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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
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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.
INTRODUCTION
IN 1995, STRUTTON et al1 described 6
patients with an eruption involving the dorsal hands. The lesions clinically
resembled those of Sweet syndrome and showed similar histologic evidence of
a dense dermal neutrophilic infiltrate. In contrast to Sweet syndrome, however,
the distribution was limited almost entirely to the dorsal hands, and definite
leukocytoclastic vasculitis was evident histologically. The term pustular vasculitis of the hands was used to describe these cases.
Six similar patients were subsequently described.2-4
Because vasculitis was absent in some of the similar cases, the term neutrophilic dermatosis of the dorsal hands (NDDH) was
recently proposed by authors who consider this disorder to be a subset of
Sweet syndrome.4
We describe clinical and histopathologic findings in 7 patients with
neutrophilic dermatosis or pustular vasculitis of the dorsal hands.
PATIENTS AND METHODS
From June 1, 1990, through August 31, 2000, 7 patients were referred
to the Department of Dermatology, Mayo Clinic Scottsdale, Scottsdale, Ariz,
for a similar pustular eruption on the dorsal hands. In each case, punch biopsies
were performed or previous biopsy specimens were reviewed. Fungal and acid-fast
staining procedures were performed on paraffin sections from biopsy specimens.
Cutaneous culture specimens were collected by means of swabbing the ulcerative
lesions (4 cases) or from punch biopsy specimens (3 cases). The specimens
were collected for the following cultures: bacterial culture in all 7 cases,
fungal culture in 6 cases, mycobacterial culture in 5 cases, and viral culture
in 1 case. An additional biopsy specimen from the dorsal hand of patient 2
was submitted for direct immunofluorescence.
Follow-up information was obtained during patients' subsequent visits
in the department. When needed, additional information was obtained by means
of questionnaires, which were sent to the patients or to the physicians who
had previously referred the patients.
RESULTS
CLINICAL AND LABORATORY DATA
Clinical and laboratory data are summarized in Table 1. All patients were women, who ranged in age from 44 to 82
years; the mean age was 64 years. Three patients had a history of minor trauma
to the hands preceding the eruption. Cutaneous infections were suspected in
most of the patients, and they had been treated unsuccessfully with systemic
antibiotics before evaluation in our department. All patients presented with
severely painful purulent nodules and plaques, frequently accompanied by ulceration,
involving predominantly the dorsal hands (Figure 1). Areas of involvement on the dorsal hands variably included
the radial aspect, the ulnar aspect, or both. In addition to the hand lesions,
other anatomic sites were minimally involved in 5 patients.
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Clinical and Laboratory Data
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Figure 1. Ulcerated pustular plaque on the
dorsal hand of patient 7.
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HISTOPATHOLOGIC, CULTURE, AND DIRECT IMMUNOFLUORESCENCE FINDINGS
Biopsy specimens from hand lesions of all patients showed dense dermal
infiltrates of neutrophils (Figure 2
and Figure 3), sometimes associated
with subepidermal edema. Abundant leukocytoclastic nuclear dust was present
extracellularly and within dermal macrophages. Variable numbers of eosinophils
and lymphocytes were also present. Fibrinoid necrosis of small vessels was
evident in all cases. Epidermal findings included spongiosis, ulceration,
neutrophilic infiltration, and in some cases, neutrophilic microabscesses.
Pseudoepitheliomatous hyperplasia was present in 2 cases. No fungi or mycobacteria
were seen with the use of special stains. All cultures of cutaneous specimens
yielded negative findings.
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Figure 2. Dense dermal infiltrate with intraepidermal
pustules in patient 3 (hematoxylin-eosin, original magnification x40).
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Figure 3. Necrotic vessel (oriented longitudinally)
surrounded by neutrophils and leukocytoclastic debris in patient 7 (hematoxylin-eosin,
original magnification x400).
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In patient 2, an additional biopsy was performed on an inconspicuous
papule on the back. This specimen showed neutrophilic and histiocytic dermal
inflammation consistent with Sweet syndrome. Vascular fibrinoid necrosis was
absent in the back lesion, but was evident in the hand specimen.
Direct immunofluorescence, which was performed on the hand specimen
from patient 2, revealed no vascular deposits of immunoglobulin, complement,
or fibrinogen.
TREATMENT AND CLINICAL COURSE
Follow-up ranged from 1 month to 4 years; median follow-up was 1 year.
Cutaneous lesions resolved in 6 of 7 patients, during a period of 1 to 9 weeks.
Those 6 patients had been treated with prednisone, dapsone, or minocycline
hydrochloride. No hematologic disorder developed in any patient. One patient
died of chronic renal failure shortly after the evaluation of her hand lesions.
COMMENT
Sweet5 described acute febrile neutrophilic
dermatosis in 1964. Most patients with this rare disorder are women, who present
with tender erythematous or violaceous plaques and nodules, predominantly
on the face, upper trunk, and extremities.6
Skin biopsy specimens show dense dermal neutrophilic infiltrates with leukocytoclasis.
