clinical case presentation hana zoubeidi department of ... · clinical case presentation hana...
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Clinical Case Presentation
Hana Zoubeidi
Department of internal medicineRabta Hospital, Tunis, Tunisia
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Mrs BH, 47 years old, was addressed from the ophtalmology department for
etiological investigation of a left anterior nodular scleritis.
The history taking:
Sicca syndrome with ocular and oral dryness
Inflammatory polyarthralgia of the metacarpophalangeal and the
proximal interphalangeal joints
Masses regarding proximal and distal interphalangeal joints of the indexes
and the right big toe
Dry cough
Nasal obstruction
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Physical examination:
Afebrile
Urine Dipstick Analysis : No Hematuria, No Proteinuria
No palpated lymphadenopathy
Cardiopulmonary auscultation: Normal
Neurological examination: Normal
Dermatological Examination : No anomalies
Articular examination:
Painful active and passive mobilization of the proximal and the distal
interphalangeal joints of the two indexes and the two big toes.
Phalangeal nodules of the two indexes and the two big toes.
Ulcers and suppuration regarding the left index and the left big toe.
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Figure 1: Ulcers of the left bigtoe
Figure 2: Ulcers and nodules of the indexes
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Specialized ophthalmologic examination:
Left anterior nodular scleritis
Shirmer Test= Abnormal.
Lab Tests
Blood Count: Hb = 14.4 g / dl; lymphocytes: 870 elements / mm3
CRP = 10.7 mg / l ( <5 mg/L)
Creat = 8mg / L (9-13)
Serum electrophoresis: hypergammaglobulinaemia at a level of
18.32g /l (6-12 )
Blood and urinary calcium and phosphate: normal
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CHEST X RAY:
Bilateral interstitial
syndrome and
mediastinal widening
Chest computedtomography:
Granulomatous nodules inperilymphatic distribution
Mediastinal adenopathies
Parenchymal lungcongestion
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Hand and feet X-rays:
Lytic bone lesions of the proximal and the second phalanx
giving a lacy appearance
Soft tissue swelling
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OTORHINOLARYNGOLOGICAL EXAMINATION AND ENDOSCOPIES:
Rhinitis
No masses
No tumors
HISTOLOGICAL STUDY:
Nasal biopsy: Granuloma without necrosis neither vasculitis.
Labial salivary gland biopsy exhibits: granulomatous
sialadenitis.
Bone biopsy: Granuloma without necrosis neither neoplastic
cells.
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To sum up:
Ocular
involvement
ORL
involvement
Oral dryness and labial granuloma
Osteolysis
Pulmonary
involvement
Biology:
Eye dryness and nodular scleritis
Nasal obstruction, epistaxis and nasal granuloma
Cough, dyspnea, interstitial syndrome and
mediastinal adenopathies
Lymphopenia,
Polyclonalhypergammaglobulinemia
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Diagnosis ?
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Infectious diseases
Tuberculousand non
tuberculousMycobacteria
Brucellosis Syphilis MycosisToxoplasmosis:
Intradermal tuberculin test: Negative
Quantiferon test: Negative
Chest X Ray and computed tomography: no
signs of tuberculosis
Negative serology Negative serology
No risk factors and no
immunodepression favoring
this type of infection
Negativeserology
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Neoplasia
No impairment of the general condition
Normal blood count (no cytopenia, no
hyperleucocytosis)
Thoracoabdominopelviccomputed
tomography: No abnormalities, no masses.
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Inflammatory diseases
Eosinophilic granulomatosis with polyangiitis(Churg-Strauss):
No history of asthma
No hypereosinophilia
No cutaneous signs of vasculitis ( Purpura, livedo…)
Histology: No eosinophilic infiltration neithervasculitis
Immunological tests: Antineutrophil cytoplasmicantibodies were negative
Granulomatosis with polyangiitis (Wegener'sgranulomatosis)
No radiologic signs
Antineutrophil cytoplasmic antibodies were negative
HISTIOCYTOSIS:
histological and immunophenotypic examination of lesional bone tissue:
Negative staining of the lesional cells with CD1a.
SARCOIDOSIS
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Clinical arguments:
No exposure to active pulmonary tuberculosis
The combination of intrathoracic and extra-thoracic
evocative locations such as nodular scleritis.
Radiological arguments:
Mediastinal adenopathies and interstitial
syndrome.
Biological and immunologicalarguments:
Tuberculin skin test anergy
Lymphopenia
Histological arguments:
Tuberculoid granuloma without necrosis in the
bone tissue, the labial tissue and the nasal
tissue
ARGUMENTS FOR SARCOIDOSIS
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We retained the diagnosis of sarcoidosis
She was treated with corticosteroids: 1mg/kg/day and Methotrexate:
15mg/week.
Corticotherapy was preceded of antibiotherapy
The evolution was favorable with:
Decrease swellings
Purulent flow disappearance
Disappearance of inflammatory signs
Wound healing but persistent strain
It is currently nine months of decline.
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TAKE HOME MESSAGES:
Sarcoidosis bone involvement is rare and occurs in approximately 5% to 10%
of patients with sarcoidosis.
Phalanges of the hands and feet are the most common sites of involvement.
On radiographs, osseous sarcoid classically manifests as lacelike lytic bone
lesions in the phalanges of the hands or feet.
Sarcoid bone lesions are often multiple and lesions may have imaging features
that may be identical to osseous metastatic disease and biopsy may be
required for definitive differentiation.
The treatment of bone sarcoidosis is not coded and based on the
analgesics,steroidal and non-steroidal antiinflammatory and immunosuppressants if
necessary.
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