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 .