Lichen myxoedematosus or papular mucinosis is a rare skin disorder characterised by mucin deposits in the skin. Papular mucinosis associated with monoclonal gammopathy of unknown significance sarvesh s thatte, atul m dongre, siddhi b chikhalkar, uday s khopkar department of dermatology, seth g. Oral focal mucinosis is the microscopic counterpart of the cutaneous focal mucinosis or cutaneous myxoid cyst. Ty bollinger cancer truths and alternative treatments for cancer. 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. Mucinosis, papular and nodular in connective tissue diseases. Papular and nodular mucinosis is a rare but wellcharacterized, distinctive form of cutaneous mucinosis associated with systemic lupus erythematosus. The lesions are asymptomatic erythematosus to skincolored papules and nodules that. Lichen myxedematosus lm, commonly referred to as papular mucinosis, is a rare papular eruption defined by mucin deposition in the dermis. For language access assistance, contact the ncats public information officer. Papular mucinosis, or localized lichen myxedematosis lm. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a serum monoclonal igg paraprotein.
Benign or noncancerous forms of follicular mucinosis benign, primary or idiopathic follicular mucinosis are often of unknown cause. Chronic papular eruption of the skin photo quiz american. Papular mucinosis pm, also called lichen myxedematosus, is a primary cutaneous mucinosis and is. Focal cutaneous mucinosis see the pathology of focal cutaneous mucinosis. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Papular mucinosis pm lichen myxoedematosus is a unique, chronic idiopathic disease characterized by lichenoid papules or nodules due to dermal mucin deposition and a variable degree of fibrosis. Sir, papular mucinosis pm, also called lichen myxedematosus, is a primary cutaneous mucinosis and is characterized by dermal mucin deposition in absence of thyroid disorder.
We report a patient with papular mucinosis who presented to us with bilateral madarosis as the only complaint. Hiromasa nikai, in diagnostic surgical pathology of the head and neck second edition, 2009. We describe the case of a patient with progressive systemic sclerosis and papular. Papular mucinosis of infancy, also known as cutaneous mucinosis of infancy, appears as firm, opalescent papules on the upper arms and trunk. Lichen nitidus is a chronic papular eruption of the skin that most commonly affects children and young adults but has been reported in the elderly. Discrete acral nonpapular localised lichen myxedematosus. Mucinoses encompass a group of diverse skin disorders which can vary from minor cosmetic nuisances to potentially. Scleromyxedema, the most common form, is almost always. Lms are a group of localized or generalized papular eruptions of unknown etiology categorized as cutaneous mucinoses in which dermal mucin deposition is the distinctive histologic feature.
Updated classification of papular mucinosis, lichen. One patient had sclerodermoid features consistent with the scleromyxedema variant of papular mucinosis. Mucinoses are a group of cutaneous diseases caused by fibroblasts producing abnormally large amounts of acid mucopolysaccharides i. Scleromyxedema sm is a generalized papular and sclerodermoid form of lm.
It has since been reported in patients with systemic lupus erythematosus 217, discoid lupus erythematosus 2, 18, and subacute cutaneous lupus. Lichen myxedematosus lm is an idiopathic cutaneous mucinosis. Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. Review of primary cutaneous mucinoses in nonlupus connective.
Congenital cutaneous mucinosis with spontaneous regression. Covering all aspects of skin disease from basic science through pathology. The condition needs to be excluded from scleromyxedema, which, in addition to the firm papular. Mar 11, 2019 the terms lichen myxedematosus, papular mucinosis, and scleromyxedema are used interchangeably to describe the same disorder. A case of localized papular mucinosis showing excellent response. Volume 44, issue 2, part 1, february 2001, pages 273281.
Mar 19, 2012 papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. Natural cure for papular mucinosis and alternative treatments. In the lymphomaassociated form, the underlying condition is usually cutaneous tcell lymphoma mycosis fungoides. Bilateral madarosis due to papular mucinosis vasanth v.
