The use of improved ruthenium red staining for the ultrastructural detection of proteoglycan aggregates in normal skin and Lichen myxoedematosus

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.     

Cutaneous mucinosis of infancy: is it a real entity or the paediatric form of lichen myxoedematosus (papular mucinosis)?

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 with associated cardiac abnormalities

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.     

Discrete papular mucinosis-a rare subtype of lichen myxoedematosus

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.     

Circumscribed myxoedema of lichen myxoedematosus as a sign of faulty formation of the proteoglycan macromolecule

Using a new method devised by our laboratory, the ultrastructure of dermal glycosaminoglycan in an involved area of lichen myxoedematosus was examined. Although histochemical and biochemical studies have indicated simply an accumulated deposition of hyaluronic acid in the lesion, the glycosaminoglycan ultrastructure within it was distinaly different from that in normal skin. The glycosaminoglycan structure of normal skin was similar to the proteoglycan aggregate model described by Rosenberg (1975). As confirmed by the enzymatic digestion procedure, it represents the ultrastructure of hyaluronic acid bound to glycosaminoglycans such as dermatan sulphate or chondroitin sulphate. In contrast, hyaluronic acid filaments observed in lesions of lichen myxoedematosus contained no glycosaminoglycan subunits.     

Cutaneous mucinoses and HIV infection

In the last few years cutaneous mucinoses have been reported with increased frequency in HIV patients. We report the occurrence of scleredema, reticular erythematous mucinosis and lichen myxoedematosus in three different HIV-infected patients, review the literature and discuss the possible relationship between mucin deposits and HIV infection. This is the first report of scleredema and the second of reticular erythematous mucinosis in an HIV-infected patient. Only the association of HIV infection with lichen myxoedematosus seems to be more than coincidental.     

Failure of PUVA in lichen myxoedematosus: acceleration of associated multiple keratoacanthomas with development of squamous carcinoma

We report a case of lichen myxoedematosus/scleromyxoedema based on IgG-kappa para-proteinaemia and associated with multiple keratoacanthomas. With PUVA therapy there was little change in the scleromyxoedema; however, the growth of keratoacanthomas was accelerated, and squamous carcinoma developed in one lesion.     

MULTIPLE SERUM PROTEIN ABNORMALITIES IN LICHEN MYXOEDEMATOSUS

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.     

Scleromyxoedema with disseminated subcutaneous nodules: rare presentation of an uncommon dermatosis

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.     

Acral persistent papular mucinosis

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.     

Remission of scleromyxoedema following treatment with extracorporeal photopheresis

Scleromyxoedema, a disseminated papular and sclerotic variant of lichen myxoedematosus, is a rare disease with a chronic progressive course, and little tendency towards spontaneous remission. The treatment of scleromyxoedema has been largely ineffective. Aggressive chemotherapeutic agents have been used, often leading to therapy-related morbidity and mortality. We report a 41-year-old woman with scleromyxoedema, associated with a monoclonal gammopathy of IgG-k type, whose condition almost completely cleared with 12 monthly sessions of extracorporeal photopheresis. The patient had previously not responded to isotretinoin, and chlorambucil with prednisolone.     

Congenital cutaneous mucinosis with spontaneous regression: an atypical cutaneous mucinosis of infancy?

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.     

Localized lichen myxoedematosus (papular mucinosis) associated with morbid obesity: report of two cases

Cutaneous diseases are often found in obese patients but, to our knowledge, mucinous disorders have not been previously reported in association with obesity. Two cases of localized lichen myxoedematosus (papular mucinosis) in two women with morbid obesity are described. Both patients underwent a low-calorie diet for a 1-year period in one case, and for 4 months in the other one, as the only treatment. There was complete resolution of cutaneous lesions at the same time that an important weight loss was observed. Nevertheless, although spontaneous regression is not frequent, it could not be disregarded in either of these two cases.     

Lichen myxoedematosus mit konsekutivem Skleromyxödem Arndt-Gottron bei Paraproteinämie,seroaktiver Toxoplasmose und Myokardhypoxie

Zusammenfassung Bericht über einen 33jährigen Mann mit Skleromyxödem, das sich innerhalb von 2 Jahren aus einem umschriebenen Lichen myxoedematosus entwickelt hat. Eine gleichzeitig nachweisbare seroaktive, klinisch stumme Toxoplasmose-Infektion und Paraproteinämie werden bezüglich ihrer Bedeutung für das Krankheitsgeschehen diskutiert. Außer einer plasmacellulären Markreaktion (Sternalmark) konnte ein Myocardschaden mit einer Erregungsrückbildungsstörung aufgedeckt werden. Gleichartige histologische veränderungen, die gemeinsame Proteinanomalie sowie der auch bei unserem Patienten beobachtete Übergang vom Lichen myxoedematosus in ein Skleromyxödem lassen für beide Dermatosen eine einheitliche Ätiopathogenese vermuten. Klinisch-morphologisch sollte jedoch zwischen beiden Dermatosen auch weiterhin differenziert und die Sonderstellung des Skleromyxödems unter den euthyreotischen Myxodermien beachtet werden.

