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
- 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]
- 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]
Gastrointestinal
- Regurgitation
Chest X-ray showed cardio-megaly and mild heart failure, and electrocardiographyshowed sinus rhythm with a left bundle branch block.A transthoracic echocardiogram showed mild con-centric left ventricular hypertrophy with mild aorticand mitral valve regurgitation [documents.tips]
CT scan finding is normal and postmortem examination reveals focal demyelinization, gliosis, and cerebral edema only occasionally Mucin deposition in the brain has never been reported ESOPHAGUS Dysphagia and nasal regurgitation are reported by 31.6% of [thedoctorsdoctor.com]
Neurologic
- 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]
Urogenital
- Renal Insufficiency
(renal insufficiency) or ophthalmological (corneal deposits) manifestations that necessitate immediate and aggressive therapeutic intervention (1–3). [medicaljournals.se]
Workup
Hypertrophy
- Ventricular Hypertrophy
Chest X-ray showed cardio-megaly and mild heart failure, and electrocardiographyshowed sinus rhythm with a left bundle branch block.A transthoracic echocardiogram showed mild con-centric left ventricular hypertrophy with mild aorticand mitral valve regurgitation [documents.tips]
Treatment
What is the treatment of lichen myxoedematosus? Treatment of scleromyxoedema is difficult and disappointing. [dermnetnz.org]
Treatment Options Treatment options for scleromyxedema are summarized in Table II. Table II. [dermatologyadvisor.com]
Medicalhistory revealed treatment of a psychological depressionby a variety of medications for about 20 years. [docslide.com.br]
HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis. [thedoctorsdoctor.com]
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]
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]
WITH PARAPROTEINEMIA Assosiated with: peripheral neuropathy, arhtropathy, carpel tunnel syndrome poor prognosis : Often due to Bronchopneumonia Coronary occlusion Hematologic abnormalities 12. [slideshare.net]
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]
DISCUSSION Scleromyxedema or lichen myxedematosus (LM) is a rare type of papular mucinosis, with a chronic and progressive course, chronic, progressive, of unknown etiology. [scielo.br]
Figure 4: 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 [docksci.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
[…] 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]
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]
Prevention
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]
[…] 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]
Recently it has been recently shown that FcRn is a protective receptor that prevents the catabolism of IgG. [ard.bmj.com]