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February 28-March 01, 2019 | Paris, France
Palliative Care, Obstetrics and Gynecology
Stroke and Clinical Trials
International Conference on
Joint Event on
International Conference on
&
Journal of Research and Reports in Gynecology and Obstetrics | Volume: 3
Chronic itching unmasking extra mammary Paget disease in a celibate patient
H Y Fanomezantsoa
1
, C Sophoclis
1
, G Barletta
1
, A M Kebaili
1
, A Sakho
1
, N R Hasiniatsy
2
, H Rabarikoto
2
and
O Dahmani
1
1
Louis Jaillon General Hospital, France
2
Centre Hospitalier de Soavinandriana, Madagascar
Background:
Paget’s disease, described by Sir James Paget in
1874 is classified as mammary and extra mammary. This later
has been described initially by Crocker in 1889 and confirmed
by Dubreuil in 1901, characterized by the presence of intra
epithelial mucin producing neoplastic cells or apocrine gland
bearing skin cells especially those located in the perineum
of both sexes, axilla, groin, thigh, eyelid, external ear and
umbilicus. It accounts for 10 %of Paget disease. It occurs mostly
in postmenopausal Caucasian women without excluding men
whom are mostly touched by the disease in Asian countries.
It could be primary or secondary to intestinal and urogenital
malignancy. Extramammary Paget ‘s disease is oftenmultifocal,
and inmany cases, it has been demonstrated extending beyond
the visible lesion. Diagnosis is based on having high clinical
index of suspicion, confirmed by the presence of Paget cell
on histopathology study and immunohistochemistry staining.
Herein, we report a case of extra mammary Paget disease
associated with urogenital malignancy discovered at the
invasive stage in a celibate patient.
Case Report:
This 86-year-old celibate lady was referred to
tertiary care with a chronic complain of pruritus. She was
known to have chronic arterial hypertension, chronic coronary
artery disease treated by percutaneous angioplasty and
recurrent pulmonary embolism without evidence of deep vein
thrombosis. Shedenied any history of tobacco smoking, alcohol,
or using illicit drugs. The sole treatment over the counter she
used to take is painkiller. She reportedahistoryof erythematous
lesion appearing on the external part of her labia majors
evolving into scaring and super imposed eczematous scale. She
had previously been treated empirically with oral and topical
antibiotics, topical antifungal agents and topical glucocorticoids
without improvement. She was celibate and has never had any
sexual activity. She was afebrile and stable hemodynamically.
The rest of physical examination was not contributory. Patient
has had a full radiological investigation including upper and
lower gastro intestinal endoscopy that was without abnormal
finding apart of sigmoid diverticulosis. Chest x-ray was
showing mild cardiomegaly with free costo-phrenic angles and
ultrasound of the breast was normal. However, ultrasound
of the abdomen and pelvis showed a polypoid bladder mass
with variable echogenicity and thickened wall of the bladder
and three hypo echogenic liver masses evoking metastasis.
Kidney sizes were appropriate to the age of the patient without
stone formation or dilation. There was no post voiding residual
volume on ultrasonography. Patient was referred to urology
clinic for evaluation and TDM and MRI abdomen and pelvis
confirmed the previous findings with extension to lymph nodes
in the retroperitoneal space and groins. Urinary cytology didn’t
show any neoplastic cell; however, cystoscopy confirmed a
neoplastic nature of the tumor. Laboratory investigation was
marked by grade I inflammatory anemia with high erythrocyte
sedimentation rate at 114, CRP at 55.4mg/L (N<5) and increased
ferritin level at 1413 ug/l (N<150). There was abnormal liver
function tests in favor of cholestatic hepatitis (GGT =579 U/L
and alkaline phosphatase at 442 U/L) and impaired kidney
function test characterized by a creatinine clearance at 41 ml/
min /1.73m2 with tubular proteinuria at 740 mg/24hours. TSH
mildly elevated and lactic dehydrogenase = 695 U/l. Plasma
protein electrophorese showed monoclonal gammopathy of
undetermined significance and tumor markers were positive for
CEA = 683(N<5), CA19.9 at 129, 5 (N <39). The rest of laboratory
datawas non-significant. Cystoscopywith biopsy of both lesions
showed picture of Paget cells and immunohistochemistry
stained for CK7, CEA, GATA -3 and negative for CK20, PS100
and MelA. Histology of bladder confirmed the presence of
infiltrate carcinomatous proliferation with focus of necrosis
and embolism of lymph node. The retained diagnosis was
infiltrating urothelial carcinoma of the bladder with high-grade
invasion (pT2 of UICC).
Discussion:
Mammary and extra mammary Paget’s disease
(EMPD) is uncommon intra epithelial adenocarcinomas.
Both conditions have similar clinical features and they are
characterized by the presence of large oval or polyhedral
intra epithelial cells that have pale cytoplasm and large
nuclei with prominent nucleoli. They can be visualized
using hematoxylin and eosin staining. The most common
presenting symptom in extra mammary Paget’s disease is
pruritus. In many circumstances it can be asymptomatic slowly
progressive, presenting as plaque, patch or just a red lesion or
complicated appearance justifying ruling out eczema, chronic
local infection, inflammatory conditions and tumors. Lesions
occasionally showed hyperpigmentation or hypopigmentation.
Unfortunately, our patient was having longstanding pruritus
and failed many therapeutic attempts associated with chronic
H Y Fanomezantsoa et al.
, Res Rep Gynaecol Obstet, Volume 3
DOI: 10.4066/2591-7366-C1-003