ARTICLE
Auteur(s) :, Alvaro
Leon-Mateos1,*, Manuel Ginarte1, Luis
León2, Jaime Toribio1
1Department of Dermatology Clinical University
Hospital Faculty of Medicine. C/ San Francisco s/n, 15782 Santiago
de Compostela, SpainFax: (+349) 81547094.
2Department of Oncology Clinical University Hospital
Santiago de Compostela, Spain
accepté le 3 Août 2004
The cutaneous mucinoses include a heterogenous group of diseases
characterized by cutaneous deposits of acid mucopolysaccharides
(mucin) in a focal or diffuse form [1, 2]. Reticular erythematous
mucinosis (REM) is a diffuse primary type of cutaneous mucinosis
which occasionally is associated with systemic diseases and
neoplasms. We report a case of REM with atypical telangiectasias in
a patient affected by essential thrombocytosis, who developed a
lung carcinoma.
Case report
A 48-year-old male smoker, with a 3-year diagnosis of essential
thrombocytosis, had an asymptomatic skin lesion over his chest. He
said it had started at the same time as the hematological disorder
and there was no relation to sun exposure or any medication. Later,
the patient reported pain in the lumbar and costal areas associated
to anorexia and loss of weight, during the past two months.
On dermatological examination, he exhibited a macular,
reticulated, erythematous lesion, slightly infiltrated in the
borders and with isolated telangiectasias ( (figure 1) ). Axillar and
inguinal lymph nodes were enlarged. Histopathological examination
showed a slight edema with broad collagen bundles in the upper and
middle dermis associated to a scanty perivascular and
periappendageal infiltrate. Alcian blue (at pH 2.5) stained large
interstitial deposits of mucin in the dermis ( (figure 2) ). Laboratory
examination revealed leukocytosis (11,700/μL; normal:
4,000-10,000/μL), anemia (haemoglobin 1.3 g/dL; normal: 13.5-17.5
g/dL) and an important thrombocytosis (625,000 platelets/μL;
normal: 150,000-400,000 platelets/μL). Erythocyte sedimentation
rate was elevated (90 mm/h; normal: 0-10 mm/h).
Antinuclear antibodies were present at 1/40 titres. Thyroid
antibodies were absent and hormone levels (TSH, T4 and T3) were
normal.
X-ray and computed tomography of the chest revealed a mass in
the upper lobe of the right lung ( (figure 3) ). A bone
scintigraphy showed multiple skeletal lesions in vertebral and
costal areas, and the bone marrow examination was compatible with
metastatic carcinoma.
Diagnosis of REM and stage IV non-small cell lung carcinoma was
made. 200 mg/day hydroxychloroquine treatment was started for his
cutaneous pathology and then the patient was remitted to the
Oncology Service.
Discussion
REM is an infrequent variant of cutaneous mucinosis characterized
by the presence of pruriginous reticulate erythema over the upper
chest and back with typical deposition of mucin in the upper and
middle dermis. Although the origin of REM is unknown,
photosensivity, immunological and viral mechanisms have been
hypothesized [3, 4]. Antimalarials are the classical treatment for
REM lesions, although a recent report describes a case successfully
treated with topical tacrolimus [5].
This type of mucinosis is not habitually associated with
systemic diseases like in other variants, and although several
cases of cutaneous mucinosis associated with different malignancies
have been described [6-8], only two cases of REM have been reported
in patients with colonic and breast carcinoma [9]. To our
knowledge, no previous published case of REM associated with lung
carcinoma exists.
Mucin synthesis is modulated by different cytokines including
TGFβ, interleukins, TNF and interferon [10]. Since alveolar
macrophages of patients with lung cancer secrete significantly more
cytokines than patients with benign lung diseases [11], these
elevated levels might be responsible for a fibroblastic stimulation
[7]. Further on, several authors have described that mucin products
of MUC1 and MUC4 genes are highly expressed in lung carcinomas [12,
13]. Therefore, although the association between REM and lung
carcinoma might be coincidental because of the high incidence of
lung carcinoma in the Spanish smoker population, the theory of a
possible common origin of both processes must be considered.
Thus, although the presence of essential thrombocytosis in a
patient with REM may be casual, we want to emphasize this situation
because of the existence of previous reports of cutaneous mucinosis
associated with diverse hematological diseases [14, 15].
The presence of vascular dilation in the upper and middle dermis
has been described between the possible histological findings in
REM [16]. However, the presentation of telangiectasias at a
clinical level is a rare finding, and we have only found one report
of clinical telangiectasias in lesions of REM similar to our case
[17].
We emphasize this case because it is an infrequent form of
reticular erythematous mucinosis, for the presence of cutaneous
lesions with telangiectasias, and the unusual association with
essential thrombocytosis and lung carcinoma.
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