Conjunctival deposits as the first sign of systemic sarcoidosis in a pediatric patient

PURPOSE: Sarcoidosis is an inflammatory disease of unknown cause, characterized by noncaseating granulomas. In this study, the authors present a sarcoidosis patient without systemic signs diagnosed by histopathologic analysis of conjunctival deposits. METHODS: A 10-year-old girl had bilateral, focal, multilobular, multiple pale yellow deposits in bulbar conjunctiva for a year. In the patient's biomicroscopic examination, numerous 0.50- to 2-mm diameter pale yellow deposits were determined in both bulbar conjunctivas. The patient had no other systemic or ocular complaints. A conjunctival biopsy was performed. RESULTS: The biopsy specimens showed noncaseating granulomas with prominent asteroid bodies. Serum angiotensin-converting enzyme levels were increased in the patient. Conjunctival deposits were the first manifestation of sarcoidosis in the patient and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. CONCLUSIONS: To the authors' knowledge, this is the second study that reports a sarcoidosis patient with non-systemic involvement diagnosed after histopathologic analysis of conjunctival deposits.     

Value of conjunctival biopsy in the diagnosis of sarcoidosis

PURPOSE: Retrospective study concerning the value of conjunctival biopsy in the diagnosis of sarcoidosis. PATIENTS AND METHODS: Between 1990 and 1996 we performed conjunctival biopsy in 11 patients (mean age 42.7 +/- 16.4 years) with suspect of sarcoidosis. RESULTS: In 8 of the 11 patients the diagnosis of sarcoidosis was established during the clinical course. In four of these eight patients conjunctival biopsy was positive. Five of the eight were under systemic steroids at the time of biopsy. Of the four patients with clinically established sarcoidosis and negative biopsy, three were under systemic steroids at the time of biopsy. In two patients diagnosis of sarcoidosis was established primarily by conjunctival biopsy. CONCLUSION: Conjunctival biopsy is a simple tool in the diagnostic of sarcoidosis. If possible, biopsy should be undertaken before systemic steroid treatment. We consider conjunctival biopsy to be useful as the first diagnostic tool before other invasive methods.     

Bilateral large conjunctival tumours as primary manifestation of sarcoidosis--successful treatment with steroid-depot-injections

INTRODUCTION: Ocular manifestations of sarcoidosis vary enormously. They include the conjunctiva, lacrimal gland, orbita, intraocular structures and eye-lid, either isolated or combined. We describe a female patient who presented with unusually large, bilateral conjunctival tumours as a primary manifestation of sarcoidosis. PATIENT: A 79-year-old white woman was referred to us for further management of a persisting "conjunctivitis", which had been refractory to treatment with multiple medications. Initial examination disclosed swollen eye-lids and bilateral large hard tumours of the inferior fornix. The obtained brush smear, which was cytopathologically evaluated, revealed epitheloid cells and multinucleate giant cells. After 4 weeks she developed three reddish-brown maculopapular lesions on her face. The subsequent biopsy from the left inferior fornix and the skin showed histopathologically a granulomatous epitheloid cell inflammation without central necrosis and without acid-proof bacilli. Therefore a sarcoidosis was included into the differential diagnosis. The systemic evaluation revealed no other manifestation. At first we tried to reduce the chronically inflammatory tumours with different immunomodulating local treatment forms. Only the repeated intralesional injection of a steroid depot showed a complete disappearance of all conjunctival and skin tumours. CONCLUSION: An isolated bilateral primary manifestation of sarcoidosis with large massive conjunctival tumours is very rare and clinically not typical. The non-invasive, cytopathological examination by means of brush smears offers a new perspective in the fast diagnosis of conjunctival manifestation of sarcoidosis. The tumours respond excellently to the intralesional injection of steroid depots.     

Conjunctival biopsy in the diagnosis of sarcoidosis.

