Melkersson-Rosenthal syndrome in the context of sarcoidosis: a case report.

BACKGROUND: Melkersson-Rosenthal syndrome is a rare disease characterized by the triad of recurrent orofacial swelling with facial paralysis and fissured dorsal tongue. Histologically, noncaseating granulomatous inflammation occurs that confirms the diagnosis. Overlaps between granulomatous diseases such as sarcoidosis and Crohn's disease are described. Systemic corticosteroid therapy is the treatment of choice for acute attacks. CASE PRESENTATION: We here present a case of a 59-year-old White woman suffering from Melkersson-Rosenthal syndrome with a past history of sarcoidosis on therapy with leflunomide in combination with low-dose tacrolimus successfully treated with the anti-leprosy drug clofazimine after failure of systemic steroid therapy. CONCLUSIONS: We propose clofazimine as an alternative treatment in steroid-refractory cases.

Cutaneous sarcoidosis: clinicopathologic study of 76 patients from Lebanon.

BACKGROUND: Sarcoidosis is a multi-system granulomatous disease of unknown etiology. The skin is involved in 25% of cases. Studies on cutaneous sarcoidosis from our region are lacking. OBJECTIVES: This study was conducted to describe clinical and histopathologic findings in all patients diagnosed with cutaneous sarcoidosis at the American University of Beirut Medical Center between 1992 and 2010 and to compare findings with those published in the literature. METHODS: Clinical charts of patients with cutaneous sarcoidosis were retrospectively reviewed. Extracutaneous lesions were classified by organ involvement. Treatment was documented. Pathology specimens were reviewed. RESULTS: Cutaneous sarcoidosis was diagnosed in 76 Lebanese patients, 79% of whom were women. Mean age at diagnosis was 48 years. A total of 29% of patients had systemic disease that was commonly associated with lupus pernio lesions and subcutaneous sarcoidosis. The most common cutaneous lesions were sarcoidal plaques. The histopathologic features in our series did not differ from those described in the literature, except for the documented presence of a grenz zone. Interestingly, 23% of biopsy specimens contained perineural granulomas, raising the possibility of tuberculoid or borderline tuberculoid leprosy. Foreign bodies were detected in 10% of cases (all had systemic involvement), supporting the opinion that sarcoidosis and granulomatous foreign body reaction are not mutually exclusive. CONCLUSIONS: The clinical and histopathologic features of cutaneous sarcoidosis patients in the present series are generally comparable with those published in the literature, with minor differences. Clinically, the most commonly seen lesion was plaque. Microscopically, cutaneous sarcoidosis may exhibit a grenz zone and may show perineural inflammation and foreign bodies.

Ichthyosiform sarcoidosis revisited.

Cutaneous manifestations of sarcoidosis are present in approximately one-third of the cases. Ichthyosiform lesion is one of the extremely rare cutaneous manifestations of sarcoidosis. It is a uncommon, but specific cutaneous manifestation of sarcoidosis that may precede or appear simultaneously with the diagnosis of systemic sarcoidosis. Approximately 20 cases of ichthyosiform sarcoidosis have been reported in the PubMed database. We report here a case of sarcoidosis with ichthyosiform skin lesions along with central nervous system (CNS) and pulmonary involvement for its rarity and interesting clinical presentation.

Hypercalcemia in two patients with sarcoidosis and Mycobacterium avium intracellulare not mediated by elevated vitamin D metabolites.

INTRODUCTION: To describe 2 unusual cases of hypercalcemia due to granulomatous diseases with normal vitamin D metabolites and no other ready explanation for the hypercalcemia. METHODS: We present the clinical, laboratory and pathologic findings of 2 patients with hypercalcemia and review previous reports of hypercalcemia in granulomatous diseases without elevated vitamin D metabolites. RESULTS: Hypercalcemia was described in various granulomatous diseases including sarcoidosis, tuberculosis, berylliosis, leprosy and, rarely, in fungal infections. Elevated serum level of vitamin D or its metabolites was linked to the pathogenesis of hypercalcemia in these disorders. The authors present the clinical, laboratory and pathologic findings in 2 patients who presented with hypercalcemia and normal vitamin D metabolites with no other ready explanation for the hypercalcemia. The first patient was diagnosed with Mycobacterium avium, whereas the second patient was found to have sarcoidosis. CONCLUSION: Although hypercalcemia in granulomatous diseases has been attributed to be mediated by elevated vitamin D metabolites, there have been several case reports that documented normal values of active vitamin D metabolites. This report illustrates the regulatory feedback mechanisms of vitamin D synthesis and introduces the term "inappropriately normal" vitamin D metabolites levels in light of low levels of parathyroid hormone.

A severe case of multisystem sarcoidosis complicated by aspergillosis and aspergillomas.

The authors present an atypical case of multisystem sarcoidosis presenting at a late stage with severe lupus pernio sarcoidosis skin lesions and stage IV pulmonary sarcoidosis complicated by semi-invasive chronic necrotising aspergillosis and aspergillomas. Lepromatous leprosy, tuberculosis and active atypical mycobacterial infection had to be ruled out en route to reaching the final diagnoses. His case presented us with a management dilemma, specifically concerning treatment of his sarcoidosis with corticosteroid and other immunosuppressive agents, as these risked aggravating his active invasive fungal disease. The patient's semi-invasive aspergillosis was treated first with antifungal agents for 6 months before treatment with corticosteroids and hydroxychloroquine was started. The patient has tolerated his treatments well, and over a 3-year follow-up period, has had a significant improvement in his respiratory and systemic symptoms, with some improvement in his lupus pernio sarcoidosis skin lesions.

