Signs and symptoms include abnormal accumulation of mucin in the skin, causing papular and sclerodermoid bumps. Hyperplasia of fibroblasts in the dermis that is a characteristic feature of papular mucinosis scleromyxedema is not observed in. Links to pubmed are also available for selected references. A 21yearold woman born of consanguineous parents presented with asymptomatic, waxy, white, beaded papules along the eyelid margins of 6 years duration. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. Gom player is a fantastic free media player for windows. Conclusions the patient showed an unusual manifestation of localized papular mucinosis with the lesions confined to the neck and iga nephropathy. We report the successful treatment of plaquelike sclerodermiform. Lichen myxedematosus lm, commonly referred to as papular mucinosis, is a rare papular eruption defined by mucin deposition in the dermis. Papular mucinosis lichen myxoedematosus is an unique, chronic, idiopathic disease characterized by lichenoid papules, nodules due to dermal mucin deposition and a variable degree of fibrosis. Scleromyxedema sm is a generalized papular and sclerodermoid form of lm. Papulonodular mucinosis pnm is a form of cutaneous mucinosis characterized by aberrant. Papular mucinosis discrete papular lichen myxedematosus jennifer bragg md, anthony c soldano md, joann m latkowski md dermatology online journal 14 5.
The combination of bortezomib and dexamethasone is an. Cutaneous mucinosis has been described in a variety of mixed connective tissue disorders. Papulonodular mucinosis pnm is a form of cutaneous mucinosis characterized by aberrant accumulation of glycosaminoglycans between collagen bundles. The generalised form also known as scleromyxoedema presents with widespread fleshcoloured papules arranged in lines and progressive skin thickening.
Scleromyxedema, a rare cutaneous mucinosis of unknown cause, is a variant of generalized papular mucinosis that is also known as generalized lichen myxedematosus. Selfhealing papular mucinosis in an adult sciencedirect. Papulonodular mucinosis in systemic lupus erythematosus. Selfhealing papular mucinosis in adults is a rare variant of localized papular mucinosis. If you have problems viewing pdf files, download the latest version of adobe reader. Selfhealing juvenile cutaneous mucinosis is an extremely uncommon disorder characterized by the acute eruption of multiple papules and subcutaneous nodules.
Papular mucinosis also known as scleromyxedema, generalized lichen myxedematosus, and sclerodermoid lichen myxedematosus is a rare skin disease. It is a rare deposition disorder characterized by generalized papular and sclerodermoid eruptions, mucin. It appears as mild, moderate, or severe exaggeration of skin folds, especially on the head, ventrum, and distal extremities. Mucinoses encompass a group of diverse skin disorders which can. Mucinoses are a group of cutaneous diseases caused by fibroblasts producing abnormally large amounts of acid mucopolysaccharides i. Dermal mucinoses are a heterogeneous group of diseases in which an abnormal deposition of mucin occurs in the dermis.
Our diagnosis was papular mucinosis synonym, lichen myxedematosus. It is characterized clinically by generalized papular or. It is a progressive disease of unknown etiology with systemic manifestations that cause serious morbidity and mortality. Selfhealing juvenile cutaneous mucinosis sciencedirect. Papular mucinosis scleromyxoedema of arndtgottron associated with paraprotein igg kappa type. Scleromyxedema sm is a sclerotic variant of lichen or papular mucinosis in which lichenoid papules and sclerodermalike features are both present. A number of authors have suggested the possibility that papular mucinosis and papulonodular mucinosis. Papular mucinosis genetic and rare diseases information. Get a printable copy pdf file of the complete article 885k, or click on a page image below to browse page by page. Oral focal mucinosis ofm is an extremely rare, benign oral soft tissue condition. One patient had sclerodermoid features consistent with the scleromyxedema variant of papular mucinosis. Lichen myxedematosus is an idiopathic, cutaneous mucinosis with 2 clinicopathologic subsets. Lichen myxedematosus an overview sciencedirect topics. Pdf oral focal mucinosis of the tongue researchgate.
Plaquelike sclerodermiform localized mucinosis rapidly. Lichen myxoedematosus or papular mucinosis is a rare skin disorder characterised by mucin deposits in the skin. The terms papular mucinosis and lichen myxoedematosus are generally used interchangeably to describe the occurrence of this condition as a localized and less severe form, while the term scleromyxoedema refers to a generalized, more severe form. A study of 14 patients with focal mucinosis revealed the usual clinical featuresa solitary, asymptomatic, fleshcolored to white papule or nodule. Pdf acral persistent papular mucinosis appm is a rare subtype of localized lichen myxedematosus. Papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. Papular mucinosis genetic and rare diseases information center. It is well known for its high video and audio playback quality and support of multiple video formats, but theres much more to this program tha. Cutaneous mucinosis of infancy cmi is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. Mucinosis definition of mucinosis by medical dictionary. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for papular mucinosis. Pdf acral persistent papular mucinosis with pruritic.
