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Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series.

A 58-year-old male was admitted to our hospital because of periungual nailfold an erythema and erythematous rash on the dorsal joints of his hands and feet, but no muscle weakness. He was thus diagnosed to have amyopathic dermatomyositis. He had moderate hypoxemia and his chest computed tomography scans demonstrated bilateral ground-glass opacities, implicating complication with interstitial pneumonia. Therapy was initiated with pulsed methylprednisolone followed by high-dose corticosteroids, pulsed cyclophosphamide, and cyclosporine. The skin manifestations improved; however, the pulmonary infiltrates and hypoxemia deteriorated during the 2-month period of the treatment. The treatment was switched from cyclosporine to tacrolimus because of an inadequate clinical response to the therapy, and this resulted in the resolution of interstitial pneumonia. This case indicates that tacrolimus administration should be considered for patients with this life-threatening disorder when it is judged to be refractory to cyclosporine.

A 47-year-old female was diagnosed to have pulmonary, ocular, and nodular-type muscular sarcoidosis. Seven years later, nodules developed in all limb muscles. She received minocycline 200 mg daily, which resulted in an obvious reduction of the muscular sarcoidosis with a significant decrease in the serum angiotensin-converting enzyme level. Nine months later, the minocycline was discontinued, thus resulting in a rapid recurrence of the disease. The immediate readministration of minocycline again resulted in a prompt improvement. We detected Propionibacterium acnes within the granulomas in the affected muscle by an immunohistochemistry. More interestingly, we found a decrease in the circulating levels of interleukin-12 p40 and interferon-inducible protein-10 during the minocycline therapy. The minocycline therapy may be effective for sarcoidosis and the fact that the disease rapidly relapsed after discontinuation of the minocycline administration suggests that the mechanism of action in this case may be immunomodulating but not antimicrobial effect.

Amyopathic dermatomyositis (ADM) is occasionally complicated by rapidly progressive interstitial pneumonia (RPIP), and in such cases, diffuse alveolar damage (DAD) is usually diagnosed at autopsy. Here, we present three patients with RPIP accompanied by ADM in whom lung disease was assessed at an early stage. High-resolution computed tomography (HRCT) carried out before the onset of dyspnoea revealed uniformly subpleural reticular opacity with faint ground-glass attenuation. At that stage, surgical lung biopsies from two patients showed histological patterns typical of cellular nonspecific interstitial pneumonia (NSIP). Despite pulse methylprednisolone and subsequent high-dose oral administration of prednisolone, lung disease progressed in all patients, with extensive areas of ground-glass opacity and consolidation observed in HRCT scans. DAD was confirmed histologically in one case. Additional administration of cyclosporine, pulse cyclophosphamide or high-dose intravenous administration of immunoglobulin rescued all patients. Our data suggest that ADM-associated interstitial pneumonia takes an aggressive course even when the radiological and histological features are consistent with NSIP. Aggressive combination therapy with high-dose steroids and immunosuppressive agents is required as early as possible for patients with this life-threatening disorder.