Mortimer's Malady revisited: a case of polymorphic cutaneous and systemic sarcoidosis.

Sarcoidosis is a systemic disorder with prominent cutaneous component. Skin lesions are of diverse morphology, of which few are specific for the disease. We describe a 30-year-old woman with polymorphic skin lesions including papules, plaques, and nodules, as well as uncommon variants like eyelid papules, palmar and digital nodules, tattoo sarcoid, as well as scar sarcoid. The patient also had stage II pulmonary sarcoidosis, and articular as well as reticulo-endothelial system involvement manifested by enlarged mediastinal and abdominal lymph nodes and hepatosplenomegaly. The presentation of polymorphic skin lesions with involvement of multiple extra-cutaneous systems is uncommon in a single patient.

[Unilateral lachrymal gland enlargement revealing sarcoidosis in an 85-year-old man]. / Tuméfaction unilatérale de la glande lacrymale révélatrice de sarcoïdose à 85 ans.

We present a case of an 85-year-old Caribbean man initially presenting with lachrymal gland enlargement and a large subcutaneous extension responsible for a hemifacial "lion-like" deformation. The most important diagnoses to suggest are adenocarcinoma of the lachrymal gland, lymphoma, leprosy, tuberculosis, and sarcoidosis. Based on the clinical, biological, and radiological findings, sarcoidosis was suspected with lachrymal gland and pulmonary lesions. Biopsy of the enlarged lachrymal gland revealed a non caseating granuloma compatible with the diagnosis of sarcoidosis. The value of this case is the atypical field and sarcoidosis revealed as a unilateral lesion in an elderly male.

Cutaneous sarcoidosis: clinical profile of 23 Indian patients.

BACKGROUND: Sarcoidosis is a multisystem disease of undetermined etiology. Indian studies on cutaneous sarcoidosis are not many and mainly comprise case reports. AIMS: This retrospective study was carried out to assess the clinical profile of sarcoidosis patients presenting with cutaneous lesions. METHODS: All histopathologically proven cases of cutaneous sarcoidosis seen consecutively between 1999 and 2004 were studied. Their age, sex, presenting features, evolution of disease and laboratory parameters were analyzed. RESULTS: A total of 23 patients (F:M 15:8) between 31 to 78 years (mean 44.3 years) of age had the mean duration of skin lesions of 1.4 years. Six patients had one to four lesions; two patients each had scar sarcoidosis and angiolupoid and one patient each had recurrent erythema nodosum, leg lymphedema and subcutaneous sarcoidosis. Others showed combination of papules, nodules, plaques and psoriasiform lesions. Peripheral lymph nodes were involved in two patients. Among 10 patients of pulmonary involvement, three had become symptomatic four months to four years after the cutaneous lesions. Routine laboratory investigations including serum calcium estimation were normal in all cases. Serum angiotensin-converting enzyme levels were raised in 3 out of 6 patients. Asymptomatic lytic lesions of digital bones were detected in hand X-ray of one patient. CONCLUSION: Skin lesions of sarcoidosis are like the tip of an iceberg indicating more changes in other organs. The symptomatology and abnormal laboratory results do not necessarily correlate with the severity of cutaneous involvement in general.

Manifestaciones cutáneas de la sarcoidosis / No disponible

Aunque las manifestaciones cutáneas de la sarcoidosis, las agudas, pueden presentar remisiones, las crónicas pueden ser persistentes, y ser motivo importante de consulta. En nuestra serie de 110 pacientes, 25 presentaron afección cutánea (22,52%), de estos 20 tenían lesiones dérmicas específicas (18,2%) y 5 (4,5%) lesiones inespecíficas. Las lesiones específicas que predominaron fueron las erupciones maculopapulares y las placas y todas presentaron relaciones con estadios radiográficos de esta entidad, fundamentalmente en el estadio I y II. La presencia de afectación clínica fue más representativa en forma de erupciones maculopapulares y en placas, al igual que la evolución crónica. El estudio histopatológico, que según el órgano afectado ayudará al diagnóstico, y la piel por su accesibilidad y su especificidad con la demostración de granulomas sarcoideos, orienta a su confirmación y evita otros exámenes más agresivos y más costosos

Although acute cutaneous manifestations of sarcoidosis may present recurrences, those chronic may persist and be important causes of patient remission to health clinics and dermatologists. In our serie of 110 patients, 25 presented cutaneous affection (22,52 %), 20 out of them had specific skin lesions (18,2%) and 5 (4,5%) non specific lesions. The specific lesions that prevailed were maculopapular eruptions and plates, and all had relationships with radiographic studies of this entity, mainly in I and II stages. The persistence of affections were more representative in maculopapular eruptions and disease, biopsy with the histopathology study which depends of the affected organ, will help to the diagnosis; and the skin due to its accessibility and specificity with the demonstration in sarcoid granulomas guides to its confirmation and prevent more aggressive and expensive exams