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. [scielo.br]

Case presentation Our patient, a 45-year-old Chinese lady, was healthy up until February 2017, when she presented to a clinic in her hometown with generalized, pruritic, mildly painful, erythematous macules and wheals all over her body. [aacijournal.biomedcentral.com]

• Fever

  Typical features include spiky fever, sudden eruption of raised painful erythematous to plum-colored nodules and plaques, neutrophilic leucocytosis, a dermal neutrophili infiltrate an a rapid response to systemic steroid. [e-ijd.org]

  Case Report A 4-year-old boy presented with a history of red raised painful skin lesions on the abdomen, chest, and extremities since two months associated with fever. [ijpd.in]

  In children or adolescents, cutis laxa usually develops after a severe illness involving fever, polyserositis, or erythema multiforme. [msdmanuals.com]

  Cutis laxa associated with fever and neutrophilic dermatosis is called Marshall syndrome 5. [rbcp.org.br]

  Acquired cutis laxa tends to follow (and may be caused by) severe illness characterized by fever, inflammation, and a severe skin rash ( erythema multiforme ); an injury to the nerves that control blood vessel dilation and contraction; or an autoimmune [dxline.info]

• Developmental Delay

  She had no history of developmental delay or any joint laxity as described for Type II recessive CL; our patient probably suffered from the Type I recessive form. [idoj.in]

  Most affected children have malformations of certain areas of the brain (cortical and cerebellar malformations) that result in delays in reaching developmental milestones (developmental delays) and intellectual disability. [rarediseases.org]

  Unlike in type 1, there is often pronounced developmental delay, seizures and neurological impairment Autosomal dominant: lax skin and a prematurely aged appearance with onset between childhood and early adulthood. [pcds.org.uk]

  Other symptoms associated with cutis laxa include: developmental delays eyes that are further apart than is typical feeding difficulties in infants fragile bones lax or loose joints low-set ears or ears that aren’t properly formed poor muscle tone short [healthline.com]

  Many individuals with this condition have severe developmental delay and seizures. [cutislaxa.org]

• Congestive Heart Failure

  The list includes: Inflammatory skin diseases Systemic lupus Rheumatoid arthritis Hematologic diseases Gastrointestinal system dysfunctions Rectal and vaginal prolapse Pulmonary fibrosis Hernias Congestive heart failure Aortic aneurysms Celiac diseases [dermanities.com]

• Abdominal Pain

  In the subsequent 3 years, she had frequent lower abdominal pain and runny stools that often occurred simultaneously with the red-brown eruptions. She also noticed wrinkle lines on her face. [materials.uscap.org]

  Primarily characterized by diarrhea and abdominal pain, other symptoms such as flatulence, nausea, vomiting, fatigue, malaise, and weight loss occur. [biomedsearch.com]

  An otherwise healthy male child of 9 years presented with paroxysmal fever and diffuse abdominal pain along with the loss of appetite and nausea lasting for 3-4 days every 4-6 weeks in the last two years. [pubfacts.com]

  The classic form of CD presents several symptoms such as diarrhea, abdominal pain, weight loss and nutritional deficiencies, particularly of iron, folate, calcium, and vitamin D[ 7 ]. [wjgnet.com]

• Heart Failure

  The list includes: Inflammatory skin diseases Systemic lupus Rheumatoid arthritis Hematologic diseases Gastrointestinal system dysfunctions Rectal and vaginal prolapse Pulmonary fibrosis Hernias Congestive heart failure Aortic aneurysms Celiac diseases [dermanities.com]

  Pulmonary emphysema, cor pulmonale, and right-sided heart failure caused by pulmonary disease have been commonly described. [idoj.in]

  Pulmonary emphysema, and right-sided heart failure caused by pulmonary disease have been commonly described. [panafrican-med-journal.com]

  In severe cases, the various vascular and cardiac abnormalities can progress to cause heart failure. [rarediseases.org]

• Muscle Weakness

  OHS is often accompanied by muscle weakness and may include mental retardation. Affect only males. Autosomal Dominant Cutis Laxa (ADCL) Symptoms usually occur anytime between birth and early adulthood. [dermanities.com]

