leukocytoclastic vasculitis in urticaria induced by sun exposure
TRANSCRIPT
Brief communication
Leukocytoclastic vasculitis in urticaria induced by sun exposure
Giuseppe Stinco1, Luca Di Gaetano1, Claudio Rizzi2, Pasquale Patrone1
1Department of Clinical and Experimental Pathology and Medicine, Institute of Dermatology, University of Udine, Udine, Italy, and 2Institute of
Pathology, A.S.S. ‘Bassa Friulana’, Pamanova (Udine), Italy
Background: Solar urticaria manifests itself immedi-
ately after solar or artificial light exposure and dis-
appears a little later. Histopathologic findings of solar
urticaria are essentially identical to those of classic
urticaria.
Case report: We report a 41-year-old man who devel-
oped urticarial lesions some hours after sunlight ex-
posure, which resolved after approximately 1 week.
Histologic examination of the lesions evidenced a
leukocytoclastic vasculitis.
Conclusion: In a literature review we found one case
of solar urticaria with histologic aspects of leukocyto-
clastic vasculitis.
Key words: leukocytoclastic vasculitis; phototest solar
urticaria.
Solar urticaria is a relatively rare form of physical
urticaria. It manifests immediately after solar or
artificial light exposure and disappears after some
minutes or hours. The triggering radiations are UVA
(320–400 nm) and visible light (400–600 nm), less fre-
quently the UVB (280–320 nm) and very rarely the
infrared (4600 nm). The clinical and histologic fea-
tures are similar to those of common urticaria (1, 2).
Vasculitic urticaria is characterized by wheals of
small-medium dimensions that persist for more days,
can be more painful than pruritic, and sometimes can
revert, leaving purpuric lesions. The biopsy of vascu-
litic urticaria reveals a leukocytoclastic vasculitis. The
cutaneous lesions may be accompanied by fever,
arthralgias, abdominal and thoracic pain, glomerulo-
nephritis and uveitis (3). Recently, we have observed a
case of solar urticaria with the histologic character-
istics of leukocytoclastic vasculitis. In the literature,
only one similar case is described (4).
Case reportA 41-year-old man presented with wheals of approxi-
mately 1 cm diameter, at times confluent, after sun-
light exposure, with the first episode occurring 9 years
ago. The lesions initially appeared only on the fore-
arms; they were non-pruritic and non-painful, devel-
oped some hours after sun exposure of large body
surface area and disappeared approximately after 1
week. The single exposure of the face and hands was
never sufficient to induce the appearance of the
wheals. The manifestations appeared every year in
the summer months with the same characteristics and,
in the last 3 years, they also extended to the superior
and inferior limbs (Fig. 1). The patient is otherwise in
good health and does not take any medication. There
was no family history of urticaria.
The laboratory studies turned out to be normal;
there were no alterations in the inflammatory indices,
autoantibodies and porphyrin assay.
Phototests were conducted. We used the unit of
phototherapy Daavlin Spectras
724-sp UVA as a
source of UVA radiations (320–400 nm), the unit of
phototherapy Daavlin Spectras
724-sp UVB as a
source of UVB radiations (280–320 nm). As a source
of visible light we used a slide projector. Irradiance of
UV sources was measured by a photometer (Wald-
mann’s UV-meter). Physical measurements of the
irradiance and spectrum of the slide projector lamp
were not available. To determine MED-UVA the
patient was exposed to 5–7.5–10 J/cm2 and no reaction
was observed. To determine MED-UVB, we used 30–
90–150–210–300mJ/cm2 and after 24 h the minimal
erythema dose was 90mJ/cm2.
In the attempt to determine the minimum urticarial
dose (MUD), we irradiated areas of 5 � 5 cm with 10
and 20mJ/cm2 of UVB, 10 and 20 J/cm2 of UVA and
with visible light for 30min. The test was read im-
mediately, after 6 and 24 h, and it was negative.
Subsequently, the patient was exposed to irradiation
Photodermatol Photoimmunol Photomed 2007; 23: 39–41Blackwell Munksgaard
r 2007 The Authors.Journal compilationr 2007 Blackwell Munksgaard
39
of the whole body surface with 10mJ/cm2 of UVB and
10 J/cm2 of UVA, with no appearance of wheals. A
provocative test with natural sunlight was performed.
Only a 30-min exposure on a sunny day produced a
positive reaction in our patient, determining the pre-
sence of wheals in the superior and inferior limbs.
The other physical tests for urticaria were negative.
Histologic examination of a wheal in the thigh area
evidenced leukocytoclastic infiltrate within and
around the walls of blood vessels at the superficial
and deep level of the dermis (Fig. 2a and b). Inside the
vessel wall, numerous neutrophils and lymphocytes,
some plasma cells and fragments of nuclei from
neutrophils (nuclear dust) were recognized (Fig. 2c).
