ARTICLE
Auteur(s) : Hui-Jun
Ma, Guang Zhao, Wen Liu, Yu-Ping Dang, Dong-Guang Li
Department of Dermatology, The Airforce General Hospital of
Chinese People’s Liberation Army, 30 Fucheng Road, Haidian
District, Beijing, 100036, P.R.China
accepté le 24 Novembre 2006
Unilateral linear capillaritis (ULC), also named quadrantic
capillaropathy or unilateral pigmented purpuric eruption, is a rare
variant of pigmented purpuric dermatosis(PPD) [1]. This condition
was first reported in 1992 by Riordan [2], and remains
characterized clinically by an extensive linear or segmental
distribution of pigmented purpuric macules, mainly affecting
adolescent boys and young men, located predominantly on the lower
extremities, and showing a favorable prognosis [1]. The
pathological feature of the entity is similar to PPD.Pigmented
purpuric dermatosis (PPD) is usually considered a capillaritis of
unknown cause. The term is used for a group of mainly asymptomatic,
sometimes pruritic dermatoses that are clinically characterized by
an eruption of purpuric lesions along with yellow, orange, red
and/or brown, often patchy pigmented areas [3]. Based on varying
clinical features, eight subtypes of PPD have been distinguished
(Table 1 ) [4]. The histopathological
findings are often indistinguishable and share common features
including perivascular lymphocytic inflammation, erythrocyte
extravasation, and hemosiderin deposition. More recent
investigations consider these subtypes as clinical variants with a
common pathogenic background [5].To date, there are altogether ten
cases of ULC that have been reported in the literature [1, 2, 6-8].
Herein, we describe two further unusual cases of ULC, both of which
showed an extensive linear distribution on the upper extremities
that loosely followed the dermatome lines, but not the course of
vessels or Blaschko lines.
Case report
Patient 1
(Table 1) A healthy 29-year-old Chinese
male presented with a 9-month history of an asymptomatic
progressive pigmented purpuric eruption which commenced around the
right elbow and spread to involve the right axillary fossa and the
wrist. Examination revealed florid macules in a patchy linear
distribution over the flexor aspect of the right arm and involving
the right axillary fossa and upper chest. Some scattered punctate
purpura was evident in the pigmented macule (figures 1A and B). No
antecedent trauma and oral drug history were reported. Routine
laboratory analysis including total leucocyte count, erythrocyte
and platelet counts was unremarkable. The analysis of antinuclear
antibody (ANA) and extractable nuclear antigen (ENA) test was
normal. Erythrocyte sedimentation rate was also within normal
limits. The result of a Rumpel-Leede test (capillary fragility
test) was negative, which differs from what would be expected of
common acute PPD. Histology showed many focal clusters of
perivascular lymphocytic infiltration around the upper-middle
dermal capillaries with marked extravasation of red blood cells and
foci of haemosiderin figures 1C and D). The
histological appearance was consistent with PPD. The patient had
been previously treated for 2 months with oral tripterygium
wilfordii in another hospital, six months after the initial onset,
due to a misdiagnosis of subacute cutaneous lupus erythematosus,
but there was no apparent clinical improvement. On review 20 months
after the onset, the eruptions had faded spontaneously from the
axillary fossa area, but a few lesions were still present on the
upper chest and the forearm (figure 1E).
Table 1 Classification of pigmented purpuric
dermatosis
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1. Purpura annularis telangiectodes (Majocchi disease)
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2. Progressive pigmentary dermatosis (Schamberg’s disease)
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3.Pigmented purpuric lichenoid dermatitis (Gougerot-Blum
disease)
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4. Eczematid-like purpura (Doucas-Kapetanakis disease)
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5. Itching purpura (disseminated pruriginous angiodermatitis)
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6. Lichen aureus (lichen purpuricus)
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7. Unilateral linear capillaritis (quadrantic capillaropathy)
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8. Granulomatous pigmented purpura
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Patient 2
An otherwise healthy 23-year-old Chinese female noticed
asymptomatic red-brown purpuric macules on the left elbow when she
was in her eighth month of pregnancy. There was a gradual extension
of similar macules along the extensor aspect of the left arm
respectively onto the left wrist and shoulder over a 15-month
period. The spontaneous delivery of her pregnancy did not stop the
aggravation of the eruption, on the contrary, three additional
purpuric macules further developed on the extensor aspect of her
left thigh. In March 2006, she came to our department for further
diagnosis and treatment. Examination revealed some faba bean-sized
pigmented purpuric macules, consisting of closely aggregated,
superficial, small red-brown flat papules, extending in a linear
distribution from the left wrist up to the extensor aspect of the
left arm to the shoulder (figure 2A). Three purpuric
pigmentations were also noted to be grouped on the extensor aspect
of the left thigh. The patient denied any medical history of oral
drug use or trauma. Results of a Rumpel-Leede test were also
negative. Skin biopsy from the left forearm showed a mild
orthohyperkeratosis and partial flattening of the rete ridges in
the epidermis. A focal perivascular lymphocytic inflammation was
observed in the upper dermis, interspersed with extravasated
erythrocytes (figure
2B). Although iron stains for haemosiderin were negative,
the histological findings were consistent with a diagnosis of PPD.
