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Scleromyxedema or generalized diffuse lichen myxoedematosus is a rare mucinosis that is associated with monoclonal gammopathy and which frequently affects multiple extracutaneous organ systems. The pathogenesis of scleromyxedema has not been fully elucidated, but includes stimulation of glycosaminoglycan synthesis. The clinical course of scleromyxedema is chronic and often progressive, leading to severe morbidity and even death. The characteristic skin findings encompass multiple waxy papules often on indurated plaques, while thickening of skin leads to conspicuous folds on glabella and dorsal aspects of finger joints. Microscopical manifestations are dermal deposits of glycosaminoglycans between collagen bundles in reticular dermis, increased numbers of fibroblasts and fibrosis as well as loss of elastic fibers. Progressive skin involvement results in decreased mobility of the mouth and joints and even contractures. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, in the kidneys or in the central and peripheral nervous system. There are no in-label or evidence-based treatments available for scleromyxedema, but by expert consensus high-dose immunoglobulins are considered as treatment of choice, followed in case of insufficient efficacy by systemic glucocorticosteroids and then lenalidomide or thalidomide. In severe and refractory cases, autologous hematopoietic stem cell transplantation has been performed. Long-term maintenance treatment is usually required to prevent recurrences. Close interdisciplinary follow-up is recommended.
UNASSIGNED: Das Skleromyxödem (generalisierter diffuser Lichen myxoedematosus) ist eine seltene Muzinose, die in Verbindung mit einer monoklonalen Gammopathie auftritt und mit einer unterschiedlich ausgeprägten systemischen Beteiligung einhergeht. Seine Pathogenese ist nicht genau geklärt, aber beinhaltet eine Stimulation der Glykosaminoglykanbildung. Der klinische Verlauf des Skleromyxödems ist chronisch, oft progressiv und dann mit schwerer Morbidität oder möglichem letalem Ausgang verbunden. Der typische Hautbefund umfasst multiple, gruppierte, wachsartige Papeln, oft auf indurierten Plaques sowie auffällig werdende Hautverdickungen an Glabella und Fingerstreckseiten. Histologische Kriterien sind die dermale Ablagerung von Glykosaminoglykanen zwischen den Kollagenbündeln der retikulären Dermis, eine Vermehrung der Fibroblasten und eine Fibrose sowie ein Verlust elastischer Fasern. Ein fortschreitender Hautbefall führt zu einer verminderten Beweglichkeit von Mund und Gelenken bis zu Kontrakturen. An Organen können Herz, Lunge, Gastrointestinaltrakt, Niere, Bewegungsapparat sowie peripheres und zentrales Nervensystem befallen sein. Eine für das Skleromyxödem zugelassene oder evidenzbasierte Therapie gibt es nicht, doch laut übereinstimmender Expertenmeinung wird als Therapie der ersten Wahl die Gabe intravenöser Immunglobuline empfohlen. Im Fall ungenügenden Ansprechens folgen systemische Glukokortikosteroide und dann Lenalidomid oder Thalidomid, bei schweren therapierefraktären Verläufen auch eine autologe hämatopoetische Stammzelltransplantation. In der Regel treten nach Absetzen einer erfolgreichen Therapie Rezidive auf. Eine engmaschige interdisziplinäre Anbindung ist ratsam.

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This case report describes erythematous patches on the face that involved the nasolabial folds, as well as numerous skin-colored homogenous waxy papules on the arms, neck, and trunk.

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Oral focal mucinosis (OFM) is a rare connective tissue disorder that is characterized by the excessive production of hyaluronic acid due to myxoid degeneration of submucosal connective tissue. The disorder typically presents as an asymptomatic nodule or mass in the gingiva or hard palate, and OFM of the tongue is even more unusual. In this report, we present a case of OFM on the tongue in a 72-year-old female patient who presented with a symptomatic lump that had been growing for 6 months on the dorsum of her tongue. The patient reported discomfort and pain while speaking and swallowing, and the lump was visually apparent on examination. OFM is a benign condition that does not have any specific clinical or radiographical features that distinguish it from other more common oral lesions, such as lipoma or fibroma. Therefore, histopathological examination is essential for a definitive diagnosis. The management of OFM typically involves surgical excision of the lesion. In this case, complete surgical removal of the lesion under general anesthesia was performed, and the patient was followed up for 10 months postoperatively. During the follow-up period, there was no evidence of recurrence, and the patient reported significant improvement in her symptoms. In conclusion, OFM is a rare connective tissue disorder that can occur in the oral cavity. Although OFM of the tongue is even rarer, it should be considered in the differential diagnosis of oral lesions. Histopathological examination is essential for definitive diagnosis, and surgical excision is typically the preferred treatment modality.

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UNASSIGNED: Self-healing juvenile cutaneous mucinosis is a rare entity, characterized by the presence of subcutaneous nodules together with frequent nonspecific systemic symptoms, which occurs in the pediatric age and characteristically resolves spontaneously. Although the diagnostic criteria do not require a biopsy to be performed, it is frequently performed, and an abundant dermal mucin deposition will be observed together with other features such as fibroblastic proliferation. Although the prognosis is benign, follow-up is required for the eventual development of a rheumatologic disease.We present 2 clinical cases, describing the clinical findings and their histopathologic correlation. Comparatively, the outcome in both cases was different: in one case, the mucinosis resolved without any related event in the follow-up, and in the other case, the resolution was accompanied by the subsequent development of idiopathic juvenile arthritis.

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