Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The differential diagnosis is determined by a combination of clinical presentation, serological studies, and histopathological examination. Currently, well-established and accepted histopathological features to distinguish localized LM from scleromyxedema have not been elucidated. Our recent publication, together with a retrospective literature review, suggests that the presence of groups of light chain-restricted plasma cells represents a distinct histopathological clue for the diagnosis of localized LM. In this report, we provide two additional cases of localized LM with lambda light chain-restricted plasma cells, together with clinical and histopathological findings that are similar to our previous publication. These cases support our theory that the light chain-restricted plasmacytic microenvironment is primarily attributed to the pathogenesis of localized LM. Therefore, we consider these cases to constitute a clinically and pathologically new variant of localized LM and name it primary localized cutaneous LM with light chain-restricted plasma cells.

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The term \'sclerosing diseases of the skin\' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

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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.