Presentation
Speculation regarding an immunologic etiology of the elastic tissue abnormality is presented herein. [ncbi.nlm.nih.gov]
[…] of leprosy. ( 30117713 ) Pawar M...Zawar V 2018 7 Acquired Cutis Laxa Presenting as Pedunculated Eyelid Plaques in an Adult. ( 30454716 ) Law MX...Kalin-Hajdu E 2018 8 Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient [malacards.org]
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]
Entire Body System
- 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]
Biopsies of both axillary and palpebral lesions were performed. [scielo.br]
- 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]
Skin
- 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]
Workup
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]
Treatment
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]
Rhytidectomy was performed as an auxiliary treatment. [rbcp.org.br]
Prognosis
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]
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]
Cardiopulmonary abnormalities are common in type I recessive cutis laxa and are the main factors to determine the prognosis and life expectancy. [panafrican-med-journal.com]
Etiology
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]
Epidemiology
An epidemiologic and therapeutic reassessment of scabies. Cutis 2000; 65:233–240. [wordscope.com]
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]
Pathophysiology
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]
Pathophysiology Degradation of elastic fibers through proteolyitc processes is believed to play a role in acquired cutis laxa. [dermatologyadvisor.com]
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]
Prevention
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. [cutislaxa.org]
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]