We prospectively studied 47 sarcoidosis suspects and compared conjunctival and transbronchial lung biopsies in these patients. Thirty-four patients had positive findings on biopsy by either method. The transbronchial biopsy was positive in 31 patients, and the conjunctival was positive in 19. The transbronchial lung biopsy was more likely to be positive in black patients (p = 0.009) and in patients with pulmonary infiltrates on chest x ray (p = 0.0044). In comparison, the conjunctival biopsy was more likely to be positive in patients with conjunctival follicles (p = 0.036), ocular abnormalities consistent with sarcoidosis (p = 0.02), and in patients with pulmonary infiltrates on chest x ray (p = 0.029). Iritis was present in 12 patients, enlarged lacrimal glands in three, and vitritis in five. We conclude that the conjunctival biopsy is an effective means of diagnosing sarcoidosis and that every sarcoidosis patient should have an ophthalmic examination.     

Conjunctival biopsy in the diagnosis of ocular sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes. A definitive diagnosis of sarcoidosis requires that a biopsy be performed. A specimen can be obtained from any affected ocular structure, including conjunctiva, lacrimal gland, eyelid skin, and orbit. Among them, conjunctival biopsy has been suggested as a sensible and safe procedure for confirming suspected sarcoidosis. We describe three patients in whom ocular symptoms were the sole initial manifestations of sarcoidosis and who were diagnosed based on the results of a conjunctival biopsy. We also discuss the efficacy of and indications for conjunctival biopsy.     

Scleritis associated with sarcoidosis

PURPOSE: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. METHODS: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. RESULTS: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. CONCLUSIONS: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.     

Optic nerve enlargement and chronic visual loss

We present four patients with sarcoidosis of the anterior visual pathways. The first patient presented with unilateral visual loss, a mass lesion at the optic nerve head, and an enlarged orbital optic nerve. The second patient presented with bilateral progressive painless visual loss, associated with optic nerve pallor and visual field loss. In these two patients, optic nerve biopsy was diagnostic of sarcoidosis. The third patient developed optic nerve and chiasmal involvement after sarcoidosis was established by lacrimal gland biopsy. The fourth pateint had optic nerve, pulmonary, and lymph node involvement with sarcoidosis. A conjunctival and lung biopsy were diagnostic. Computed tomography and magnetic resonance imaging have greatly facilitated diagnosis of sarcoidosis of the anterior visual pathways. Sarcoidosis of the anterior visual pathways may occur alone or in association with other ocular or systemic manifestations. A conjunctival or lacrimal gland biopsy may be preferable as the initial diagnostic approach. Treatment of patients with this condition may require systemic immunosuppression, in addition to corticosteroids, to prevent permanent visual loss.     

Scleritis associated with sarcoidosis

Purpose: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. Methods: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. Results: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. Conclusions: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.     

Conjunctival Biopsy as an Aid in the Evaluation of the Patient with Suspected Sarcoidosis

Conjunctival biopsy is an underused but simple technique in the evaluation of the patient with sarcoidosis and occasionally other systemic diseases. In 55% of patients with biopsy-proven sarcoidosis from other sites, a blind conjunctival biopsy was positive. Bilateral conjunctival biopsies and the examination of multiple sections of each biopsy were essential to obtain this high of a yield. There was no relationship between an anterior uveitis and a positive conjunctival biopsy.     

Lacrimal gland enlargement in familial sarcoidosis

Familial sarcoidosis is rare. It is unusual for lacrimal gland enlargement to be the initial manifestation of sarcoidosis. Two sisters are described with lacrimal gland enlargement as the initial manifestation of sarcoidosis. Gallium-67 scan was a useful adjunct to lacrimal biopsy in sarcoidosis. Sarcoid dacryoadenitis responded dramatically to a short term course of low dose prednisone.     

Conjunctival biopsy of anophthalmic socket in sarcoidosis

A case of sarcoidosis was diagnosed by a random conjunctival biopsy from an anophthalmic socket. A 22-year-old black female patient presented with anterior and posterior uveitis and subconjunctival nodules in the left eye. Her right eye was enucleated due to a penetrating injury 3 years before admission. Biopsy of subconjunctival nodules in the left eye failed to demonstrate granulomatous inflammation; however, a random conjunctival biopsy from the normal-appearing anophthalmic socket conjunctiva revealed noncaseating granulomas. Conjunctival biopsy is a safe, cost-effective means of diagnosis in sarcoidosis suspects. To our knowledge, this is the first reported case of sarcoidosis confirmed with random conjunctival biopsy from an anophthalmic socket.     

