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Br J Dermatol. Cutaneous granulomas associated with primary immunodeficiency disorders. We report cutaneous granulomas in a child with ataxia telangiectasia AT and compare the clinical course with similar lesions in an adult with common variable immunodeficiency CVI. A 4-year-old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids. Disease control was obtained initially with oral antibiotics and low-dose oral steroids.

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Br J Dermatol. Cutaneous granulomas associated with primary immunodeficiency disorders. We report cutaneous granulomas in a child with ataxia telangiectasia AT and compare the clinical course with similar lesions in an adult with common variable immunodeficiency CVI. A 4-year-old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids.

Disease control was obtained initially with oral antibiotics and low-dose oral steroids. On cessation of oral steroids, significant relapse of the facial granuloma occurred. Pulsed and then oral steroids were required to stop the disease process leaving significant scarring. The second case is of cutaneous granulomas in a year-old man, with CVI, who presented with an erythematous reticulate rash on the legs.

We consider it useful to report this patient here as disease control was obtained in a similar way with systemic immunosuppression. In this patient a combination of oral steroids and azathioprine was used. These cutaneous granulomas are thought to be a manifestation of immune dysregulation.

No infectious cause has been found so far. We recommend the use of broad-spectrum antibiotics in conjunction with systemic steroids for progressive granulomas, as these patients are immunosuppressed and infection with an unidentified organism cannot be excluded.

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Cutaneous granulomas associated with primary immunodeficiency disorders.

Anche se le lesioni possono essere sgradevoli, il granuloma anulare di solito non provoca altri sintomi e non ha impatto a lungo termine sulla salute. Link sponsorizzati Nella maggior parte dei casi le lesioni scompaiono da soli entro due anni. Queste protuberanze possono assomigliare alla tigna. Lieve prurito in alcune persone, anche se le lesioni di solito non causano dolore o prurito. Anche se le lesioni possono essere sgradevoli, il granuloma anulare di solito non provoca altri segni o sintomi.

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Granuloma anulare : cause, aspetto, diagnosi e terapie

Sarcoidosis and cancer may mimic one another, making the distinction difficult. Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory process involves the alveoli, small bronchi, and small blood vessels. Caution is recommended, as it only shows a general relation with physiological markers of the disease and the variation is such that it has limited applicability in individual assessments, including treatment decisions. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems. Pulmonary arterial hypertension occurs by two mechanisms in cardiac sarcoidosis: reduced left heart function due to granulomas weakening the heart muscle or from impaired blood flow. Development of scleral nodule associated with sarcoidosis has been observed. It also frequently causes an increase in 1,dihydroxy vitamin D, the active metabolite of vitamin D , which is usually hydroxylated within the kidney, but in sarcoidosis patients, hydroxylation of vitamin D can occur outside the kidneys, namely inside the immune cells found in the granulomas the condition produces.

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