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Nodular lichen myxoedematosus is a localised form of lichen myxoedematosus, a chronic idiopathic cutaneous mucinosis of known aetiology. Ustekinumab is a human interleukin-12/23 monoclonal antibody that could directly or indirectly increase mucin production. Herein, we report for the first time a case of nodular lichen myxoedematosus associated with ustekinumab.

Papular mucinosis (PM) (lichen myxoedematosus) is a unique, chronic idiopathic disease characterized by lichenoid papules or nodules due todermal mucin deposition and a variable degree of fibrosis. PM is a quite rare disease of unknown etiology, with fewer than one hundred andfifty cases reported. In this paper, we present two cases of PM with no associated monoclonal gammopathy in two male patients aged 75 and38 years, for its rare occurrence in the literature., Papüler müsinozis (PM) (liken miksödematozis) likenoid papüller ve nodüller, dermal müsin birikimi ve değişken derecedeki fibrozis ile karakterizederinin oldukça nadir, kronik ve idiyopatik bir hastalığıdır. PM etiyolojisi bilinmeyen, literatürde yüz elli olgudan daha az bildirilmiş olan, oldukçanadir görülen bir hastalıktır. Literatürde seyrek görülmesi nedeniyle ek bir hastalıkla ilişkili olmayan iki PM olgumuzu sunmak istedik. Burada, 75ve 38 yaşında iki erkek PM’li hasta monoklonal gammopati olmaksızın nadir olarak görüldüğünden dolayı sunulmaktadır.

Scleromyxoedema, also known as generalized lichen myxoedematosus, is a cutaneous mucinosis characterized by a generalized papular and sclerodermoid eruption, mucin deposition, increased fibroblast proliferation and fibrosis. It is often associated with underlying monoclonal gammopathy, and it responds poorly to treatment. There are very few reports of nodular eruption in scleromyxoedema. We report a case of a prominent nodular eruption in an adolescent boy with scleromyxoedema without any underlying paraproteinaemia, and review the literature.

The nomenclature and classification of cutaneous mucinosis is quite complex. An updated classification of idiopathic cutaneous mucinosis (lichen myxoedematosus), included three clinicopathological subsets: a generalized papular and sclerodermoid form, a localized papular form, and an atypical or intermediate form. Cutaneous mucinosis occurring in infancy is very rare. We report a 7-month-old boy with a history of multiple opalescent papules over his fingers and toes since birth. The lesions spontaneously regressed and became indiscernible after 5 years of clinical follow-up. The condition was similar to cutaneous mucinosis of infancy but was also unique because of its spontaneous regression and acral location.

Chronische Dermatose aus dem Formenkreis der Muzinosen mit Vermehrung von Fibroblasten und verstarkter Kollagensynthese. Assoziation mit Paraproteinamie/monoklonaler Gammopathie. Keine Assoziation mit Schilddrusenfunktionsstorung.

Das Skleromyxodem zahlt zu den seltenen Muzinosen bei Euthyreose. Nahezu obligat assoziiert findet sich eine monoklonale Gammopathie. Durch die Ablagerungen der Glykosaminglykane nicht nur in der Haut, sondern auch in inneren Organen wie z. B. dem kardiovaskularen System, stellt das Skleromyxodem eine Systemerkrankung mit unbestimmter Prognose dar. Wir berichten uber eine 74-jahrige Patientin mit initialem Skleromyxodem, die fruhzeitig mit Melphalan behandelt wurde. Eine vollstandige Remission mit derzeit anhaltender Rezidivfreiheit konnte nach nur vier Behandlungszyklen mit Melphalan und Prednisolon erreicht werden.

Lichen myxoedematosus is an uncommon and distinct disease entity characterized by cutaneous mucin deposition which, depending on the distribution and overall skin involvement, can be classified into several subtypes. We now describe the case of a discrete papular type of lichen myxoedematosus in a patient without any conspicious laboratory findings including normal thyroid function and the absence of any abnormal immunglobulins.

In 1986 Rongioletti introduced the term acral persistent papular mucinosis for persistent papules on the dorsum of the hands and distal forearms. Skin specimens reveal mucin deposits which resemble those in lichen myxedematosus (LM). Laboratory results are usually unremarkable. Our 54-year-old female patient showed a 2 1/2 year history of skin lesions in the typical acral distribution pattern. This entity appears to be an acral papular subtype of LM. In the classification of LM, acral papular lichen myxedematosus should replace the discrete papular form. This latter form has been well known for many years, but is not sufficiently definable and is of secondary clinical importance.

