doi: 10.1111/1346-8138.

13270 Journal of Dermatology 2016; : 1–4

CONCISE COMMUNICATION
Recurrent neutrophilic dermatosis of the face: A report of
two cases and review of the published work
Tomoko MAKI, Koichi YANABA, Yozo ISHIUJI, Yoshinori UMEZAWA, Akihiko ASAHINA,
Hidemi NAKAGAWA
Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan

ABSTRACT
Recurrent neutrophilic dermatosis of the face is a disease that is morphologically and histopathologically compat-
ible with Sweet’s syndrome, but is distributed on the face without fever, laboratory abnormalities or associated
disorders. At present, it is unclear whether our cases belong to the chronic and mild variant of Sweet’s syndrome
or are independent entities. Here, we present two cases of recurrent neutrophilic dermatosis of the face with
good response to systemic corticosteroids or potassium iodine, as well as those of cases reported in the
published work.

Key words: corticosteroid, face, neutrophilic dermatosis, recurrence, review.

INTRODUCTION leukocytoclastic debris in the dermis (Fig. 1b), without vasculi-

In 1968, Whittle et al.1 described two patients who presented
with recurrent lesions on the face. Histological findings were
compatible with a diagnosis of Sweet’s syndrome, but there
was no fever or any associated disorders. Therefore, these
cases were considered a localized variant of Sweet’s syn-
drome and the condition was named recurrent neutrophilic der-
matosis of the face. Here, we report two cases that seemed to
fit a diagnosis of recurrent neutrophilic dermatosis of the face
and we review the published work.
CASE REPORTS
Patient 1
A 31-year-old Japanese woman presented with a 4-year
history of recurrent eruptions on the face. Physical examination
revealed multiple painful erythematous, edematous plaques
with superficial ulceration on the forehead and around the eyes
(Fig. 1a). There were no general symptoms, such as fever or
arthralgia. She was initially treated with oral and topical antibi-
otics and systemic agents for herpes virus at another hospital
but did not improve. Bacterial culture of the skin lesions was
negative and laboratory investigations showed no abnormal
findings in the complete blood count, erythrocyte sedimenta-
tion rate (ESR), C-reactive protein (CRP) level or antinuclear
antibody (ANA). Serological tests for immunoglobulin (Ig)M anti-
bodies specific for Epstein–Barr virus and cytomegalovirus
were negative. The result of serum immunoelectrophoresis was
normal. Skin biopsy revealed moderate perivascular and peri-
adnexal cell infiltrates composed of mainly neutrophils with
tis or fibrinoid necrosis (Fig. 1c). Direct immunofluorescence
was negative. We therefore made a diagnosis of neutrophilic
dermatosis. Because therapy with topical corticosteroids was
ineffective, she was treated with oral prednisolone 15 mg/day
leading to marked improvement. Diaminodiphenyl sulfone
50 mg once daily was added, followed by subsequent tapering
of the prednisolone dose to 2 mg/day.
Patient 2
A 36-year-old Japanese woman was referred to us because of
an 8-month history of recurrent eruptions on the face. She was
initially treated with topical corticosteroids at another hospital
but showed only slight improvement. Physical examination
revealed multiple, painful, erythematous, edematous plaques
on the forehead (Fig. 2a). No fever or other general symptoms
were observed. Laboratory tests showed no abnormal findings
in white blood cell count, ESR, CRP level or ANA. Serological
tests for IgM antibodies specific for Epstein–Barr virus and
cytomegalovirus were negative. The result of serum immuno-
electrophoresis was normal. Skin biopsy revealed massive
neutrophil infiltration in the upper to middle dermis (Fig. 2b,c).
Epidermal spongiosis and neutrophilic exocytosis, in addition
to leukocytoclastic debris, were also observed, although vas-
culitis and fibrinoid necrosis were absent. A diagnosis of
neutrophilic dermatosis was made and the patient was
treated with potassium iodide for 1 month, which resulted in
obvious improvement. Although discontinuation of potassium
iodide resulted in rapid recurrence in the same area, she
showed marked improvement after the treatment was started
again.

