cutis laxa is a rare connective tissue disorder , which may be inherited or acquired , and is characterized by progressive looseness of skin , associated with abnormalities of other organs and structures such as lung , vasculature or gastrointestinal tract as they contain elastic tissues .
the hereditary form of cutis laxa may be inherited as either autosomal dominant , x - linked recessive or autosomal recessive trait .
the acquired form is even rarer than the inherited form and is often associated with some form of preceding or accompanying cutaneous eruptions like eczema , urticaria , erythema multiforme , sweet 's syndrome or multiple myeloma .
the clinical appearance is characterized by inelastic , loose and pendulous skin that produces a wrinkled , prematurely aged appearance .
the primary change is in the elastic fiber , which is decreased or nearly absent in the dermis . here
an itchy eruption had developed on the anterior neck 5 years ago , and it lasted for about a month .
several months later , she noticed that the skin of that area was becoming wrinkled and loose .
the wrinkling and looseness progressed for 4 to 5 months after which it became stable .
she was otherwise well and had no history of dyspnoea , chest pain , palpitation or visual problems .
family history of similar skin lesion was absent . physical examination revealed a circumscribed palm sized area of lax , extensible , wrinkled skin localized to the anterior part of the neck [ figure 1a ] .
lesion on the neck of a 14-year - old female lesion on the neck showing stretching of the lax skin a 40-year - old muslim male presented to us with a complaint of loose skin all over his body for last 10 years .
he was well till the age of 30 , when he noticed that his skin was gradually becoming wrinkled and loose .
it started from forehead and progressed slowly downwards over a couple of years after that the process stabilized . at the time of presentation
the skin of his forehead [ figure 2a ] , neck , abdomen and back [ figure 2b ] was diffusely wrinkled and it hung in folds .
lesion on the scalp of a 40-year - old male lesion on the back of a 40-year - old male in both the cases investigations revealed normal values for complete blood count , erythrocyte sedimentation rate , routine urine analysis , blood biochemistry , hepatic enzymes and serum electrophoresis .
biopsy specimens taken from the involved area were stained with hematoxylin - eosin ( h and e ) , von kossa and verhoeff - van gieson stain .
h and e staining revealed normal epidermis with diminished elastic fibres and mild non - specific chronic lymphohistiocytic infiltrate in upper dermis [ figure 3a ] .
the elastic fibres present were fragmented , stained uneven and showed granular appearance , as evidenced in verhoeff - van gieson stain [ figure 3b ] .
histopathology of skin lesion of case 1 showing normal epidermis with diminished elastic fibres and mild non - specific chronic lymphohistiocytic infiltrate in upper dermis ( h and e , original magnification 10 ) histopathology of skin lesion of case 2 showing fragmented and unevenly stained elastic fibres with granular appearance , ( verhoeff - van gieson stain , original magnification 10 )
an itchy eruption had developed on the anterior neck 5 years ago , and it lasted for about a month .
several months later , she noticed that the skin of that area was becoming wrinkled and loose .
the wrinkling and looseness progressed for 4 to 5 months after which it became stable .
she was otherwise well and had no history of dyspnoea , chest pain , palpitation or visual problems .
family history of similar skin lesion was absent . physical examination revealed a circumscribed palm sized area of lax , extensible , wrinkled skin localized to the anterior part of the neck [ figure 1a ] .
lesion on the neck of a 14-year - old female lesion on the neck showing stretching of the lax skin
a 40-year - old muslim male presented to us with a complaint of loose skin all over his body for last 10 years .
he was well till the age of 30 , when he noticed that his skin was gradually becoming wrinkled and loose .
it started from forehead and progressed slowly downwards over a couple of years after that the process stabilized . at the time of presentation
the skin of his forehead [ figure 2a ] , neck , abdomen and back [ figure 2b ] was diffusely wrinkled and it hung in folds .
lesion on the scalp of a 40-year - old male lesion on the back of a 40-year - old male in both the cases investigations revealed normal values for complete blood count , erythrocyte sedimentation rate , routine urine analysis , blood biochemistry , hepatic enzymes and serum electrophoresis .
biopsy specimens taken from the involved area were stained with hematoxylin - eosin ( h and e ) , von kossa and verhoeff - van gieson stain .
h and e staining revealed normal epidermis with diminished elastic fibres and mild non - specific chronic lymphohistiocytic infiltrate in upper dermis [ figure 3a ] .
the elastic fibres present were fragmented , stained uneven and showed granular appearance , as evidenced in verhoeff - van gieson stain [ figure 3b ] .
histopathology of skin lesion of case 1 showing normal epidermis with diminished elastic fibres and mild non - specific chronic lymphohistiocytic infiltrate in upper dermis ( h and e , original magnification 10 ) histopathology of skin lesion of case 2 showing fragmented and unevenly stained elastic fibres with granular appearance , ( verhoeff - van gieson stain , original magnification 10 )
acquired cutis laxa usually begins in adulthood with insidious development of loose skin often starting at face and neck .
the first case with localized lesion described above could be differentiated from pseudo xanthoma elasticum ( pxe ) by the absence of the characteristic yellow color of pxe and lack of calcification with von kossa stain .
the second case could be differentiated from elher danlos syndrome , as the skin was lax and recoiled slowly , and there was no joint involvement .
the girl ( case 1 ) was referred to a reconstructive surgeon for treatment , and after successful excision of the loose skin only a fine linear scar was visible . but not much could be done for the older patient ( case 2 ) .
he was advised to take one vitamin e capsule ( 400 mg ) daily for 2 months .
there was not much improvement after 2 months and the patient was lost to follow up .
this option may be a less invasive way to improve facial defects in these patients .
the cause of acquired cutis laxa is largely unknown , though various hypotheses such as altered copper metabolism , excess elastase , and immune - mediated mechanisms have been proposed .
is that elastolysis may result from release of granulocytic elastase because neutrophils contain large amounts of elastase , and is also found in macrophages and platelets .
up to 100-fold increase in the serum elastase level has been demonstrated in a patient with autosomal recessive cutis laxa .
a suggested mechanism of cutis laxa is that , unknown causes stimulate mast cells to release mediators of inflammation , such as histamine , leucotrienes , neutrophil and eosinophil chemotactic factors .
consequently , there is a progressive infiltration of neutrophils and eosinophils around the blood vessels in the papillary dermis and between the collagen fibers in the mid- and reticular dermis .
these neutrophils release elastase , and elastolysis first begins in areas with neutrophilic infiltration around the blood vessels and between collagen fibers , and then it gradually spreads to the entire dermis .
these changes could be localized , as in the first case or they could be generalized leading to universal involvement , as in the second case .
 cutis laxa is a rare disease that may be either inherited or acquired . 
 the acquired form is rarer than the inherited form . 
 pathogenesis of this disease is largely unknown . 
 two cases of acquired cutis laxa are reported here and neither of them had any systemic involvement or any history of drug intake . 
 one of them had localized disease with history of preceding cutaneous inflammation . 
 the other patient with generalized lesion lacked any history of preceding illness . 
 the patient with localized lesion was treated satisfactorily by reconstructive surgery . 
 the other patient had generalized involvement , for which no satisfactory treatment could be offered . 
Introduction
Case Reports
Case 1
Case 2
Discussion