ARTICLE
Eruptive pseudoangiomatosis was first reported by Cherry et al.
in four children [1]. The authors recognized the role of echovirus infection.
Since then, occasional cases of this entity, always affecting children,
have been reported. The disease has never been observed in adulthood.
We describe here, for the first time, nine cases arising in adult patients
over a ten year period.
Clinical report
We report on 9 patients who were referred to our service for the same
eruption. The patients, 8 women and one man (24 to 94-year-old) had an
acute eruption of numerous papules measuring 2-5 mm in diameter. They
were disseminated on the face, limbs and trunk. Mucous membranes were
spared. Palms and soles were rarely involved. The papules were erythematous
and slighty telangiectatic, surrounded by a white halo. The lesions blanched
completely with pressure, and refilled from the center on release. Slight
pruritus was observed in some patients. Dermographism was absent in all
patients. The duration of the eruption ranged from 1 to 3 months. During
this period few lesions resolved and several new lesions developed in
some patients. Finally, all lesions disappeared spontaneously within 1
to 3 months. Clinical examination revealed no abnormalities other than
the cutaneous eruption. In particular, no adenomegaly, liver or spleen
enlargement was observed. The main clinical, epidemiological and evolutive
data are summarized in Table I.
The results obtained in four patients who had paraclinical investigations,
including skin biopsies and serological analysis are detailed.
Case 1
A 47-year-old white woman, nurse in a psychiatric hospital, was referred
to our institution for an eruption of little telangiectatic papules of
2-5 mm in diameter, surrounded by a blanching halo, affecting the arms,
legs and abdomen (Fig. 1).
The patient explained that the eruption had begun 2 months earlier, after
a febril, flu-like episode of illness. She complained of pruritus and
asthenia but, except for cutaneous lesions, the clinical examination was
normal without any adenomegaly or hepatosplenomegaly.
Histological examination revealed dilatation of dermal capillaries associated
with a slight lymphocytic perivascular infiltrate. The laboratory results
included hematocrit 43.8%, platelet count of 268,000/mm3, leucocyte
count of 12,300/mm3 (with 8,831 neutrophils, 2,595 lymphocytes
and 615 eosinophils), and a sedimentation rate of 12. The following serological
results were negative or showed evidence of a past infection of echovirus,
hepatitis B and C, EBV, Coxsackie virus, CMV, HSV 1 and 2. The serodiagnosis
of Parvovirus B19 showed the presence of positive IgM and negative IgG.
A control was undertaken 1 month later and was still positive for IgM
and negative for IgG, suggesting a false-positive reaction. Moreover,
the PCR assessement for Parvovirus B19 in the skin was negative. The lesions
disappeared spontaneously in 3 months.
Case 2
A 77-year-old woman was referred to our institution for an eruption
of telangiectatic papules surrounded by a blanching halo, disseminated
on the trunk and all four limbs. The patient had undergone chemotherapy
for a metastatic ovarian adenocarcinoma. No relation between eruption
and the chemotherapy course could be established. Histological examination
showed a slight atrophy of the epidermis. The superficial dermis vessels
were dilated and endothelial cell nuclei were prominent in the vessel
lumen. A polymorphous perivascular infiltrate without vasculitis was observed
in the superficial dermis. The serological examination was negative or
highlighted an old infection for the following viruses: EBV, CMV, Parvovirus
B19, Coxsackie A and B and echovirus. The lesions disappeared spontaneously
within 2 months.
Case 3
An 88-year-old woman, living in a retirement home was refered to us
for an eruption of numerous nonpruritic pseudoangiomatous papules measuring
2-4 mm in diameter. The eruption was localized on the face, trunk, arms
and dorsal aspect of the hands without any associated symptoms or recent
administration of new drugs. Histological examination of lesions showed
dilated vessels in the upper part of the dermis surrounded by mononuclear
cells and some polymorphonuclear neutrophils without evidence of vasculitis
or proliferation of the vessels. The lesions resolved spontaneously in
6 weeks.
Case 4
A 55-year-old woman was hospitalized in the respiratory disease unit
for asthma decompensation secondary to a probable pulmonary viral infection.
She had a nonpruriginous pseudoangiomatous eruption localized on the face,
neck, trunk and dorsal aspect of the hand (Figs.
2 and 3) .The lesions disappeared
without any treatment in a few weeks. Histological examination of the
skin showed dilated vessels lined with plump endothelial cells in the
superficial dermis. The cell nuclei were sometimes slighty enlarged. A
perivasular infiltrate with lymphocytes, neutrophils and eosinophils was
present but without any vasculitis (Fig.
4).
Discussion
For the first time, we report a series of nine adult patients, eight
women and one man (24 to 94 years old) presenting with a cutaneous eruption
similar to a disease that until now has only been described in children,
i.e. eruptive pseudo-angiomatosis.