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Lichen myxedematosus with consecutive skleromyxedema arndt-gottron and paraproteinemia, seroactive toxoplasmosis and cardiac hypoxia

Summary Report of a 33 years old male whit scleromyxedema which developed from a lichen myxedematosus in the course of two years. At the same time a seroactive, clinical silent toxoplasmosis and paraproteinemia were found which are discussed in relation to their significance of pathogenesis of scleromyxedema. Besides a plasmacellulare marrow-reaction (sternal-marrow) a cardiac hypoxia was revealed electrocardiografically. Similar histologic changes, commensurate proteinanomalies and the passage from lichen myxedematosus to scleromyxedema observed in our case suspect a homonymous aetiopathogenesis of both dermatoses.Clinical-morphological it should be differentiated between lichen myxedematosus and scleromyxedema and it should be emphasized the exceptional position of scleromyxedema within the euthyreodic myxodermias.

     

肢端持久丘疹性黏蛋白沉积症一例

患者女,46岁,双手背、手腕伸侧对称多数肤色小丘疹4年,不伴瘙痒、疼痛.皮肤组织病理检查:真皮浅中层胶原断裂,可见片状淡染物质沉积,未见成纤维细胞增生.阿新蓝染色显示:真皮浅中层胶原纤维间黏蛋白沉积,证实为肢端持续丘疹黏性蛋白沉积症.肢端持续丘疹黏蛋白沉积症属于皮肤黏蛋白沉积症局限型中的一个亚型,临床上需要与黏液水肿性苔藓散发丘疹型、皮肤局灶性黏蛋白沉积症及伴甲状腺病性黏蛋白沉积症进行鉴别.     

肢端持久丘疹性黏蛋白沉积症一例

患者女,46岁,双手背、手腕伸侧对称多数肤色小丘疹4年,不伴瘙痒、疼痛.皮肤组织病理检查:真皮浅中层胶原断裂,可见片状淡染物质沉积,未见成纤维细胞增生.阿新蓝染色显示:真皮浅中层胶原纤维间黏蛋白沉积,证实为肢端持续丘疹黏性蛋白沉积症.肢端持续丘疹黏蛋白沉积症属于皮肤黏蛋白沉积症局限型中的一个亚型,临床上需要与黏液水肿性苔藓散发丘疹型、皮肤局灶性黏蛋白沉积症及伴甲状腺病性黏蛋白沉积症进行鉴别.     

肢端持久丘疹性黏蛋白沉积症一例

患者女,46岁,双手背、手腕伸侧对称多数肤色小丘疹4年,不伴瘙痒、疼痛.皮肤组织病理检查:真皮浅中层胶原断裂,可见片状淡染物质沉积,未见成纤维细胞增生.阿新蓝染色显示:真皮浅中层胶原纤维间黏蛋白沉积,证实为肢端持续丘疹黏性蛋白沉积症.肢端持续丘疹黏蛋白沉积症属于皮肤黏蛋白沉积症局限型中的一个亚型,临床上需要与黏液水肿性苔藓散发丘疹型、皮肤局灶性黏蛋白沉积症及伴甲状腺病性黏蛋白沉积症进行鉴别.     

Discrete papular lichen myxoedematosus: a rare subtype of cutaneous mucinosis

Primary cutaneous mucinoses are characterized by abnormal mucin deposits in the skin. Discrete papular lichen myxoedematosus (DPLM) is an unusual subtype. Only 11 of the cases described in the literature to date showed no relation to human immunodeficiency virus (HIV) infection. We report a 21‐year‐old woman with numerous symmetrical asymptomatic papules on her trunk, arms and thighs. Results of investigations were normal. On histological examination, the upper dermis showed a focal mucin deposit. DPLM can be associated with HIV or C hepatitis virus (HCV) infection and therefore an early diagnosis is very important.     

Scleromyxoedema (lichen myxoedematosus) associated with a paraprotein, IgG1 of type kappa

A case of scleromyxoedema (lichen myxoedematosus) associated with an IgG₁, type kappa paraproteinaemia is reported. Culture of bone marrow material demonstrated that this tissue synthesized monoclonal IgG (subclass γ I) with a light chain of type kappa. The culture of a biopsy specimen of the diseased skin demonstrated low-speed IgG synthesis. It is concluded that the bone marrow and pathological skin synthesize a paraprotein with the same characteristics as that in the serum. Microchemical analysis of pathological skin demonstrated an increase in the content of neutral glycoproteins, hyaluronic acid and chondroitin sulphates as well as the presence of heparin and keratan sulphates. It could not be demonstrated that the serum of this patient contained antibody against connective tissue ground substance or other skin constituents.     

Spontaneous resolution of lichen myxoedematosus

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.