Thorough investigation is warranted to arrive at an accurate diagnosis of the underlying condition. Most patients previously reported to have papular mucinosis had the generalized lichenoid papular form sderomyxedema with abncrma gamma globulin. Jun 17, 2012 cutaneous mucinosis covers a heterogeneous group of diseases involving a diffuse and or focal mucin accumulation in the skin and its annexes. Papular mucinosis associated with monoclonal gammopathy of. Microscopic examination of affected skin showed increased acid mucopolysaccharides in the dermis and proliferation of fibroblasts. If you have problems viewing pdf files, download the latest version of adobe reader.
Mucinosis definition of mucinosis by medical dictionary. Pdf papular mucinosis lichen myxoedematosus is an unique, chronic, idiopathic disease characterized by. Madarosis is a window to diagnosis for several diseases. Usually, the patient with madarosis consults a dermatologist for cosmetic reasons. Mucinosis, papular and nodular in connective tissue. A spectrum of disease appears to exist, with the more localized, less severe forms, which are generally called lichen myxedematosus or papular mucinosis, and the more sclerotic, diffuse form, which is referred to as sc. Signs and symptoms of the condition include the presence. M u c i n deposition dissecting the dermal collagen fibers colloidal iron discussion scleromyxedema or lichen myxedematosus lm is a rare type of papular mucinosis, with a chronic and progressive course, chronic, progressive, of unknown etiology. An updated classification of idiopathic cutaneous mucinosis lichen myxoedematosus, included three clinicopathological subsets.
Scleromyxedema, a rare cutaneous mucinosis of unknown cause, is a variant of generalized papular mucinosis that is also known as generalized lichen myxedematosus. Pueden asociarse a enfermedades sistemicas escleromixedema o no mucinosis papulares o liquen mixedematoso localizado, aunque en algunos casos. Pm is a quite rare disease of unknown etiology, with fewer than one hundred and fifty cases reported. Mucinosis, papular and nodular in connective tissue diseases mucinosis, pinkus follicular mucinosis, reticular erythematosus mucinosis, self. Using papular mucinosis or lichen myxedematosus as your search term should help you locate articles. Papulonodular mucinosis pnm is a form of cutaneous mucinosis. Papulonodular mucinosis, also termed nodular cutaneous lupus mucinosis, was first reported in 1954 by gold, who described an unusual papular eruption seen in systemic lupus erythematosus. Lichen myxedematosus lm, also called papular mucinosis, was first described by dubreuilh1 in 1906 and later classified by. Frequently, all three forms are regarded as papular mucinosis. It is a progressive disease of unknown etiology with systemic manifestations that cause serious morbidity and mortality. A patient with papular mucinosis scleromyxedema developed an erosive seronegative rheumatoidlike arthropathy.
Papular mucinosis genetic and rare diseases information. Papular mucinosis in alabama papular mucinosis in alaska. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. Localised lichen myxedematosus lm is a chronic idiopathic papular mucinosis that can be divided into five subtypes. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Use the advanced andor limits search features to narrow your search results. Papular mucinosis abnormal paraprotein in 90% of cases, usually of the igg type plasmacell dyscrasia of ten present skin biopsy shows a horizontal band of mucinous material between collagen bundles in the upper dermis increase in fibroblasts and dermal fibrosis. Papular and nodular mucinosis as a presenting sign of. Papular mucinosis abnormal paraprotein in 90% of cases, usually of the igg type plasmacell dyscrasia of ten present skin biopsy shows a horizontal band of mucinous material between collagen bundles in the upper dermis increase in fibroblasts and dermal fibrosis glycosaminoglycan positive for alcian blue at ph 2. Papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. A case of localized papular mucinosis showing excellent response to cyclophosphamide. Pdf hyaluronidase in the treatment of papular dermal mucinosis. The localised form presents with firm, waxy papules with localised distribution and without systemic features.
