share the main clinical and histopathologic features of Sweet’s syndrome.3 The only difference is the presence or absence of vasculitis. Nevertheless, not all the cases are so clear. Strutton et al2 describe 2 cases (numbers 1 and 5) as Sweet’s syndrome with a vasculitic component, and case number 2 in the report by Galaria et al1 shows some perivascular fibrin deposition. On the basis of histopathologic findings, we consider our case to be Sweet’s syndrome with isolated and partial phenomena of fibrinoid necrosis.4 One explanation for these differences could be the evolution of the lesions; all of the patients described by Strutton et al underwent biopsy at least 1 week after the onset of the lesions, Galaria et al1 do not give the precise time, and our patient had a biopsy performed only 48 hours after the onset. Therefore, there could be a continuum, considering the vascular damage caused by the intense inflammatory neutrophilic infiltrate.3 We agree with the proposal of Galaria et al1 to name this entity neutrophilic dermatosis of the dorsal hands and consider it as a localized form of Sweet’s syndrome. Yolanda Gilaberte, MD Carmen Coscojuela, MD Maria Dolores Garcı´a-Prats, MDa Departments of Dermatology and Pathologya Hospital San Jorge Huesca, Spain E-mail: [email protected]
Letters 963
J AM ACAD DERMATOL VOLUME 46, NUMBER 6
Fig 1. Hemorrhagic bullous lesions with pustules on dorsal right hand.
REFERENCES 1. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol 2000;43:870-4. 2. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995;32:192-8. 3. Cohen PR, Kurzrock R. Sweet’s syndrome: a neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol 2000;18:265-82. 4. Jordaan HF. Acute neutrophilic dermatosis: a histopathological study of 37 patients and a review of the literature. Am J Dermatopathol 1989;11:99-111. 16/8/120574 doi:10.1067/mjd.2002.120574
Neutrophilic dermatosis of the dorsal hands versus pustular vasculitis To the Editor: We read with interest the article by Galaria et al,1 reporting 3 cases of a localized form of Sweet’s syndrome on the dorsal hands, similar to the cases of pustular vasculitis of the hands reported by Strutton et al2 but without histopathologic vasculitis. Two years ago, we saw a patient with clinical and histologic findings similar to those described by the authors. A 61-year-old man had a 48-hour history of bullous lesions on the dorsal hands. The lesions began as erythematous papules, which rapidly progressed to large hemorrhagic bullous lesions with pustules and a purple-red border (Fig 1). There was intense edema surrounding the lesions, which were remarkably limited to the dorsa of both his hands and fingers. The lesions were painful but not accompanied by adenopathy, fever, or malaise. Medical his-
J AM ACAD DERMATOL JUNE 2002
tory was significant for adenocarcinoma of the colon 3 years before. Laboratory investigations revealed an elevated white blood cell count of 14.8 with neutrophilia (11.8 ⫻ 109/L), erythrocyte sedimentation rate of 81 mm/h, and fibrinogen level of 991 mg/dL. Cultures of specimens from the lesions for bacteria and viruses were all negative. The biopsy specimen revealed a partially necrotic epidermis with marked exocytosis of neutrophils. There was a prominent papillary dermal edema with presence of some neutrophils and a dense neutrophilic infiltrate with leukocytoclasis in the reticular dermis. Very scattered and focal images of fibrinoid necrosis can be observed in isolated dermal vessels but not a true leukocytoclastic vasculitis. No signs of recidivism or metastases of colon carcinoma were detected. The patient had been treated with antibiotics without any response. We started oral administration of prednisone (45 mg/d), and the lesions cleared rapidly without scarring. Seventeen months later, the patient had a new flare, less intense than before, which showed a dramatic response to prednisone. The cases published by Galaria et al1 and Strutton et al2 and our case all share the main clinical and histopathologic features of Sweet’s syndrome.3 The only difference is the presence or absence of vasculitis. Nevertheless, not all the cases are so clear. Strutton et al2 describe 2 cases (numbers 1 and 5) as Sweet’s syndrome with a vasculitic component, and case number 2 in the report by Galaria et al1 shows some perivascular fibrin deposition. On the basis of histopathologic findings, we consider our case to be Sweet’s syndrome with isolated and partial phenomena of fibrinoid necrosis.4 One explanation for these differences could be the evolution of the lesions; all of the patients described by Strutton et al underwent biopsy at least 1 week after the onset of the lesions, Galaria et al1 do not give the precise time, and our patient had a biopsy performed only 48 hours after the onset. Therefore, there could be a continuum, considering the vascular damage caused by the intense inflammatory neutrophilic infiltrate.3 We agree with the proposal of Galaria et al1 to name this entity neutrophilic dermatosis of the dorsal hands and consider it as a localized form of Sweet’s syndrome. Yolanda Gilaberte, MD Carmen Coscojuela, MD Maria Dolores Garcı´a-Prats, MDa Departments of Dermatology and Pathologya Hospital San Jorge Huesca, Spain E-mail: [email protected]
Letters 963
J AM ACAD DERMATOL VOLUME 46, NUMBER 6
Fig 1. Hemorrhagic bullous lesions with pustules on dorsal right hand.
REFERENCES 1. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol 2000;43:870-4. 2. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995;32:192-8. 3. Cohen PR, Kurzrock R. Sweet’s syndrome: a neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol 2000;18:265-82. 4. Jordaan HF. Acute neutrophilic dermatosis: a histopathological study of 37 patients and a review of the literature. Am J Dermatopathol 1989;11:99-111. 16/8/120574 doi:10.1067/mjd.2002.120574