Correspondence: Koichi Yanaba, M.D., Ph.D., Department of Dermatology, The Jikei University School of Medicine, 3-25-8 Nishishimbashi,
Minato-ku, Tokyo 105-8461, Japan. Email: yanaba@jikei.ac.jp
Received 15 October 2015; accepted 21 November 2015.

© 2016 Japanese Dermatological Association 1

T. Maki et al.
(a)
(b)
(c)
Figure 1. Clinical and pathological findings of patient 1. (a)
Erythematous lesions are present on the forehead and around
the eyes. (b) Perivascular and periadnexal cell infiltrations are
found in the dermis (hematoxylin–eosin [HE], original magnifica-
tion 940). (c) Dense infiltrates with a predominance of neu-
trophils are seen under high-power magnification (HE, 9400).
2 © 2016 Japanese Dermatological Association
(a)
(b)
(c)
Figure 2. Clinical and pathological findings of patient 2. (a)
Erythematous lesions are present on the forehead. (b) Diffuse
inflammatory cell infiltrates are found in the dermis (hema-
toxylin and eosin [HE], original magnification 940). (c) Inflam-
matory cells consisting of mainly neutrophils are seen under
high-power magnification (HE, 9400).
 Recurrent neutrophilic dermatosis of the face

Table 1. Comparison of clinical features of recurrent neutrophilic dermatosis of the face

Age Laboratory Associated

Case (years) Sex Fever abnormalities conditions Treatment Response Recurrence Reference
1 34 Female No Leukocytosis, No Systemic steroids Good Yes Whittle et al.1
neutrophilia
2 57 Male Yes Leukocytosis, No Systemic Good Yes Whittle et al.1
neutrophilia, erythromycin
high ESR
3 43 Female No No No Systemic steroids Good Yes Borrie7
4 50 Female No No No Systemic steroids Good Yes Arita et al.8
5 31 Female No No No Systemic steroids, Good Yes Current case 1
diaminodiphenyl
sulfone
6 36 Female No No No Systemic steroids, Good Yes Current case 2
potassium iodine

ESR, erythrocyte sedimentation rate.

DISCUSSION Several cases of the chronic variants of Sweet’s syndrome

have been reported, named chronic recurrent annular neu-
Sweet’s syndrome, also known as acute febrile neutrophilic
trophilic dermatosis9 and chronic recurrent neutrophilic der-
dermatosis, is characterized by fever, leukocytosis and painful
matosis.10,11 Similar to our cases, these variants generally lack
erythematous plaques with neutrophil infiltration.2 The major
fever, laboratory abnormalities and associated conditions. Con-
diagnostic criteria for Sweet’s syndrome are abrupt onset of
sidering that the face is one of the most frequently affected
painful erythematous skin lesions and neutrophil infiltration in
sites in Sweet’s syndrome,12 it is possible that our cases were
the dermis without leukocytoclastic vasculitis; minor criteria
a chronic variant of Sweet’s syndrome. Follow up of the dis-
are accompanying general malaise and fever, associated
ease course in our cases is necessary to monitor for develop-
infection, inflammatory or malignant disease, elevated serum
ment of skin lesions on sites other than the face or a change
inflammatory markers, and good response to systemic corti-
to a typical presentation of Sweet’s syndrome. Accumulation
costeroids or potassium iodine. The presence of one major
of additional cases is required to clarify whether recurrent neu-
and two minor criteria is required for a diagnosis of Sweet’s
trophilic dermatosis of the face is a localized mild form of
syndrome.3,4 Some localized variants of Sweet’s syndrome,
Sweet’s syndrome or an independent entity.
such as neutrophilic dermatosis of the dorsal hand and palm,
have been proposed.5,6 However, it is still controversial
CONFLICT OF INTEREST: None declared.
whether these are indeed subsets of Sweet’s syndrome or
independent entities.
We reviewed six possible cases of recurrent neutrophilic
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