After 3 months, we observed only a moderate improvement of the cutaneous lesions. a linear set up. Erythema and edema, infiltrated plaques, diffuse sclerodermoid induration, acrosclerosis, leonine facies and alopecia may also happen [2, 3]. Deposition of mucin in nodular lesions is very rare and has been only sporadically reported [4, 5, 6]. Involvement of the periorbital region with mucinous nodules has been reported even more hardly ever [5]. In addition to cutaneous manifestations, extracutaneous involvement is present in the majority of patients, and is associated Bisoprolol fumarate with severe morbidity and improved mortality [2, 3, 7]. A variety of different restorative approaches for dealing with this chronic and progressive mucin deposition disorder have been used. Compared to older medicines, intravenous immunoglobulin (IVIg) has shown excellent results with a good security profile [8]. There have been only sporadic reports of treatment experiences for nodular mucinous pores and skin involvement. Case Statement A 42-year-old male had been well until December 2008, when he mentioned progressive swelling of the face, hands and forearms, accompanied by pruritus, increased hair loss, arthralgia, myalgia and fatigue. In the following weeks, pores and skin induration of the hands and forearms, papular eruption within the neck and top trunk, and nodular lesions on the face and fingers developed. The patient was admitted to the local hospital, where systemic fibrosing disorder was suspected and systemic glucocorticoid treatment (methylprednisolone 1 mg/kg body weight) was launched. Since only partial regression of the facial and hand edemas Bisoprolol fumarate Bisoprolol fumarate was observed, he was referred to the University Medical Centre Ljubljana, initially to the Department of Rheumatology and later to the Department of Dermatovenereology. At admission in February 2009, dense papular lesions were present around the neck, retroauricular areas, scalp and upper trunk, partly in a linear arrangement. Sclerodactyly with impaired finger motility was present. Tender nodules of a hard consistency could be palpated bilaterally around the upper and lower eyelids, around the forehead, lateral parts of the nose, behind the ears and on the fingers (fig. ?(fig.1).1). Sclerodermoid induration of the skin with irregular loss of hair was present around the forearms. The patient still complained of general symptoms and dysphagia. Open in a separate window Fig. 1 Scleromyxedema: nodular lesions on the face (a) and papular mucinosis around the neck (b). Complete blood cell count, serum levels of inflammatory parameters, electrolytes, glucose, calcium, creatinine kinase, myoglobin, aldolase as well as tumor markers were within normal range. Renal function assessments and urinalysis were normal. Testing for antitopoisomerase and Rab25 anticentromeric antibodies, antiphospholipid antibodies and cryoglobulins was unfavorable. Thyroid function was normal. Serum protein electrophoresis showed polyclonal hypergammaglobulinemia without paraproteinemia. In addition, immunofixation electrophoresis of serum and urine did not show monoclonal paraproteinemia. Chest radiograph was normal, as were the results of lung function assessments, abdominal ultrasound, echocardiography, nailfold capillaroscopy, radiographs of the hands and feet and bone marrow examination. Since electromyography detected signs consistent with myopathy, neurological examination Bisoprolol fumarate and muscle biopsy were performed. The former revealed no pathology but the biopsy exhibited moderate myopathy with atrophy involving selectively type 2 fibers without inflammation. Photoplethysmography did not confirm Raynaud’s phenomenon. Computer tomography of the periorbital region excluded involvement of extracutaneous tissue with nodules. Biopsies of the nodules around the hand and periorbital region and of papular lesions around the trunk were performed. Histopathological examination was characteristic of scleromyxedema in all three Bisoprolol fumarate specimens. There was abundant proliferation of large stellate fibroblasts in the reticular dermis and subcutis, with prominent deposits of a myxoid material between thick collagen bundles. Mucin deposition was exhibited with.