Presentation
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 [ncbi.nlm.nih.gov]
APPM is much more commonly seen in women and presents as white to flesh-coloured papules exclusively on the dorsal hands and extensor forearms. [casereports.bmj.com]
Of these reports presentation with plaques and nodule are rare. [ijdvl.com]
We present a case of this type in an 80-year-old African American woman. She was treated with pimecrolimus cream, which resulted in symptomatic relief. [mdedge.com]
Entire Body System
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Swelling
Figure 1 Pink plaques with fine scales located on the metacarpophalangeal and proximal interphalangeal joints with swelling of the proximal digits. [casereports.bmj.com]
Examination revealed multiple nontender firm subcutaneous nodulocystic swelling varying from 5mm to 4cm distributed over face scalp, neck, bilateral upper arms, posterior axillary folds and dorsum of hands and feet. [ijdvl.com]
Table 2 The result of our cases and those previously published are reported CASE REPORTS Case 1 In June 1997 a 74 year old man, a smoker (40 cigarettes/day), complained of a Raynaud's phenomenon, of a painful swelling of the wrists, metacarpophalangeal [ard.bmj.com]
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Pseudotumor
The woman's medical history was significant for gastric cancer status post a partial gastrectomy 7 years prior, pseudotumor cerebri, hypertension, and osteoporosis. She is followed regularly for these conditions, which are stable. [mdedge.com]
Respiratoric
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Pharyngitis
Abstract quote BACKGROUND: Scleromyxedema is a rare chronic fibromucinous disorder that can have devastating clinical manifestations, including sclerosis of the skin with progressive pharyngeal and upper airway involvement, resulting in high mortality [thedoctorsdoctor.com]
Urogenital
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Renal Insufficiency
insufficiency) or ophthalmological (corneal deposits) manifestations that necessitate immediate and aggressive therapeutic intervention (1–3). [medicaljournals.se]
Neurologic
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Confusion
[…] myxoedematosuswhich, possibly due to its asymptomatic discretepresentation, has been described rarely in the literature.As the pathogenetic mechanism of mucin deposition isnot completely understood, the classification of cutaneousmucinoses is still confusing [docslide.com.br]
Treatment
What is the treatment of lichen myxoedematosus? Treatment of scleromyxoedema is difficult and disappointing. [dermnetnz.org]
The eruption disappeared almost completely after 35 treatments at a cumulative dose of 202 J/cm2. Histologically, mucin deposition was greatly diminished after the therapy. [ncbi.nlm.nih.gov]
Treatment Options Treatment options for scleromyxedema are summarized in Table II. Table II. [dermatologyadvisor.com]
HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis. [thedoctorsdoctor.com]
Prognosis
However, when myeloma is present, the patient generally has a poor prognosis. Scleromyxedema usually has a poorer prognosis than that of the other forms. [emedicine.medscape.com]
Systemic symptoms were only reported in SHPM.32,33 In general, localized LM is self-limited and associated with a good prognosis.6 The histologic features of LM are summarized in Table 1.5 The pathology lies within the dermis for all types of this disease [mdedge.com]
Involvement of central nervous system, heart, kidney and the progression to overt myeloma worsens the prognosis. Treatment Options Treatment options for scleromyxedema are summarized in Table II. Table II. [dermatologyadvisor.com]
Etiology
Lichen myxoedematosus is an unusual disorder of unknown etiology and pathogenesis. Although several treatments have reportedly been tried, therapeutic efficacies are variable and unsatisfactory. [ncbi.nlm.nih.gov]
Abstract References Citations Supplementary Data Article Media Metrics Suggestions Lichen myxoedematosus is an unusual disorder of unknown etiology and pathogenesis. [ingentaconnect.com]
A1 - VON ZEZSCHWITZ,K A, PY - 1959/1/1/pubmed PY - 1959/1/1/medline PY - 1959/1/1/entrez KW - LICHEN PLANUS/complications KW - MYXEDEMA/etiology and pathogenesis SP - 301 EP - 16 JF - Archiv fur klinische und experimentelle Dermatologie JO - Arch Klin [unboundmedicine.com]
Successful treatment of lichen myxoedematosus with PUVA photochemotherapy Photodermatology Photoimmunology & Photomedicine 16(5): 229-231, October 2000 Lichen myxoedematosus is an unusual disorder of unknown etiology and pathogenesis. [eurekamag.com]
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.3-5 LM has no relation to endocrine gland disorders, particularly [mdedge.com]
Epidemiology
Epidemiology Race Persons of any race can be affected. Sex No sex-related predilection is reported. Age Most individuals with lichen myxedematosus are aged 30-70 years. [emedicine.medscape.com]
Pathophysiology
Etiology Pathophysiology The etiopathogenesis of scleromyxedema is unknown. The significance of the monoclonal gammopathy is a matter of debate. Paraprotein levels do not correlate with either the extent or the progression of the disease. [dermatologyadvisor.com]
[…] referred to as lichen myxedematosus, papular mucinosis, or lichen fibromucinoidosis, the clinical circumstance of mucin deposited in the skin in the form of papules, nodules or plaques is now generally accepted as the cutaneous expression of the same pathophysiologic [annals.org]
Pathophysiology The etiology is unknown; however, the disease is commonly associated with plasma cell dyscrasia. The basic defect is hypothesized to be a fibroblast disorder, which causes the increased mucin deposition in the skin. [emedicine.medscape.com]
Prevention
[…] intralesionalinjections of hyaluronidase and triamcinolone, with littlesuccess.3,6 Surgical interventions using the carbondioxide laser for dermabrasion have been used to goodeffect, giving a moderate cosmetic improvement, but thistreatment does not prevent [documents.tips]
Current medical research has not established a method of preventing the occurrence of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms. [dovemed.com]
In this Article: Treating Symptoms with Home Remedies Treating Lichen Planus with Medical Care Preventing Outbreaks Community Q A 16 References. [sklxsp.ga]
Recently it has been recently shown that FcRn is a protective receptor that prevents the catabolism of IgG. [ard.bmj.com]