Symptoma

Acquired Cutis Laxa

Cutis Laxa Acquisita

Speculation regarding an immunologic etiology of the elastic tissue abnormality is presented herein. [ncbi.nlm.nih.gov]

  • Axillary Lesion

    A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. [ncbi.nlm.nih.gov]

  • Swelling of the Finger

    The skin laxity was preceded by swelling of the fingers and toes and by the appearance of papular urticaria. Dapsone therapy was effective in controlling the swelling. [ncbi.nlm.nih.gov]

  • Plethora

    This case presents a plethora of cutaneous findings (primary systemic amyloidosis, acquired cutis laxa and mucinosis), all linked to MM. There is not, at present, another description of all these findings on the same patient. REFERENCES 1. [scielo.br]

  • Constitutional Symptom

    There were no constitutional symptoms and the urticaria was not associated with any specific food or drug intake. [ijdvl.com]

  • Pathologist

    Beyond its usefulness to general pathologists, dermatopathologists, and hematopathologists, this information is intended to be helpful for dermatologists, hematologists/oncologists, fellows, and residents. [books.google.com]

  • Insect Bite

    bites, or drug intake, such as penicillin or penicillamine. [jle.com]

    bite, following drug intake or a malignancy. [e-ijd.org]

    Type 1 acquired cutis laxa can be either generalized or localized may occur at any age, though the usual onset is in adulthood, usually follows episodes of inflammatory dermatosis, hypersensitivity reaction to insect bite or drugs such as penicillin, [ijpd.in]

    bites and related to an underlying hematologic disorder (ie, multiple myeloma, lymphoma, immunoglobulin heavy-chain deposition disease, plasma cell dyscrasia). 1 - 4 Acquired cutis laxa has also been reported in patients following cutaneous mastocytosis [jamanetwork.com]

  • Erythema of the Chest

    The primary clinical manifestation was an erythema of the chest. Gradually there developed persistent circumscribed lesions in other areas of the skin, spreading into large erythematous plaques with loose skin. [ncbi.nlm.nih.gov]

  • Paresthesia

    In the last four years, the patient reports pain and paresthesia in a stocking-like pattern affecting the lower limbs. The patient denied any familial history of cutaneous lesions. [scielo.br]

Evaluation for an underlying gammopathy is essential for the workup of a patient with new-onset ACL. [ncbi.nlm.nih.gov]

Figure 1: Preoperative picture showing lax and pendulous skin in infraorbital region Click here to view On further workup of the patient, hemogram, serum electrophoresis, and erythrocyte sedimentation rate were found within normal range. [e-ijd.org]

Evaluation for an underlying gammopathy is essential for the workup of a patient with new-onset ACL. Cutis laxa (CL) is a rare dermatosis resulting in loose, wrinkled, redundant skin secondary to defects in dermal elastic tissue. [jamanetwork.com]

A full workup for multiple myeloma (MM) was then performed, and he was noted to have immune globulins within normal limits except for IgG of 648 mg/dl, free lambda elevated to 27.85 mg/dl with a decreased kappa lambda ratio to 0.02 and serum and urine [elsevier.es]

The etiology, prevention and treatment options are discussed. [ncbi.nlm.nih.gov]

Following one year of treatment, the anasarca subsided leaving behind loose wrinkled skin all over the body. [ijdvl.com]

Reversal of cutis laxa with treatment of a systemic association has not yet been reported. [dermatologyadvisor.com]

Prognosis The prognosis for cutis laxa varies with the form of the disorder. The effects may be relatively mild with individuals living a fairly normal, full life, or the disease may be fatal. [dxline.info]

Early onset is associated with better prognosis, and the lesions are more likely to fill out with growth. [5] Majority of the acquired cases come under the generalized category (Type I), which usually begins in adulthood and follows an urticarial reaction [e-ijd.org]

Affected individuals should talk to their physician and medical team about their specific case, associated symptoms and overall prognosis. [rarediseases.org]

OTHER INFORMATION PROGNOSIS The prognosis for Cutis Laxa varies with the form of the disorder. The effects may be relatively mild with individuals living a fairly normal, full life, or the disease may be fatal. [cutislaxa.org]

Patients with CREST syndrome have a better prognosis. Differential diagnosis includes diabetic sclerodema, scleromyxedema, and chronic graft-versus-host disease. [clevelandclinicmeded.com]

The etiology, prevention and treatment options are discussed. [ncbi.nlm.nih.gov]

While the etiology of tracheobronchiomegaly is unknown, the coexistence of two rare conditions in the same patient suggtsts a common fundamental elastic tissue defect. [pediatrics.aappublications.org]

The heterogeneous set of conditions, diversity among presentations and severity explains the lack of epidemiological data and clinical features. [elsevier.es]

Epidemiology Frequency Cutis laxa (elastolysis) is rare. Congenital forms of cutis laxa (elastolysis) are more common than acquired disease. The recessively inherited form is most frequent and most severe. Autosomal dominant forms also exist. [emedicine.medscape.com]

This article describes several cases of intoxication, gives regional epidemiological data on this phenomenon, and includes current nomenclature. [biomedsearch.com]

Epidemiological studies have shown that CD is very common and affects about 1 in 250 people[ 2 ]. CD is a multigenic disorder associated with HLA-DQ2 (DQA1*/DQB1*2) expressed in more than 90% of patients, or HLA-DQ8 (DQA1*0301/DQB1*0302)[ 3 ]. [wjgnet.com]

Pathophysiology Degradation of elastic fibers through proteolyitc processes is believed to play a role in acquired cutis laxa. [dermatologyadvisor.com]

The collection will showcase studies on the dysregulation of pathways, disease progression, approaches to treat and modify disease course in animal models, technologies for studying muscle pathophysiology and regeneration, and advances in the development [dmm.biologists.org]

Its pathophysiology is not well understood. Hypotheses include abnormal copper metabolism or copper deficiency, decreased serum elastase inhibitor (α 1-antitrypsin), low lysyl oxidase activity, and immune-mediated mechanisms. [pubs.sciepub.com]

Pathophysiology Cutis laxa (elastolysis) is characterized by degenerative changes in the elastic fibers resulting in loose, pendulous skin. The skin is sagging, redundant, and stretchable, with reduced elastic recoil. [emedicine.medscape.com]

The etiology, prevention and treatment options are discussed. [ncbi.nlm.nih.gov]

However, there is no specific medicine that can completely cure or prevent the symptoms related with Cutis Laxa. Prevention of Cutis Laxa You cannot prevent Cutis Laxa as it is a genetic disease. [lazoi.com]

However, there is no specific medicine that can completely cure or prevent the symptoms related with Cutis Laxa. 6. You cannot prevent Cutis Laxa as it is a genetic disease. [slideshare.net]

Prevention The inherited forms of cutis laxa are genetically determined and are not currently preventable. Genetic counseling can be helpful for anyone with a family history of cutis laxa. [dxline.info]

PREVENTION The inherited forms of cutis laxa are genetically determined and are not currently preventable. Genetic counseling can be helpful for anyone with a family history of cutis laxa. [cutislaxa.org]