Lacrimal bypass surgery in patients with sarcoidosis

PURPOSE: To examine the results of lacrimal bypass surgery in patients with sarcoidosis. METHODS: Patients with sarcoidosis who underwent dacryocystorhinostomy (DCR) or conjunctivodacryocystorhinostomy (CDCR) in two practice settings from 1986 through 1995 were identified and their medical records reviewed. RESULTS: Twelve patients, of whom eight were women, underwent bilateral DCR or CDCR to treat nasolacrimal duct obstruction associated with sarcoidosis. The initial diagnosis of sarcoidosis was established in four patients from a biopsy specimen obtained during DCR. The ages of the patients at diagnosis of sarcoidosis ranged from 39 to 64 years (mean, 49.6 years; median, 45.5 years), whereas their ages at the time of surgery ranged from 42 to 72 years (mean and median, 55 years). The average duration of postoperative follow-up evaluation was 44 months (median, 38.5 months; range, 10 to 82 months). All patients received local corticosteroids postoperatively, and nine patients (75%) were treated with prednisone. Of the 24 lacrimal procedures, 23 (95.8%) were patent to irrigation at the last follow-up examination, and all patients were asymptomatic. CONCLUSION: Lacrimal drainage obstruction may be the initial manifestation of sarcoidosis, and tissue obtained during DCR may help to establish the diagnosis. A successful surgical outcome may require intensive and occasionally long-term therapy with local and systemic corticosteroids.     

Ophthalmologic peculiarities of sarcoidosis

The present paper deals with the frequency of different ophthalmic sarcoid changes and their morphological and photographic characteristics. In addition, the diagnostic value of conjunctival biopsy is evaluated. The findings were based on repeated ophthalmological examinations of an unselected group of 281 patients with histologically confirmed sarcoidosis. Conjunctival (37/218) and lacrimal gland (33/254) changes were encountered more often than sarcoid uveitis (22/281), and a conjunctival granuloma was the most frequent single sarcoid ophthalmic finding. Sarcoid changes in the eyes and the adnexae were often characterized by a scarcity of symptoms and a typical outward appearance. In some cases of sarcoid uveitis fluorescein angiography revealed in the iris and the retina nodules which showed features suggesting the proliferative nature of the disease. The iris infrared transillumination technique illustrated the affinity of sarcoid nodules in the pupillary area. Conjunctival biopsy showed epithelioid cell granulomas compatible with sarcoidosis in nearly half of the patients in whom it was suspected in a slit-lamp examination of the conjunctivae. In the diagnosis of sarcoidosis, conjunctival biopsy is a procedure to be recommended before more demanding methods are tried.     

Retinal cotton-wool spots as the first sign of systemic sarcoidosis.

Isolated retinal cotton-spots have not yet been reported as the sign of ocular sarcoidosis. We describe three patients with retinal cotton-wool spots of initially unknown etiology in whom systemic sarcoidosis was diagnosed 6-16 months later. The diagnosis was confirmed by conjunctival biopsy. These observations indicate that sarcoidosis should be included in the differential diagnosis of retinal cotton-wool spots and patients with these lesions should be followed for the development of this disease.     

Retinal pigment epithelial detachment in sarcoidosis

PURPOSE: To report primary retinal pigment epithelial (RPE) detachments in sarcoidosis. DESIGN: Case report. METHODS: Clinical findings, fluorescence angiography and optical coherence tomography (OCT) results are presented and discussed. RESULTS: A 54-year-old Hispanic male with biopsy proved sarcoidosis presented with multiple RPE detachment in both eyes. Except for acute iritis, there were no other ocular manifestations of sarcoidosis. CONCLUSIONS: Detachments of the RPE may be a rare manifestation of ocular sarcoidosis.     