She was treated orally for 4 weeks with hydroxychloroquine and
topically with steroid cream, but there was no clinical
improvement. After 2 months of therapy with PUVA, the purpuric
eruption became less visible and leaving a faded pigmentation. No
relapse has occurred since then.
Discussion
Unilateral linear capillaritis (ULC) was first reported in 1992 by
Riordan [2]. The terms quadrantic capillaropathy [5] and unilateral
pigmented purpuric eruption [1] have also been used to describe
this condition. ULC is characterized clinically by an extensive
linear or segmental distribution of pigmented purpuric macules,
mainly affecting adolescent boys and young men. These lesions are
located predominantly on the lower extremities, and show a
favorable prognosis [1, 2]. Because of its distinct clinical
character and similar histopathological findings to PPD, in some
textbooks of dermatology, ULC has been classified as a new uncommon
subtype of PPD [4]. The reasons why the lesions were distributed
unilaterally and linearly are, however, still unknown.
The diagnosis of PPD is generally made clinically and supported
by histopathological examination. No consistent laboratory
abnormalities have been identified [4]. In fact, the appearance of
extensive pigmented purpuric eruptions in our cases are consistent
with the characteristics of PPD. The two patients we present both
reveal histopathological features typically seen in PPD
characterized by dermal focal lymphocytic infilatration with
extravasation of red blood cells. The diagnosis of PPD can
therefore be made in our patients. Our cases are notable for their
extensively unilateral linear distribution on the upper extremities
and their benign prognosis, for this reason we further classify the
lesion according to the established subtypes as ULC. The young age
of our patients differs from Schamberg’s disease which most
commonly affects elderly patients. The following features of our
cases were consistent with these previous reports of ULC: the lack
of prodromal symptoms; the distribution of eruptions along one side
of the body; the age of onset between 7 and 38 years [1, 2]; and
the resolution of the purpura occurring over a period of months.
Our cases are also unusual because the lesions seen are localized
on the upper extremities which is a rare occurrence in PPD and
never reported in ULC. In patient 2, the extent of lesions
(apparently, thigh and arm on the same side) is different from any
published cases. The eruption may be considered to have developed
within two different dermatomes.
Several other subtypes of PPD may need to be considered in the
differential diagnosis of ULC. Among them, ULC is most closely
represented as Lichen aureus (LA). They all have a predilection for
young adults with a peak incidence in the second and third decade.
In particular, they can present with a unilateral and linear or
segmental distribution, but the lesions of LA can often be solitary
or numerous and linear segmental lesions may occasionally occur.
These lesions tend to be chronic, remaining stable or slowly
progressive [8]. However more commonly, the lesions of ULC are more
extensive and often resolve spontaneously within a timeframe of
approximately 2 years [1, 2, 6-8]. ULC has a typical pathology of
PPD [1, 2], however, LA has a distinctive histology where there is
a band-like dermal inflammatory infiltrate that accompanies the
typical pathology of the PPD [9]. In addition, secondary causes of
PPD need to be excluded, and the possibility of mycosis fungoides
should be borne in mind.
The etiology of ULC is unknown. Several explanations concerning
the pathogenic mechanism of PPD have been postulated. One possible
explanation is the involvement of cellular immunity. Most of the
infiltrating cells in the lesions were CD4+ T cells, and
these cells, as well as keratinocytes, express HLA-DR antigens
[10]. However, the hypothesis cannot explain the evidence that
treatment with tripterygium wilfordii had no effect on the skin
lesions in Patient 1, but after 20 months of onset, the eruption of
ULC disappeared spontaneously. Other investigators have suggested
drugs [8], chemical ingestion [11], capillary fragility [12],
infection [13] or venous stasis [14] as causative factors of PPD.
However, there was no evidence of these conditions observed in our
cases. In addition, the lesions in Patient 2 originally developed
when she was in her eighth month of pregnancy. It seems unlikely
that there is some link between ULC and pregnancy because the
patient’s spontaneous delivery did not stop the aggravation of the
lesions.
Acknowledgments
Financial support: none.
Conflict of interest: none.
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