The endothelial cells appeared swollen. This picture
was compatible therefore with leukocytoclastic vascu-
litis.
In the past the patient had used hydroxyzine (25mg
twice a day), cetirizine (10mg daily) and loratadine
(10mg daily) without benefit. A combination of lor-
atadine (10mg daily) and ranitidine (150mg twice a
day) did not improve the clinical course of the disease.
He was also not compliant with the use of sunscreens.
On two occasions, systemic corticosteroids, oral pre-
dnisone (50mg daily) or deflazacort (60mg daily) did
not modify the duration of the manifestations.
DiscussionSome cases of solar urticaria have been described in
the literature, where the wheals appeared after a
longer period of latency and their duration was longer,
but there has never been a histologic documentation
(1). In only one case the histologic examination
revealed a leukocytoclastic vasculitis. It was a case
of a 35-year-old woman who, similar to our patient,
presented urticarial lesions that appeared 1–4 h after
irradiation with sunlight or artificial UV radiations
and lasted several days before resolving. The patient
used sunscreens to control the manifestations (4).
In our case, the wheals do not develop after the
exposure of the face and hands, but only if the patient
exposes larger areas of the body. The phototests we
performed were negative and the complete irradiation
of the entire body surface was not sufficient to elicit
the reaction. Only a provocative test with natural
sunlight produced a positive reaction. Consequently
Fig. 1. Wheals in the thigh area appeared 3 h aftersunlight exposure at the seaside.
Fig. 2. Perivascular infiltrate at the superficial anddeep level of dermis (hematoxylin–eosin, originalmagnification � 10) (a); leukocytoclastic infiltratewithin and around wall of a blood vessel, which showsswelling of endothelial cells (hematoxylin–eosin, ori-ginal magnification � 20) (b); nuclear dust secondaryto degeneration of neutrophils (hematoxylin–eosin,original magnification � 40) (c).
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Stinco et al.
it was not possible to establish the wavelength of the
radiation which could induce the urticaria. However,
the inability to reproduce the manifestations of the
solar urticaria by using phototests was already known
in the literature (5). It depends mainly on the fact that
the tests are conducted with exposures of few minutes
on small body areas, while the wheals appear after a
longer sunlight exposure on large body surface areas.
We do not know exactly whether the wheals appear
after a long and massive exposure to light or whether
there is a local factor that determines the development
of the wheals localized on the limbs and spares the
other photo-exposed regions.
The most difficult differential diagnosis is polymor-
phous light eruption (PLE). We consider that our
patient did not have a PLE because he showed only a
monomorphic eruption of wheals localized only on
the limbs, while the most frequent locations of this
photodermatosis, the V area and the neck, were not
involved. Moreover, in the literature leukocytoclastic
vasculitis has never been described in PLE, whose
histologic features consist of spongiosis, acanthosis,
parakeratosis and perivascular round cell infiltrate of
the dermis.
Considering the rarity of solar urticaria, the treat-
ment of choice is not known. The patient used anti-H1
antihistamines alone and in association with anti-H2,
but they were not effective in prophylaxis and did not
modify the evolution of the wheals. Not even the
usage of systemic corticosteroids influenced the clin-
ical outcome. The patient was noncompliant in using
sunscreens so we do not have any data on their
protective capacity, even though we assume their
effectiveness if applied in a constant and careful way.
A therapeutic approach could be the use of photo-
therapy (PUVA), or the prescription of colchicine or
systemic photoprotectors such as hydroxychloro-
quine. Considering the random appearance of the
lesions and their mild clinical course, we think that a
preventive measure of avoiding solar exposure and the
follow-up of the patient are the preferred course of
treatment.
References1. Beattie PE, Dawe RS, Ibbotson SH, Ferguson J. Characteristics
and prognosis of idiopathic solar urticaria. Arch Dermatol
2003; 139: 1149–1154.2. Horio T. Solar urticaria – idiopathic? Photodermatol Photo-
immunol Photomed 2003; 19: 147–154.
3. Iannello S, Asmundo GO, Cataliotti A, et al. Urticarial vascu-
litis syndrome. A case report and review of the literature.Minerva Med 1997; 88: 459–467.
4. Armstrong RB, Horan DB, Silvers DN. Leukocytoclastic vas-
culitis in urticaria induced by ultraviolet irradiation. Arch
Dermatol 1985; 121: 1145–1148.5. Ryckaert S, Roelandts R. Solar urticaria. A report of 25 cases and
difficulties in phototesting. Arch Dermatol 1998; 134: 71–74.
Accepted for publication 19 October 2006
Corresponding author:
Giuseppe Stinco
Institute of Dermatology, University of Udine,
Ospedale ‘San Michele’
piazza Rodolone 1
33013 Gemona del Friuli (Udine), Italy
Tel: 139-0432-989-78
Fax: 139-0432-989-209
e-mail: [email protected]
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Leukocytoclastic vasculitis in urticaria