Cutaneous mucinoses, also called lichen myxoedematosus, are a group of diseases characterized by mucin deposit on the skin. They may be associated to systemic diseases (scleromyxedema) or be primary ones (papular mucinosis, localized lichen myxoedematous), although they share common findings (intermediate or atypical forms) in some cases. Acral persistent papular mucinosis (APPM) is a type of papular mucinosis that is located exclusively on the back of the hands and in the distal zone of the forearms and is not associated to any systemic disease. We present the case of a 52-year-old healthy woman who had skin lesions on the back of her hands and whose histological study confirmed an APPM.

Lichen myxoedematosus (LM), or papular mucinosis, is an uncommon papular eruption caused by the dermal deposition of mucin. Three clinical forms can be distinguished, namely localised, disseminated (involving more than one site), and generalised LM, the last is called scleromyxoedema, and demonstrates erythema and skin sclerosis. Paraproteinaemia, often consisting of an abnormal IgG with lambda light chains, is usually present in patients with LM. Visceral involvement has also been documented. An association between LM and human immunodeficiency virus (HIV) infection has been reported recently. We now describe two further HIV-positive patients with LM and present a review of the literature regarding this association.

Eine 23jahrige Patientin stellte sich mit einer seit dem 9. Lebensjahr bekannten kutanen Muzinose vor. Die Hande zeigten teigige Schwellungen und Akrozyanose und starke Bewegungseinschrankung, das Gesicht wies zahlreiche Angiome und Odeme auf, in der Sakralregion war ein dicker hautfarbener Plaque vorhanden. Nach Hautbefund, Histologie und weiteren klinischen Befunden wurde die Erkrankung als papulose Muzinose (Lichen myxoedematosus) eingeordnet. Wegen des starken Leidensdruckes der noch jungen Patientin und eines bereits erfolglos durchgefuhrten Therapieversuches mit Chloroquin entschieden wir uns fur den therapeutischen Einsatz der Plasmapherese ohne Begleittherapie. Bereits nach dem ersten Behandlungszyklus zeigte sich ein gebesserter Haut- und Allgemeinzustand. Nach insgesamt 4 Zyklen uber einen Zeitraum von 18 Monaten (Gesamtaustausch ca. 38,4 l) war ein objektiver Ruckgang der Plaques und insbesondere der Fingerschwellungen erkennbar. Die Plasmapherese ist eine effektive Behandlung fur schwere Formen der kutanen Muzinose, die ansonsten therapeutisch schwer zuganglich ist.

Lichen myxoedematosus is an unusual disorder of unknown etiology and pathogenesis. Although several treatments have reportedly been tried, therapeutic efficacies are variable and unsatisfactory. A patient with severe skin changes of this disease was successfully treated with PUVA photochemotherapy. PUVA therapy was carried out using 30 mg of 8-methoxypsoralen orally, and subsequent exposure to UVA starting at 4 J/cm2. The eruption disappeared almost completely after 35 treatments at a cumulative dose of 202 J/cm2. Histologically, mucin deposition was greatly diminished after the therapy. The photochemotherapy may exert its effect directly by inhibiting proliferation of fibroblasts and synthesis of mucopolysaccharides, and also indirectly by immunomodulating action.

We report a patient with acquired immunodeficiency syndrome (AIDS) who developed a widespread papular eruption due to deposition of mucin in the dermis. Paraproteinaemia was demonstrated. Lichen myxoedematosus type 2 was diagnosed. This is the third case of this rare disorder reported in a human immunodeficiency virus (HIV)-seropositive subject.

Lichen myxoedematosus (papular mucinosis) is characterized by papular lesions (and sometimes by internal involvement) caused by a dermal infiltrate of acid mucopolysaccharides which induce extensive thickening and hardening of the skin. The aetiopathogenesis of the disease is unknown. The therapy is generally by immunosuppression. We report the case of a 63-year-old man with lichen myxoedematosus successfully treated with cyclosporin A.

We describe a 42-year-old woman who developed lichen myxoedematosus. Twenty years after the onset of the disease she became breathless and hypertensive, and an echocardiogram showed a mass on the mitral valve, which was thought to be a mucin deposit. Her hypertension was resistant to treatment with combination antihypertensives. To our knowledge, this is the first report to link lichen myxoedematosus with a valvular mucinous mass. This case also demonstrates the slow clinical progression of the disease over 20 years.

We describe a girl presenting with a childhood dermal mucinosis in which we had the unique opportunity to find all the transitional histological features of lichen myxoedematosus (papular mucinosis), from its early focal mucin deposition in the reticular dermis to its late findings of interstitial mucin deposition, dermal fibrosis and fibroblast proliferation. Her father reported having had similar lesions when he was a child, which completely disappeared during adolescence. This case, and a re-evaluation of the literature, suggests that cases of cutaneous mucinosis of infancy that are not hamartomatous conditions such as mucinous naevi are in fact the infantile presentation of lichen myxoedematosus (papular mucinosis) and, in addition to other cases in the literature, suggests a genetic and familial factor in lichen myxoedematosus (papular mucinosis).