Associated findings may include fever, peripheral blood neutrophilia, and
an elevated erythrocyte sedimentation rate. Diagnostic criteria for Sweet
syndrome were proposed by Su and Liu7 in 1986,
and were modified by subsequent authors.6, 8-9
Although the absence of vasculitis has been considered a diagnostic criterion,
some reports describe focal fibrinoid necrosis of superficial vessels in a
minority of cases with Sweet syndrome.10-11
In 1995, Strutton et al1 described 6
women with lesions resembling Sweet syndrome, but with a distribution limited
almost entirely to the dorsal hands. In contrast to most cases of Sweet syndrome,
those 6 cases showed definite leukocytoclastic vasculitis. The term pustular vasculitis of the hands was used to describe this
eruption. In all cases, the hands were the predominant site of involvement,
although lip ulceration was present in 1 patient. Similar dorsal hand lesions
showing fibrinoid vascular necrosis were subsequently reported in 3 patients,
including 2 men.2-3
Galaria et al4 recently described 3 additional
patients with hand lesions that clinically resembled the previous cases of
pustular vasculitis. In their 3 cases, however, dense dermal neutrophilic
infiltrates were present without diagnostic features of vasculitis. They therefore
proposed that the entity be renamed neutrophilic dermatosis
of the dorsal hands and suggested that it was a subset of Sweet syndrome.
Further links to Sweet syndrome were evident in patient 2 of the present
series. The biopsy specimen from her hand showed a neutrophilic dermal infiltrate
with fibrinoid vascular necrosis, whereas the biopsy specimen from a papule
on her back showed a neutrophilic infiltrate without vasculitis. Five of our
patients also had lesions at sites other than the hands. These areas of involvement
were typically minimal and, in some cases, had not been noticed by the patients.
However, the clinical and histopathologic features of the hand lesions were
very similar to those described in previous cases of NDDH. Additional features
resembling Sweet syndrome in these 7 patients included female sex (7 cases),
pathergy (3 cases), fever (3 cases), and peripheral blood neutrophilia (2
cases). At the time of laboratory testing, 1 of the 2 patients with neutrophilia
was already receiving treatment with oral prednisone, which may have caused
the neutrophilia.
The original description of acute febrile neutrophilic dermatosis noted
the association with a preceding upper respiratory tract infection.5 Subsequent reports have linked Sweet syndrome to myeloproliferative
disorders, myelocytic leukemia, visceral malignancies, inflammatory bowel
disease, connective tissue diseases, pregnancy, infections, drug reactions,
and other disorders.6, 8 Because
relatively few reported cases of NDDH exist, the possible systemic associations
in these patients are less clear. In previous reports, associations of uncertain
significance have included a history of breast carcinoma in 2 patients,1 preceding pharyngitis,2
metastatic renal adenocarcinoma,2 and Raynaud
disease with arthritis.4
Three patients in the present series had concurrent bowel disorders
at the onset of their cutaneous lesions. These active problems included diverticulosis,
diverticulitis, acute proctitis (with a sigmoidoscopic appearance suggestive
of diverticulitis or segmental colitis), a tubulovillous adenoma, and possible
diabetic gastropathy. In addition, 1 of the 3 patients had a history of small-bowel
obstruction. It is not clear whether these bowel disorders are related to
the cutaneous eruption. Another neutrophilic dermatosis, bowel-associated
dermatosis-arthritis syndrome, has been reported in patients with previous
bowel bypass procedures and other bowel disorders.12
The syndrome may be related to bacterial antigens and immune complexes that
occur in the setting of bacterial overgrowth in the bowel.13
In contrast to NDDH, bowel-associated dermatosis-arthritis syndrome typically
has a widespread distribution on the upper extremities and trunk.12 Another neutrophil-rich cutaneous disorder, pyoderma
gangrenosum,14 is commonly associated with
inflammatory bowel disease.
In patient 2, an additional possible association included a concurrent
urinary tract infection. Resolution of her hand lesions occurred after antibiotic
therapy for the urinary tract infection and simultaneous treatment with oral
prednisone. Thus, it is difficult to know which action may have contributed
to her improvement.
Sweet syndrome may sometimes occur as a drug reaction.15
In typical cases, the initiation of the medication therapy and the onset of
the eruption are closely associated in time. None of the medication therapy
described in Table 1 had a clear
temporal relationship with eruption in these 7 patients.
Neutrophilic dermatosis of the dorsal hands may respond to treatments
that are commonly used for Sweet syndrome. The usefulness of oral corticosteroids1-4 and dapsone4 has been described previously. Minocycline16-17 may be helpful for pyoderma gangrenosum,
but its use has not previously been reported in NDDH. In the present study,
both patients who were treated with minocycline initially improved, then experienced
subsequent recurrences during minocycline therapy. In addition, minocycline
has been reported as a possible cause of drug-induced Sweet syndrome.18-19
Appropriate treatment of NDDH may be delayed because of misdiagnosis.
Six of the 7 patients in the present report were originally believed to have
cutaneous infections. Systemic antibiotics were given without benefit. Histologic
findings, such as neutrophilic infiltrates and pseudoepitheliomatous hyperplasia,
may also mimic infection.
CONCLUSIONS
Neutrophilic dermatosis of the dorsal hands is a neutrophil-rich cutaneous
disorder, which may be a subset of or closely related to Sweet syndrome. Associated
clinical features include pathergy, fever, and peripheral neutrophilia in
some cases. The localized distribution, clinical appearance, and histopathologic
findings often lead to misdiagnosis as a cutaneous infection. Oral prednisone
and dapsone may be effective treatments, whereas minocycline is of uncertain
benefit. With all treatments, recurrences are common. Bowel disorders, such
as diverticulitis, may be associated with NDDH. Additional studies are needed
to investigate further the systemic diseases that may be associated with this
rare disorder.
AUTHOR INFORMATION
Accepted for publication September 26, 2001.
Corresponding author and reprints: David J. DiCaudo, MD, Mayo Clinic
Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259.
From the Department of Dermatology, Mayo Clinic Scottsdale, Scottsdale,
Ariz.
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