Papular mucinosis, a localized form of lichen myxedematosus, is characterized by papules, nodules and plaques. Unlike scleromyxedema, papular mucinosis does not present monoclonal gammopathy, sclerosis or systemic manifestations. Scleromyxedema form of lichen myxedematosus is not reported in children. Scleromyxedema usually affects adults above thirty years of age. There is no gender predilection in scleromyxedema affliction.
Causes of scleromyxedema and papular mucinosisThe cause of primary lichen myxedematosus disorder is idiopathic and unknown.
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Signs and symptoms of papular mucinosisThe 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. The papules in papular mucinosis contain mucin and on injury the papular wound may exude mucin.
Signs and symptoms of scleromyxedemaThe generalized lichen myxedematosus disorder covers larger parts of the body with scleroderma-like induration and skin stiffening.
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Systemic manifestations of scleromyxedemaScleromyxedema presents systemic manifestations. Systemic involvements of generalized scleromyxedema include neurologic complications, rheumatologic manifestations, respiratory problems, gastrointestinal abnormalities, renal problems and cardiac abnormalities. Some of the systemic manifestations of scleromyxedema are listed below.
- Difficulty in swallowing
- Proximal muscle weakness
- Reduced oxygen intake
- Arthritis of the joints
- Renal failure
- Inflammatory myopathy
- Raynaud syndrome
- Ectropion eyelid
- corneal opacities
- Acute neurologic complications
- Peripheral neuropathy
- Cardiovascular abnormalities
- Myocardial ischemia
- Valvular mucin deposition
- Nasal regurgitation
- Decreased epiglottis mobility
- Decreased vocal cord mobility
- Paraproteinemia - typical symptom of Scleromyxedema
Diagnosis of scleromyxedema and papular mucinosisIn scleromyxedema histologic examination may reveal mucinosis, fibroblast proliferation, and fibrosis.
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Atypical forms of lichen myxedematosus disorder do not meet the criteria for either papular mucinosis or scleromyxedema. Peng Wang et al have described case of an atypical localized lichenoid plaque form in a patient appearing like papular mucinosis. The patient had lichenoid plaque eruptions restricted to neck region. There were no paraproteinemia or thyroid dysfunction in the patient. The patient also suffered from the systemic involvement of kidney with immunoglobulin A nephropathy (IgA nephropathy). There was proliferation of fibroblasts and mucin deposition in the renal interstitium and glomeruli.
Myxedema of thyroid disease, scleroderma (systemic sclerosis), scleredema, and nephrogenic systemic fibrosis may have to be ruled out by differential diagnosis lichen myxedematosus.
Treatment options for lichen myxedematosusTreatment of both mucinosis and scleromyxedema include use of topical tacrolimus, retinoids, isotretinoin, thalidomide, corticosteroids, high-dose dexamethasone, methotrexate, psoralen UV-A (PUVA), plasmapheresis, UV phototherapy, intravenous immunoglobulin, dermabrasion, carbon dioxide laser excision, electrocoagulation, autologous stem cell transplantation and lenalidomide. These treatments shows various degrees of success.
M. Dolenc-Voljc et al reported successfully treating scleromyxedema having subcutaneous nodules with thalidomide and intravenous immunoglobulin. Biopsies of the scleromyxedema lesions showed fibroblasts proliferation and mucinosis between collagen bundles. Treatment with thalidomide and intravenous immunoglobulin lead to complete scleromyxedema remission. Dilip K Sa et al reported a case of localized papular mucinosis. Treatment with oral cyclophosphamide showed excellent response and papular mucinosis lesions had disappeared.
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1.M. Dolenc-Voljc, V. Jurcic, A. Hocevar, M. Tomsic. Scleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin. Case Rep Dermatol. 2013;Sep-Dec; 5(3): 309–315.
2.Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. 2001; Feb;44(2):273-81.
3.Dilip K, Aparajita Ghosh, Piyush Kumar, Ramesh C Gharami. A Case of Localized Papular Mucinosis Showing Excellent Response to Cyclophosphamide. Indian J Dermatol. 2014 Mar-Apr; 59(2): 202–204. doi: 10.4103/0019-5154.127697.
Current topic in the dynamic natural skin care: What is Papular mucinosis - Lichen myxedematosus - Scleromyxedema.