Lichen myxoedematosus (LM), a form of primary cutaneous mucinosis, may present either as localized less severe form called papular mucinosis or diffuse more severe form called scleromyxoedema. The diffuse form is almost always associated with monoclonal gammopathy, whereas localized form is not. We report an atypical case of localized form of LM associated with multiple myeloma in a 66-year-old male, who presented with asymptomatic waxy papular eruption on extremities, which on histopathological examination confirmed the diagnosis of cutaneous mucinosis. After initially being put on steroids and hydroxychloroquine with minimal improvement, patient subsequently presented with encephalopathy and on evaluation revealed hypernatremia, hypercalcemia, hypergammaglobulinemia, reversal of albumin-globulin (A/G) ratio, azotemia, and lytic lesions in skull X-ray. Bone marrow aspiration and biopsy confirmed multiple myeloma. Patient was successfully treated with standard treatment regimen for multiple myeloma with bortezumib and dexamethasone and his skin lesions subsided completely.

A case report of plaque and nodular type of lichen myxoedematosus of sudden onset and rapid progression without any systemic manifestation in a pregnant female.

Bericht uber einen 33jahrigen Mann mit Skleromyxodem, das sich innerhalb von 2 Jahren aus einem umschriebenen Lichen myxoedematosus entwickelt hat. Eine gleichzeitig nachweisbare seroaktive, klinisch stumme Toxoplasmose-Infektion und Paraproteinamie werden bezuglich ihrer Bedeutung fur das Krankheitsgeschehen diskutiert. Auser einer plasmacellularen Markreaktion (Sternalmark) konnte ein Myocardschaden mit einer Erregungsruckbildungsstorung aufgedeckt werden. Gleichartige histologische veranderungen, die gemeinsame Proteinanomalie sowie der auch bei unserem Patienten beobachtete Ubergang vom Lichen myxoedematosus in ein Skleromyxodem lassen fur beide Dermatosen eine einheitliche Atiopathogenese vermuten. Klinisch-morphologisch sollte jedoch zwischen beiden Dermatosen auch weiterhin differenziert und die Sonderstellung des Skleromyxodems unter den euthyreotischen Myxodermien beachtet werden.

Nach einer zusammenfassenden Darstellung der bislang in der Literatur mitgeteilten Falle von Lichen myxoedematosus wird uber einen eigenen Fall ausfuhrlich berichtet. Es konnte eine Uberempfindlichkeit gegen Heparin und eine auserordentlich gute, vorubergehende Beeinflussung durch Prednison festgestellt werden. Die Beziehungen zu dem kurzlich von Gay Prieto mitgeteilten Fall einer “eruptiven Kollagenose” werden erortert.

A case is presented of lichen myxoedematosus (urticarial type) in a man aged 46, followed for 9 years without having developed extracutaneous manifestations. Some aspects of the classification of cutaneous mucinoses are discussed.

Improved ruthenium red staining has been applied to normal human skin and lichen myxoedematosus skin lesions for the detection of the fine structure proteoglycan aggregates. In 4 samples of normal skin and in a patient with lichen myxoedematosus, the typical fine ultrastructures of proteoglycan aggregates, i.e., many short irregularly-curled filaments of varying lengths attached to long and rather straight filaments of hyaluronic acid were shown. Although lichen myxoedematosus is thought to be due to the faulty formation of proteoglycan aggregates, we suggest a different view, that this improved method of ruthenium red staining is useful for the detection of proteoglycan aggregates, and provides a better preparation for electron microscopy.

SUMMARY Lichen myxoedematosus was diagnosed in a 44-year-old woman in 1957. Despite the persistence of an abnormal serum protein, the skin lesions cleared by 1965. The protein abnormality disappeared several years later.

SUMMARY.— A case of lichen myxoedematosus is described in which multiple serum protein abnormalities have been demonstrated by disc electrophoresis, in addition to the previously described abnormal basic globulin fraction migrating in the post-gamma region on paper electrophoresis. Immunohistological studies indicate that there are abnormalities in the connective tissue between the collagen bundles as well as deposition of IgG globulin.

To the Editor.— Case reports and studies to date on lichen myxoedematosus, in particular its rare variant scleromyxoedema of Gottron 1 have stressed the absence clinically and biochemically of associated or complicating hypothyroidism. Most recently, Hill et al 2 described the successful use of radiation therapy in a patient with lichen myxoedematosus who had laboratory confirmation of normal thyroid function. They propose as one of three criteria for the diagnosis of this condition the exclusion of thyroid dysfunction by clinical and laboratory means. We think this is too restricted a view. Brigden 3 recently reported a case from our Unit of lichen myxoedematosus in a now 60-year-old man who has been troubled throughout the course of his disease by symptoms attributable to occlusive vascular disease that affect his left internal carotid artery. Increasing lethargy and poverty of thought led to assess