Papular mucinosis discrete papular lichen myxedematosus. Selfhealing juvenile cutaneous mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, and is characterized by. Scleromyxedema sm is a rare primary cutaneous inflammatory mucinosis characterised by papular mucinosis, monoclonal gammopathy and extracutaneous involvement. Most therapeutic options have failed in sm but high. A diagnosis of localized papular mucinosis with iga nephropathy atypical form was made after the biopsy. For language access assistance, contact the ncats public information officer. In the latter, papules are the usual clinical presentation. Papular mucinosis lichen myxedematosus is a rare cutaneous mucinosis in which.
In this report, we describe one of the youngest patients reported to date with selfhealing juvenile cutaneous mucinosis and further. Lichen myxedematosus lm is a primary mucinosis that is not accompanied by thyroid disease. Papulonodular mucinosis in systemic lupus erythematosus victoria g ortiz md a, ravi s krishnan md a, ling l chen md b, and sylvia hsu md a dermatology online journal 10 2. Nevus mucinosis genetic and rare diseases information. Apple inc free itunes legacy as one of the most popular and successful programs out there truly speaks for. Papular and nodular mucinosis is a rare but wellcharacterized, distinctive form of cutaneous mucinosis associated with systemic lupus erythematosus.
In the literature, the terms lm, papular mucinosis, and scleromyxedema have been often used. Pubmed is a searchable database of medical literature and lists journal articles that discuss nevus mucinosis. The diagnosis of papular mucinosis, a pseudoscleroderma syndrome, should be considered in a patient with atypical arthritis, median neuropathy, myopathy. Papular mucinosis is a quite rare disease of unknown etiology, with fewer than one hundred and fifty. The pathologic changes consisted of fibroblastic proliferation in the corium, an increased production of hyaluronic acid by these fibroblasts and an. In the literature, the terms lm, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of lm or papular mucinosis without indication of. Pdf papular mucinosis lichen myxoedematosus is an unique, chronic, idiopathic disease. It is described in some connective tissue diseases but never in association with mixed. Beaded papules along the eyelid margins mdedge dermatology. Scleromyxedema is a rare condition characterized by deposition of mucin in the skin and other.
Frequently, all three forms are regarded as papular mucinosis. In the updated classification of lm given by rongioletti et al. Localized lichen myxedematosus is a group of skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, a disease for which there is no treatment 185 see also. Papular mucinosis, or localized lichen myxedematosis lm. In dermal mucinoses, either a diffuse infiltration or a focal deposit of mucin may occur. Papular mucinosis of infancy is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, characterized by skincolored or translucent papules 186 see also. The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin or within the hair follicle. Review of primary cutaneous mucinoses in nonlupus connective.
Additionally, collagen bundles are thickened with clear separation between them. Although selfhealing papular mucinosis is not associated with systemic organ involvement, the disease is. Papular and nodular mucinosis as a presenting sign of. A clinicopathologic and histochemical study of eight cases. The localised form presents with firm, waxy papules with localised distribution and without systemic features. Localized and disseminated cases are called papular. The lesions are asymptomatic erythematosus to skincolored papules and nodules that. It is rare in dogs, except for chinese shar pei dogs. Papular mucinosis affects adults of both sexes equally and appears between ages 30. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Papular mucinosis definition of papular mucinosis by. Using papular mucinosis or lichen myxedematosus as your search term should help you locate articles. The clinical presentation is gradual with the development of firm, fleshcolored, 2 to 3mm papules often involving the hands, face, and neck that.
Click on the link to view a sample search on this topic. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a serum monoclonal igg paraprotein. Papular mucinosis, or localized lichen myxedematosis lm discrete papular type 2017. Alcian blue staining is a useful way to determine kidney involvement of papular mucinosis. If the inline pdf is not rendering correctly, you can download the pdf file here. Although the juvenile variant is characterized by its association with systemic symptoms, the adult variant has no clinical predictive markers. A 56yearold woman presented with small, skincolored papules on. 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. Scleromyxedema genetic and rare diseases information. Selfhealing juvenile cutaneous mucinosis wikipedia. Use the advanced andor limits search features to narrow your search results. Papular mucinosis scleromyxedema, also known as generalized lichen myxedematosus, is a rare dermal mucinosis characterized by a papular eruption that can have an associated igg. Mucin deposition is a common histological finding in patients with cle, but it is unusual for the mucin to be present in such a quantity to produce detectable skin lesions.