Scleromyxedema

Georgakis, Clara-Dina Cokonis; Falasca, Gerald F.; Georgakis, Alexander; Heymann, Warren R.

doi:10.1016/j.clindermatol.2006.07.011

Cited by 79 publications

(55 citation statements)

References 26 publications

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“…Classically, papules are small, waxy and symmetric in shape with lesions, most commonly affecting the face, neck, distal forearms and hands. Lichenification may be noted as well [1,2] . Although affected areas are not pruritic, koebnerization in areas of excoriation is widely reported [2] .…”

Section: Discussionmentioning

confidence: 99%

“…In some cases, skin may appear shiny as in scleroderma; however, the lack of telangiectasia and cutaneous calcinosis preclude this diagnosis. Diffuse involvement of the face may also produce characteristic leonine facies [1] . Definitive diagnosis of papular mucinosis is largely based on histopathologic findings, as clinical presentation may be non-specific.…”

Section: Discussionmentioning

confidence: 99%

“…This rare condition predominantly affects middle-aged adults between 30-80 years old with no predilection for race or gender [1,2] . Significant morbidity and mortality may be seen with ex-doi: 10.18282/jsd.v1.i2.43 tracutaneous manifestations involving the cardiac, gastrointestinal, pulmonary and central nervous systems [1] . Clinical presentation is characterized by widespread dome-shaped, flesh colored papules that are closely spaced or linearly arranged.…”

Section: Discussionmentioning

confidence: 99%

“…Papular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition with increased collagen in the skin and internal organs [1][2][3][4][5][6] . Also known as discrete papular lichen myxedematosus, there are four subtypes of this entity that include discrete papular mucinosis, acral mucinosis, cutaneous mucinosis of infancy, and nodular mucinosis.…”

Section: Discussionmentioning

confidence: 99%

“…Papular mucinosis is a relatively uncommon disease characterized by diffuse papular eruption due to mucin deposition in the skin [1][2][3][4][5][6] . This disease most commonly affects middle-aged adults and has been associated with monoclonal gammopathy, systemic lupus erythematosus and discoid lupus, independently [2][3][4][5][6][7][8][9][10][11][12][13][14] .…”

Section: Introductionmentioning

confidence: 99%

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Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

2016

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Papular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition and increased collagen in the skin and internal organs. Its clinical presentation is characterized by dome-shaped, flesh colored papules that are closely spaced or linearly arranged. Papular mucinosis has been individually associated with several entities that include discoid lupus erythematosus, systemic lupus erythematosus and monoclonal gammopathy of undetermined significance. We encountered a 60-year-old woman with papular mucinosis in the setting of three concurrent disorders: discoid lupus erythematous, systemic lupus erythematosus and IgG paraproteinemia. Furthermore, we have reported the first case in North America of papular mucinosis being successfully treated with intralesional hyaluronidase.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

2016

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Cutaneous Mucinoses

Rongioletti

2016

Rook's Textbook of Dermatology, Ninth Edition

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The cutaneous mucinoses are a heterogeneous group of disorders whose main characteristic is abnormal mucin deposition in the skin. The aetiopathogenesis of cutaneous mucinoses is unknown, although cytokines such as tumour necrosis factor α and β, interleukin 1, interleukin 6 and transforming growth factor β and/or polyclonal and monoclonal immunoglobulins and other unidentified factors in the serum of affected patients may induce up‐regulation of glycosaminoglycan synthesis. The cutaneous mucinoses are divided into two categories: (i) primary cutaneous mucinoses in which the mucin deposit is the main histological feature resulting in clinically distinctive lesions; and (ii) secondary mucinoses in which the mucin deposition is only an additional histological epiphenomenon. Primary mucinoses can be divided into dermal and follicular mucinoses. The former includes lichen myxoedematosus, reticular erythematous mucinosis, scleroedema, mucinoses in thyroid disease, papular and nodular mucinosis in connective tissue diseases, self‐healing juvenile cutaneous mucinosis, cutaneous focal mucinosis, digital myxoid cyst; the latter include Pinkus follicular mucinosis and urticaria‐like follicular mucinosis. Associated disorders include paraproteinaemia (scleromyxoedema, scleroedema), diabetes mellitus (scleroedema), hyperthyroidism (pretibial myxoedema), hypothyroidism (generalized myxoedema) and lupus erythematosus, dermatomyositis or scleroderma (papular and nodular mucinosis in connective tissue diseases).

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The Skin and Disorders of the Haematopoietic and Immune Systems

Gniadecki

2016

Rook's Textbook of Dermatology, Ninth Edition

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This chapter reviews skin diseases and symptoms that develop secondarily to underlying malignant or benign diseases of the haematopoietic system. Skin lesions may be caused directly by infiltration of the skin with malignant cells (leukaemia cutis and lymphomatous infiltrates) or by paraproteins secreted by malignant plasma cells (amyloid light‐chain amyloidosis, type I cryoglobulinaemia and macroglobulinaemia cutis). A number of skin signs are an indirect effect of the underlying malignancy, such as paraneoplastic conditions or dermatological syndromes associated with haematological neoplasms (Schnitzler syndrome, POEMS, AESOP and TEMPI syndromes, neurofibromatosis, juvenile xanthogranuloma and juvenile myeloid leukaemia). Kikuchi–Fujimoto disease, Kimura disease, Rosai–Dorfman disease and IgG4‐related disease are idiopathic lymphoadenopathies presenting with characteristic skin lesions. Skin infections, eczematous lesions and vasculitis are the most common cutaneous signs of an underlying immunodeficiency. Chronic skin ulcers may accompany different haemoglobinopathies, such as thalassaemias and sickle cell anaemia. Finally, the skin signs of transfusion reactions (acute allergic reactions, transfusion‐associated graft‐versus‐host disease and post‐transfusion purpura) are reviewed. Diseases of the haematopoietic system often manifest themselves in the skin, and some manifestations are so typical that the dermatologist may be the first physician to detect the underlying blood disorder. More commonly, however, skin symptoms arise in the context of a known haematopoietic disease.

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Scleromyxedema

Cited by 79 publications

References 26 publications

Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

Cutaneous Mucinoses

The Skin and Disorders of the Haematopoietic and Immune Systems

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