  Symptoms include: cutis laxa stomach issues such as hernias muscle weakness OHS is associated with milder cutis laxa symptoms. Autosomal Dominant Cutis Laxa (ADCL) ADCL symptoms can start any time from birth to young adulthood. [healthline.com]

  She was hospitalized in February 2012 for fever, arthralgia, pelvic and shoulder muscle weakness with myalgia, malar rash, thrombocytopenia, leucopenia and lymphocytopenia Immunological tests showed Antinuclear Antibodies (ANA) (+) to 1/640, AC anti-DNA [sciepub.com]

  There can also be muscle weakness, and intelligence ranges from low normal to mild mental retardation. OHS is a disorder of copper metabolism caused by mutations (changes) in the ATP7A gene. [cutislaxa.org]

  In February 2012, she was admitted in the internal medicine department for fever, arthralgia, proximal muscle weakness, diffuse myalgia, eyelid erythema, a "butterfly" rash. [pubs.sciepub.com]

• Fracture

  Osteoporosis may also present, resulting in fragile bones that are prone to fracture. More severe complications such as cardiovascular or pulmonary symptoms rarely develop. [rarediseases.org]

  Pseudoxanthoma elasticum with vitamin K dependent coagulation factor deficiency displays severe skin laxity yet histopathologic examination will reveal calcium deposits around fractured elastic fibers in the dermis. [dermatologyadvisor.com]

  Other features include a small jaw, hip dislocations, hernias, osteoporosis, fractures, and dwarfism. As with ARCL3, there are typically neither cardiovascular nor pulmonary symptoms. [cutislaxa.org]

  CT scans revealed punctate lytic lesions at the vertebral bone and nondisplaced fractures in the fourth and fifth ribs. [elsevier.es]

• Hip Dislocation

  […] birth Emphysema early in life Type II Growth retardation Usually present at birth Severe over hands, feet and abdomen Bilateral congenital dislocation of hips The features of acquired cutis laxa are mainly loose skin, especially in areas of previous [dermnetnz.org]

  In addition to the loose skin, this form of the disease is characterized by bone abnormalities, the delayed joining of the cranial (skull) bones, hip dislocation, curvature of the spine, flat feet, and excessive tooth decay. [dxline.info]

  Other features include a small jaw, hip dislocations, hernias, osteoporosis, fractures, and dwarfism. As with ARCL3, there are typically neither cardiovascular nor pulmonary symptoms. [cutislaxa.org]

  […] of the hip. [rarediseases.org]

• Hyperlaxity

  […] involving dermal elastic fibers. ( 25133950 ) O'Malley J.T....Grossman M.E. 2014 18 Acquired cutis laxa of face with multiple myeloma. ( 25201849 ) Yadav T.A....Khopkar U.S. 2014 19 Acral Acquired Cutis Laxa Associated with IgA Multiple Myeloma, Joint Hyperlaxity [malacards.org]

  Bessis, “Acral acquired cutis laxa associated with IgA multiple myeloma, joint hyperlaxity and urticarial neutrophilic dermatosis,” Acta Dermato Venereologica, vol. 94, no. 6, pp. 743-744, 2014. View at: Publisher Site | Google Scholar F. G. [hindawi.com]

• Macrocephaly

  MACS Syndrome This condition causes both males and females to have a larger-than-usual head, which is called macrocephaly. [healthline.com]

  MACS Syndrome MACS Syndrome is macrocephaly (large head), alopecia (sparse hair), cutis laxa, and scoliosis. Other features include puffy eyelids, flat feet, loose joints, and short stature. [cutislaxa.org]

  Symptoms include an abnormally large head (macrocephaly), partial or complete hair loss (alopecia) and the skin symptoms of cutis laxa. Cutis laxa is mild with redundant skin on the face the most common manifestation. [rarediseases.org]

