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Concept: Lupus pernio

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BACKGROUND: Sarcoidosis is a systemic disease characterized by the formation of noncaseating granulomas in various tissues. Cutaneous involvement occurs in 20 to 35 percent of the patients and may be the initial manifestation of the disease. Our study was performed to discriminate the clinical, laboratory, and prognostic differences between patients with specific and nonspecific cutaneous involvement. The second aim was to asses the diagnostic usefulness of punch biopsy in sarcoidosis. METHODS: The clinical, laboratory, pathological features, and skin biopsy results of 120 patients with cutaneous sarcoidosis were evaluated. The patients fulfilled clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseating granulomas.Skin involvement was the initial finding in 30% of the patients. Erythema nodosum and lupus pernio were the most common skin lesions. Almost all of the patients with LP were either stage 0 or 1. Respiratory symptoms occurred in 72.2% of the patients with specific skin involvement. BronchoalveolarLavage (BAL) lymphocytosis, high ratio of CD4/CD8 and elevated serum Angiotensin Converting Enzyme (ACE) were more frequent in patients with specific cutaneous lesions. The frequency of progressive disease was significantly higher in this group. Punch skin biopsy was diagnostic in 81.6% of the patients with a complication rate of 4%. CONCLUSIONS: Specific cutaneous lesions along with BAL lymphocytosis, high CD4/CD8 ratio and elevated serum ACE levels may be predictors of progressive disease in sarcoidosis. Punch biopsy is a simple technique with a high diagnostic yield and a low complication rate for cutaneous sarcoidosis.

Concepts: Medical terms, Biopsy, Pathology, Sarcoidosis, Granuloma, Skin biopsy, Erythema nodosum, Lupus pernio

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The aim of this manuscript is to describe radiological findings of extra-pulmonary sarcoidosis. Sarcoidosis is an immune-mediated systemic disease of unknown origin, characterized by non-caseating epitheliod granulomas. Ninety percent of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Typical imaging features of liver and spleen sarcoidosis include visceromegaly, with multiple nodules hypodense on CT images and hypointense on T2-weighted MRI acquisitions. Main clinical and radiological manifestations of renal sarcoidosis are nephrolithiasis, nephrocalcinosis, and acute interstitial nephritis. Brain sarcoidosis shows multiple or solitary parenchymal nodules on MRI that enhance with a ring-like appearance after gadolinium. In spinal cord localization, MRI demonstrates enlargement and hyperintensity of spinal cord, with hypointense lesions on T2-weighted images. Skeletal involvement is mostly located in small bone, showing many lytic lesions; less frequently, bone lesions have a sclerotic appearance. Ocular involvement includes uveitis, conjunctivitis, optical nerve disease, chorioretinis. Erythema nodosum and lupus pernio represent the most common cutaneous manifestations encountered. Sarcoidosis in various organs can be very insidious for radiologists, showing different imaging features, often non-specific. Awareness of these imaging features helps radiologists to obtain the correct diagnosis.

Concepts: Kidney, Organ, Magnetic resonance imaging, Lymphatic system, Radiology, Sarcoidosis, Erythema nodosum, Lupus pernio

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A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported.

Concepts: Liver, Organ, Skin, Finger, Ring finger, Hand, Sarcoidosis, Lupus pernio

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Background: The clinical outcome of sarcoidosis is variable. Several features have been proposed as predictive of chronic sarcoidosis. Objectives: To examine the predictive role of clinical features on the clinical outcome score (COS) of sarcoidosis patients five years after initial diagnosis. Methods: A cohort of newly diagnosed sarcoidosis patients seen at one clinic were re-evaluated five years after initial diagnosis. The COS was determined at this five year time point and compared to initial age, race, sex, forced vital capacity (FVC) and chest roentgenogram. We also compared COS to whether patients had Lofgrens syndrome, lupus pernio, or cardiac disease and what treatment they received during the five years of observation. Patients with neurologic disease were divided into those with seventh cranial nerve paralysis alone versus those with other neurologic disease (CNS). Results: Of the 335 newly diagnosed patients seen over a three year period, 213 (64%) were evaluated five years after initial diagnosis. Of these patients 168 (79%) were still requiring systemic therapy at five years (COS 7, 8, or 9). Seven features were associated with chronic disease: CNS alone (Odds Ratio (OR)=19.24, p<0.05); CNS, cardiac, and/or lupus pernio (OR=5.86, p<0.02); FVC<80% (OR=7.04, p<0.02); treatment with prednisone (OR=6.35, p<0.0001); methotrexate (OR=7.2, p<0.0001); azathioprine (OR=19.24, p<0.05); anti-tumor necrosis therapy (OR=13.98, p>0.05). Conclusion: In our study, the majority of sarcoidosis patients were receiving systemic therapy five years after initial diagnosis. Patients with reduced lung function, neurologic disease, lupus pernio, and cardiac disease were more likely to require prolonged treatment.

Concepts: Osteoporosis, Medicine, Medical terms, Heart, Lupus erythematosus, Spirometry, Sarcoidosis, Lupus pernio

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An 80 year old female patient was seen in the Otolaryngology department with a skin manifestation of her sarcoidosis, known as lupus pernio. Around 25% of patients with sarcoidosis have skin disease and the presence of lupus pernio may suggest a more aggressive form of the disease. Photography of this relatively rare presentation not only helped us diagnose her underlying medical condition but also served as a baseline during treatment.

Concepts: Medicine, Disease, Infectious disease, Death, Medical terms, Physician, Sarcoidosis, Lupus pernio

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Sarcoidosis is an antigen-mediated disease of unknown aetiology, characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous involvement in sarcoidosis is classified as specific, when biopsy reveals non-caseating granulomas, and non-specific, typically erythema nodosum. Granulomatous skin lesions occur in 9% to 37% of patients. The skin is the second most commonly involved organ after the lung. A skin biopsy is easy to perform and enables an early diagnosis with a minor invasive procedure. Some types of specific lesions have prognostic significance and may help to predict the outcome of the systemic disease. Maculopapules, subcutaneous nodules and scar sarcoidosis are usually transient or tend to follow the course of the systemic disease. Skin plaques and lupus pernio are associated with chronic sarcoidosis. Although most cutaneous lesions of sarcoidosis do not cause significant morbidity and do not require treatment, some have cosmetic importance because they may be disfiguring and can have a strong psychosocial impact. Treatment of these lesions is a challenge since they do not respond well to conventional treatments. This manuscript reviews the clinical characteristics of the more frequent types of specific cutaneous lesions of sarcoidosis, the relationship between cutaneous involvement and systemic disease, the prognostic significance of lesions and the present state of treatment of difficult cases of cutaneous sarcoidosis.

Concepts: Lung cancer, Organ, Tuberculosis, Skin, Sarcoidosis, Granuloma, Erythema nodosum, Lupus pernio