Eruptive pseudo-angiomatosis was first described in 1969 by Cherry et
al. [1] in four children with an acute echovirus infection. The lesions
were described as erythematous papules of 2-4 mm diameter surrounded by
a 1-4 mm white halo. The lesions disappeared after pressure and refilled
from the center on release. The role of echovirus was suspected on account
of the presence of this virus in the four children. Lesions were preceded
by fever, pharingitis, diarrhea or vomiting in some patients. The papules
disappeared spontaneously in a few days. Others publications have confirmed
the clinical specificity of this eruption in children but the role of
echovirus has never been well documented [2, 3]. Histological examination
was first undertaken by Prose et al. [2], showing dilated blood
vessels with plump endothelial cells but no evidence of an increased number
of vessels and this author proposed the term eruptive pseudoangiomatosis.
To our knowledge, all reported cases concern only children and the disease
has never been described in adulthood. The nine patients described in
our series had an eruption very similar to those reported in children.
However, three differences were noted when comparing the children and
adult forms: firstly, in children, the eruption follows an episode of
malaise and fever, but this was only observed in two adult patients; secondly,
the duration of the disease appears to be longer in adulthood than in
children. Indeed, the lesions disappear in adults within 1-3 months, whereas
in children, they disappear after few days. Finally, we noted a predominance
of female patients in adulthood (8 cases among 9) but not in children.
The histological picture was also similar to those reported by Prose et
al. In the four cases we biopsied, the vessels were dilated and lined
with plump endothelial cells. A sparse perivascular infiltrate with lymphocytes
and polymorphonuclear neutrophils, more rarely eosinophils, was present
in the superficial dermis. The epidermis remained normal. There was no
vasculitis. This pattern is not specific and can be observed in some dermatological
disorders such as viral exanthemas or drug eruption. A viral etiology
is highly probable but no systematic research was undertaken in our patients.
Serological examination was not informative for the three patients tested
and acute echovirus infection was not demonstrated (cases 1, 2 and 9).
However, it was interesting that, in four of the nine patients, the disease
onset occurred during hospitalization for treatment of various conditions,
including metastatic cancer and asthma. Among the five remaining patients,
two were hospitalized in a retirement home and one was a nurse in a psychiatric
hospital. These epidemiological characteristics suggests that the eruption
could be linked with an infectious disease. In some patients, an immune
deficiency (chemo or radiotherapy for cancer in three cases), a previous
bronchial disease (1 case) or residence in a collectivity could favour
viral infection development. The chronology of the eruption and disappearance
of lesions despite continuation of the previous treatment or the lack
of administration of new drugs are not in favour of an adverse drug eruption.
The lesions should be differentiated from other
acute vascular eruptions. Acquired telangiectasia does not disappear spontaneously.
The papules are quite different from spider angioma, multiple pyogenic
granulomas or bacillary angiomatosis, and histological examination showed
the absence of vascular proliferation but only slight vessel dilatation.
A case resembling tufted angioma with spontaneous healing in a liver transplant
recipient has been described, but in this case, clinical appearance is
different because of the large size of the lesions. Moreover, histological
examination shown densely cellular lobules of capillaries rimmed by myxoid
connective tissue [4]. Eruptive vascular tumors associated with chronic
graft-versus-host disease [5] is clinically different. The lesions
appears as black or violaceous friable nodules measuring 1 mm to 2 cm
in diameter and look like pyogenic granuloma. Differential diagnosis could
be more difficult with eruptive angiomatosis [6]. This entity has been
described in association with POEMS syndrome, lymphoma, cancer [7] or
after cyclosporine treatment in a patient with psoriasis [8]. The clinical
aspect is similar to cherry angioma or Campbell de Morgan spots. Similarly,
Seville et al. described an angioma outbreak in more than 1,000
patients 30 years ago in England [9]. In these cases, the eruptions had
an epidemic aspect but they found no evidence of any virus or other pathogenic
organism. An increase in lesions was observed when the temperature rose.
The lesions were true cherry angiomas and did not disappear after pressure.
Histological examination showed a proliferation of dilated capillaries
in the dermis. Eruptive pseudo-angiomatosis is also different from that
described in patients by Colver and Kemmett [7]. These authors described
a group of three young women with rapid onset of petechial rash evolving
over more than 10-36 months. Moreover, histological examination showed
a small anastomotic meshwork of dilated capillaries with no evidence of
inflammatory infiltrate.
The diagnosis of eruptive pseudoangiomatosis could be thus evoked when
there is a typical clinical aspect, even in adult patients and not only
in children. The aetiology remains unclear but is probably of viral origin.
Description of new cases in adulthood and systematic virological studies
could lead to a better understanding of the disease.
Article accepted on 22/5/00
REFERENCES
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lesions associated with ECHO viral infections. Pediatrics 1969;
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3. Calza AM, Saurat JH. Eruptive pseudoangiomatosis: a unique
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4. Chu P, LeBoit PE. An eruptive proliferation resembling acquired
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5. Garnis S, Billick RC, Srolovitz. Eruptive vascular tumors
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8. De Felipe I, Redondo P. Eruptive angiomas after treatment
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134: 1487-8.
9. Seville RH, Rao PS, Hutchinson DN, Birchall G. Outbreak of
Campbell de Morgan spots. Br Med J 1970; 1: 408-9.
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