The incidence and pathology of conjunctival ulceration in Behçet's syndrome

AIMS: To describe the results of a study of the incidence of conjunctival ulceration and its pathology as a manifestation of Beh?et's syndrome. METHODS: The authors reviewed retrospectively medical charts of 152 patients with Beh?et's syndrome seen at the uveitis service of Okayama University Hospital from January 1995 to December 1999. Criteria used to diagnose Beh?et's syndrome in this study required the presence of oral aphthous ulceration plus any two of three other manifestations-genital ulceration, eye lesions such as iridocyclitis and uveoretinitis, or skin lesions such as erythema nodosum. The presence of conjunctival ulceration was determined in patients with Beh?et's syndrome through standardised ophthalmological examinations. A biopsy of the conjunctival ulceration was performed in one patient. The incidence of conjunctival ulceration in patients with Beh?et's syndrome was calculated and the clinical and pathological features of this ulceration were examined. RESULTS: Four of 152 patients (2.6%) developed conjunctival ulceration in concordance with exacerbation of their systemic symptoms. Histopathological examinations of a conjunctival biopsy specimen in one patient revealed intraepithelial and perivascular infiltration with neutrophils and lymphocytes, consistent with Beh?et's syndrome. The conjunctival ulcerations of the study patients showed good response to topical corticosteroids or topical steroids plus oral colchicine. CONCLUSIONS: Conjunctival ulceration should be noted as an uncommon but possible manifestation of Beh?et's syndrome. Accordingly, routine examination of the conjunctiva is recommended in patients with Beh?et's syndrome, and Beh?et's syndrome should be included in a list of differential diagnoses for patients with conjunctival ulcerations.     

Scleral Nodule and Bilateral Disc Edema as a Presenting Manifestation of Systemic Sarcoidosis

Aim: To report a rare presentation of a large scleral nodule with bilateral disc edema as the initial manifestation of sarcoidosis.

Materials and Methods: Retrospective interventional case report.

Results: A 58-year-old woman was referred for evaluation of nodular scleritis. Slit-lamp examination showed a large scleral nodule superiorly in the right eye. Fundus showed bilateral disc edema. High-resolution computed tomography of the thorax showed mediastinal and bilateral hilar lymphadenopathy. Transbronchial lymph node biopsy showed epitheloid granulomas. The scleral nodule and disc edema resolved at 6 weeks with oral steroids.

Conclusions: This case highlights a rare presentation of a large scleral nodule with bilateral disc edema as the initial manifestation of systemic sarcoidosis.     

Conjunctival non-caseating granulomas in a human immunodeficiency virus (HIV) positive patient attributed to sarcoidosis

PURPOSE: To report a rare case of isolated ocular sarcoidosis in an HIV positive patient. DESIGN: Case report. METHODS: A 47-year-old HIV+ Caucasian male was referred for chronic bilateral follicular conjunctivitis. RESULTS: A conjunctival biopsy that was performed on the right eye showed sarcoidosis. General medical evaluation including a spiral thin cut chest CT scan revealed no systemic involvement. The ocular signs and symptoms resolved completely with topical corticosteroid treatment. CONCLUSIONS: HIV infection and sarcoidosis rarely coexist, presumably because their immunopathogenesis mechanisms diverge. In the absence of systemic involvement, a definite diagnosis can only be made by biopsy of the ocular tissues.     

Corneal ulcer as an atypical presentation of ocular cicatricial pemphigoid

PURPOSE: To describe three patients, each presenting noninfective corneal epithelial damage as first manifestation of ocular cicatricial pemphigoid (OCP). METHODS: Case report. RESULTS: Patients 1 and 2 were referred to the authors' clinic for corneal ulcer while Patient 3 for relapsed epithelial defects. All patients had negative history for systemic diseases and microbiological tests were negative. Topical steroid treatment induced the complete resolution of corneal damage. During the follow-up period, the onset of mild conjunctival fibrosis in the lower fornix allowed the authors to suspect OCP, confirmed by conjunctival biopsy. CONCLUSIONS: In the three patients corneal damage was an early sign of OCP, in the absence of typical signs of conjunctival fibrosis. The authors thus suggest considering conjunctival biopsy as a useful additional test in the management of idiopathic corneal ulcers.