Localized acquired cutis laxa secondary to interstitial granulomatous dermatitis

We report the case of a 68-year-old woman who had interstitial granulomatous dermatitis associated with seronegative polyarthritis. Two years later, this had evolved to become localized acquired cutis laxa.     

Acrolocalized acquired cutis laxa

Summary A 55-year-old man presented with a 14-year history of loose, redundant skin on the palmar aspects of the finger tips and toe pulps. which gave a peculiar ‘chewing gum’ appearance. Skin biopsies of involved areas showed a normal appearance, and only a discrete decrease in the elastic fibres could be identified with an elastic tissue stain. Ultrastructural examination, however, demonstrated marked fragmentation and degeneration of the elastic tissue. Localized cutis laxa in an acral distribution, was fully developed at presentation. He gave a history of repeated episodes of swelling and urticaria of the involved areas, which we regard as being the initial inflammatory stages of this disease process.     

Acral localized acquired cutis laxa associated with rheumatoid arthritis.

We report the first case of the acral localization of the acquired form of cutis laxa associated with severe rheumatoid arthritis. The skin laxity was preceded by episodes of itching and swelling of the hands and feet. Histopathology showed that the elastic fibers were lost in the areas of cutis laxa and decreased in adjacent skin. The pathogenetic relationship with rheumatoid arthritis or the intake of related drugs is discussed.     

Post febrile acquired cutis laxa

Acquired cutis laxa following enteric fever has been described in a male in the neck region. Biopsy revealed fragmented elastic fibres in the dermis which were better visualised with special stain for elastic tissue. This case is reported for rarity of its occurrence at the localised site following febrile illness.     

Acral localized acquired cutis laxa

We report the first case of acral localized acquired cutis laxa. 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. Examination of skin biopsy specimens showed fragmentation and almost total loss of elastic fibers in the areas of cutis laxa. Electron microscopy showed no abnormalities in elastic structure and function in unaffected skin. In addition, electron microscopic examination of an urticarial lesion showed a neutrophilic dermatosis with polymorphonuclear leukocytes attached to the surface of either normal elastic fibers or fibers showing early degenerative changes. These findings suggest that there is no primary defect in the elastic fibers and that the polymorphonuclear leukocytes play a significant role in the destruction of the elastic fibers and the subsequent development of cutis laxa in this case.     

获得性皮肤松弛症一例

患儿男,12岁,面部、躯干、上肢风团1年余,面部、双耳皮肤松弛1年,无自觉症状。患儿于1年前无明显诱因面部、躯干、上肢出现淡红色米粒到1分硬币大小的风团,无痛、痒,2 ~ 3 d可自行消退,消退后无色素沉着,无发热及关节痛……     

获得性皮肤松弛症一例

患者男,49岁.因颈部、腋下、腹股沟皮肤松弛2年半来我院就诊.患者于2年半前无明显诱因出现颈部皮肤松弛下垂,无明显自觉症状,皮损渐加重并累及双侧腋下、腹股沟皮肤.     

获得性皮肤松弛症

报告1例获得性皮肤松弛症。患者女,17岁。面颈、双腋窝及股部皮肤松弛2年。皮肤科检查:面颈、双腋窝及股部皮肤明显松弛,皱纹密集,皮沟加深,无弹性。左颈部皮损组织病理示:表皮厚度大致正常,网篮状角化,真皮浅层血管周围少量淋巴细胞浸润。弹性纤维染色示:真皮浅层弹性纤维数量明显减少、形态异常。诊断:获得性皮肤松弛症。     

Acquired cutis laxa associated with multiple myeloma

Cutis laxa is an uncommon condition characterized by loose and redundant skin. Biopsy results are positive for a reduction in or an absence of elastic fibers in the dermis. Cutis laxa is acquired or congenital. The acquired form is either a generalized insidious form (type I) or a form associated with prior inflammation (type II). Cardiovascular, pulmonary, gastrointestinal, and urologic complications may occur. In the past, cutis laxa was associated with plasma cell dyscrasia. We report on a characteristic cause of cutis laxa to alert clinicians to this uncommon manifestation of multiple myeloma.     

获得性局限性皮肤松弛症

报告1例获得性局限性皮肤松他症。患者男,31岁。有臀部局部皮肤明显松弛、肤色加深2年,无任何自觉症状。皮损组织病理检查示弹性纤维明显减少,形态异常,有断裂。给予整形手术治疗。     

Elastic tissue damage in cephalic acquired cutis laxa

Cutis laxa is a rare condition characterized by diminished elastic tissue. We report the histological changes from a 22-year-old male patient, who presented since 4 years hanging and inelastic facial skin, giving him an older appearance. The palpebral, preauricular and submandibular skin obtained after a facelift were examined. Conventional light microscopy with hematoxylin-eosin staining showed perivascular lymphocytic infiltrates and crossed-over collagen fibers. With Weigert staining, a lessening of elastic fibers with fragmentation, shortening and clumping was seen. The oxytalanic fibers in the papillary dermis were also affected, ranging from absence or reduction to flattening with clumping of the fibers, giving an irregular contour to the basal membrane zone. These findings were more intense in the palpebral skin. Transmission electronic microscopy showed reduction of the elastic fibers, a granular degeneration of the elastic tissue was found and collagen fibers were normal. Similar to light microscopy, at the ultrastructural level, the basal membrane has an irregular contour.     

Increased fibroblast elastase activity in acquired cutis laxa.

BACKGROUND: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. OBSERVATION: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. CONCLUSION: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.