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. The papules in papular mucinosis contain mucin and on injury the papular wound may exude mucin. Papular mucinosis, international journal of dermatology. The generalised form also known as scleromyxoedema presents with widespread fleshcoloured papules arranged in lines and progressive skin thickening. Per the familys report, the lesion had grown significantly since it was first noticed 2 years ago, with current measurements of 7. Papular mucinosis scleromyxoedema is an uncommon disorder characterized by generalized papular eruption and cuta neous induration, which may be associated with a variety of extracutaneous manifestations. Mucinous disorders of the skinbeyond the dermal mucinosis. Updated classification of papular mucinosis, lichen myxedematosus. Pdf ppapular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition and. The nomenclature and classification of cutaneous mucinosis is quite complex. Papular mucinosis, or localized lichen myxedematosis lm discrete papular type 2017. Scleromyxedema, the most common form, is almost always associated with paraproteinemia. Mucinosis papular asociada a lupus eritematoso sistemico dialnet.
M u c i n deposition dissecting the dermal collagen fibers colloidal iron discussion scleromyxedema or lichen myxedematosus lm is a rare type of papular mucinosis, with a chronic and progressive course. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. Papular mucinosis definition papular mucinosis is categorized as a highly rare skin disease. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a s. Mucin deposition is a common histological finding in patients with cle, but it is unusual for the mucin to be present in. Dermoscopic images of papules showing orange background and round vessels. Papular mucinosis or lichen myxedematosus is a disorder of cutaneous mucin deposition.
Papular mucinosis pm, also called lichen myxedematosus, is a primary cutaneous mucinosis and is characterized by dermal mucin deposition in absence of thyroid disorder. It is characterized clinically by generalized papular or scleroderma. This type of mucinosis has not been reported in the dog to the authors knowledge. Aug 11, 2014 signs and symptoms of papular mucinosis the localized form of lichen myxedematosus disorder involves mucinosis of limited areas on hands, face and extremities and appears as waxy solid bumps, nodules, papules and plaques. Although scleroderma and papular mucinosis share many features, they are clinically and histologically distinct entities. Papulonodular mucinosis in systemic lupus erythematosus. Lichen myxedematosus lm, also called papular mucinosis, was first described by dubreuilh1 in 1906 and later classified by montgomery and underwood2 in 1953. Papular mucinosis definition of papular mucinosis by. We present a case of a 4yearold fairskinned girl, with no significant medical history, who presented to the outpatient clinic of an academic medical center with an asymptomatic, fleshcolored, cobblestoned plaque on her lateral left thigh fig 1. Conclusions the patient showed an unusual manifestation of localized papular mucinosis with the lesions confined to the neck and iga nephropathy. Papular mucinosis genetic and rare diseases information center.
Lichen myxoedematosus papular mucinosis acral persistent papular mucinosis. A diagnosis of localized papular mucinosis with iga nephropathy atypical form was made after the biopsy. Cutaneous mucinosis in mixed connective tissue disease. It is described in some connective tissue diseases but never in association with mixed. Lichen myxedematosus is condition characterized by localized areas of dermal deposition of mucin, presenting with firm papules localized to few areas of the body.
Papular mucinosis, destructive arthropathy median neuropathy, and. Papular mucinosis also known as scleromyxedema, generalized lichen myxedematosus, and sclerodermoid lichen myxedematosus is a rare skin disease. It is characterized clinically by generalized papular or. Alcian blue staining is a useful way to determine kidney involvement of papular mucinosis. Scleromyxedema papular mucinosis lichen myxedematosus. Rooks textbook of dermatology is the most comprehensive work of reference available to the dermatologist. Mucinosis papular acral persistente actas dermosifiliograficas. 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. Primary mucinoses can be divided into dermal and follicular mucinoses dermal mucinoses include lichen myxoedematosus, reticular erythematous mucinosis, scleredema, mucinoses in thyroid disease, papular and nodular mucinosis in connective tissue disease, self.
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