• Eruptions

  The eruption is believed to represent a hypersensitivity reaction to an antecedent infection or malignancy. There have been rare reports of acquired cutis laxa (Marshall’s syndrome) following Sweet’s syndrome. [e-ijd.org]

  We report a 35-year-old man with generalized acquired cutis laxa (GACL) associated with a persistent papular erythematous eruption that histopathologically showed some resemblance to dermatitis herpetiformis. [ncbi.nlm.nih.gov]

  The first eruption resolved within 24 hours. However, in the ensuing months, she had several similar eruptions that failed to resolve. [materials.uscap.org]

  The relationship between acquired cutis laxa and urticaria-like eruption is still not clear. [aacijournal.biomedcentral.com]

• Skin Lesion

  We report an 8-month-old infant in whom Sweet's syndrome was diagnosed and who developed acquired cutis laxa in the skin lesions. [ncbi.nlm.nih.gov]

  A bacterial infection of upper respiratory tract infection, tonsillitis, or intestinal infection may precede skin lesions by 1–3 weeks. [ijpd.in]

  CD is often associated with DH which has some features in common with skin lesions of porphyrias. [wjgnet.com]

  She saw a physician at the time, who described the skin lesions as heterogeneous red-brown patches and plaques. The first eruption resolved within 24 hours. However, in the ensuing months, she had several similar eruptions that failed to resolve. [materials.uscap.org]

  Type 2 usually develops following acute inflammatory skin lesions, in which the areas affected by the skin lesions develop cutis laxa skin symptoms. [rarediseases.org]

• Alopecia

  Alopecia areata Alopecia areata (AA) is a chronic autoimmune disease characterized by non-scarring alopecia. Histologically it is possible to find perifollicular lymphocyte infiltration, pointing to an immunologic etiology such as CD. [wjgnet.com]

  Voir sur Pubmed Successful Hair Transplantation for Scarring Alopecia Associated With Incontinentia Pigmenti. Park M et al. Park M et al. Successful Hair Transplantation for Scarring Alopecia Associated With Incontinentia Pigmenti. [genodermatoses-network.org]

  MACS Syndrome MACS Syndrome is macrocephaly (large head), alopecia (sparse hair), cutis laxa, and scoliosis. Other features include puffy eyelids, flat feet, loose joints, and short stature. [cutislaxa.org]

  Symptoms include an abnormally large head (macrocephaly), partial or complete hair loss (alopecia) and the skin symptoms of cutis laxa. Cutis laxa is mild with redundant skin on the face the most common manifestation. [rarediseases.org]

  Epidermolysis Bullosa Acquisita Epidermolysis bullosa acquisita ( Fig. 9 ) is an uncommon bullous disease characterized by skin fragility, milia (small cysts), scarring alopecia, and nail dystrophy. [clevelandclinicmeded.com]

• Sparse Hair

  Additional symptoms include: short stature sparse hair cutis laxa Acquired Cutis Laxa Some people have symptoms, but don’t have the genetic changes associated with cutis laxa. This type of cutis laxa is known as acquired cutis laxa. [healthline.com]

  MACS Syndrome MACS Syndrome is macrocephaly (large head), alopecia (sparse hair), cutis laxa, and scoliosis. Other features include puffy eyelids, flat feet, loose joints, and short stature. [cutislaxa.org]

  Table 4 Clinical characteristics of ADCL and type 1 recessive cutis laxa syndromes Dysmorphism Long philtrum, beaked nose Long philtrum, large ears, beaked nose Long philtrum, large ears, beaked nose, sparse hair on temporal sides Skin Loose redundant [ojrd.biomedcentral.com]

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]

Rhytidectomy was performed as an auxiliary treatment. [rbcp.org.br]

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 CL and are the main factors to determine the prognosis and life expectancy. [idoj.in]

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]

An epidemiologic and therapeutic reassessment of scabies. Cutis 2000; 65:233–240. [wordscope.com]

The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. [books.google.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]

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]

Infant death in keratitis-ichthyosis-deafness (KID) syndrome is recognized; its association with specific genotypes and pathophysiology is inadequately understood. [genodermatoses-network.org]

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]