Acquired localized cutis laxa confined to the face: case report and review of the literature

BACKGROUND: Cutis laxa is an uncommon entity characterized by laxity of the skin, which hangs in loose folds, producing the appearance of premature aging. It can be subdivided into congenital and acquired. This latter variant is rare and the skin involvement varies from generalized to localized. We report a case of a localized acquired cutis laxa confined to the face, without preceding inflammatory lesions or systemic compromise. Four similar cases have been reported to date. The etiology remains unknown and there is no definitive treatment. METHODS: A 27-year-old White woman came to our hospital with a wrinkled face, pendulous earlobes and drop eyelids. Changes began 5 years prior, and she appeared much older than her age. RESULTS: Histological analysis and ultrastructural examination of skin biopsy revealed reduction and fragmentation of elastic fibers, confirming the diagnosis of cutis laxa. No systemic involvement was diagnosed. The patient was submitted to plastic surgery for repair, with satisfactory results to date. CONCLUSIONS: Acquired localized cutis laxa confined to the face without preceding inflammatory lesions is extremely rare. The etiology remains unknown. Clinical features and histopathologic findings confirm the diagnosis. Surgical repair seems to be the only therapeutic choice, but the results are variable and temporary.     

获得性皮肤松弛症

患者男,21岁。 主诉：面部皮肤松弛、下垂4年。     

获得性局限性皮肤松弛症一例

患者,女，24岁。面颈部皮肤逐渐松弛2年。皮肤科查体：面颈部、双上眼睑皮肤松弛下垂，呈早老外观。疲劳试验（-）。皮损组织病理学检查示真皮层弹性纤维减少，形态异常，呈扭曲状。诊断：获得性局限性皮肤松弛症。     

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.     

Acquired cutis laxa and multiple myeloma

We report a patient with acquired cutis laxa and multiple myeloma, and the possible association between the two conditions is discussed.     

Acquired cutis laxa concomitant with nephrotic syndrome

A 41-year-old woman developed laxity and wrinkling of the skin. This process spread slowly to involve the skin of almost the entire body, without any previous inflammatory skin disorder. The skin of her face, neck, and trunk hung in loose folds, leading to a prematurely aged appearance. One year later she noticed edema on her face and legs. Laboratory studies disclosed low C3 and CH50 serum levels and proteinuria. Skin biopsy specimens revealed extensive loss and fragmentation of dermal elastic fibers. A renal biopsy specimen showed membranoproliferative glomerulonephritis (type 2), and an immunofluorescent study disclosed C3 and IgG deposition in the mesangial matrix and along the glomerular basement membrane. We propose that, in this case, cutis laxa may be related to an abnormal immune response.     

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。     

Acquired cutis laxa secondary to Sweet syndrome in a child (Marshall syndrome): A rare case report

Sweet syndrome is rare in the pediatric population and usually responds well to treatment, resolving without sequelae. Marshall syndrome is a rare pediatric skin disease characterized by loss of elastic tissue (cutis laxa) secondary to acquired, localized neutrophilic dermatitis without any internal organ involvement. Only few cases of Marshall syndrome (acquired cutis laxa type II) have been reported. Systemic steroids and dapsone show excellent results in Sweet syndrome. Although there is no satisfactory treatment for cutis laxa, dapsone can be used in the acute phase for control of swelling.     

Jadassohn-Pellizari型皮肤松弛症并面部局限性皮肤松弛症一例

患者女,17岁.因面颈部皮肤松弛及躯干起淡白斑12年,于2011年4月25日来我院就诊.患者自5岁起,面颈部皮肤开始出现松弛,无明显不适,随年龄增长,皮肤松弛逐渐加重,面部皮肤出现较多皱折,双颌部及耳垂皮肤松弛、下垂,呈现老人外貌.同时,胸腹及背部皮肤出现少许圆形或椭圆形扁平隆起性皮损,逐渐增多、增大,初为淡红色,渐变为淡白色,无自觉症状.曾多次在外就诊,予以维生素类或中药治疗(具体不详),未见明显疗效.患者为第2胎,足月顺产,出生时发育正常.     

Acquired cutis laxa and myeloma: large vacuolated cells in the dermis

INTRODUCTION: Cutis laxa is a rare disorder characterized by loss of elastic tissue. Several organs are often involved such as the skin, lungs, heart, digestive system or genitourinary tract. It may be inherited or acquired, generalized or localized. Its pathogenesis is unclear. Association of acquired cutis laxa with myeloma or plasma cell dyscrasia is very rare. We report a case of acquired cutis laxa associated with a myeloma. CASE REPORT: A 59 year-old woman was admitted for skin hyperlaxity present for a number of years. Light microscopic examination of a skin sample revealed fragmented elastic fibers. Electron microscopic examination of the elastic network demonstrated numerous large vacuolated cells with the appearance of macrophages around abnormal elastic and collagen fibers of the reticular dermis. In addition, a stage-1 IgG lambda myeloma was detected. The patient was treated by thalidomide for one year. After this treatment, electron microscopy examination did not reveal any large vacuolated cells in the dermis, and elastic and collagen fibers were not modified and skin laxity seemed to be stabilized. DISCUSSION: Acquired cutis laxa may be associated with many systemic diseases or can appear after inflammatory skin diseases. Seven cases of generalized cutis laxa associated with myeloma and four cases associated with plasma cell dyscrasia have been reported in the literature. In our case, as in 2 previously described cases, large vacuolated cells resembling macrophages were seen in the dermis. They were thought to play a role in cutis laxa.     

Acquired cutis laxa associated with cutaneous angiocentric T-cell lymphoma

A 64-year-old man presenting with adenomegaly was diagnosed with stage IV A angiocentric cutaneous T-cell lymphoma in 1988. Since he showed a poor response to psoralen plus UVA (PUVA) after 1 year of therapy, he was switched to systemic chemotherapy (COP), and was considered to be in remission after completion of 5 months of this regimen. In December 1989, the patient noted an asymptomatic lesion at the superior and internal aspect of his right thigh; on examination, there was a poorly circumscribed, noninflammatory, whitish, atrophic macula near the inguinal fold and a palpable adenopathy; its surface was irregular, showing small wrinkling, and could be depressed on palpation. There were no preceding inflammatory lesions and no cutaneous lymphoma lesions at this site. Slowly, the initial macule extended, and new similar lesions insidiously appeared symmetrically on the left thigh. In October 1990, the patient received electron beam therapy for a relapse that presented as troncular nodules and responded well to this therapy. In the months that followed, the skin of his whole body became progressively sagging and pendulous, without recoil. This was not preceded by an inflammatory phase or eruption. There was no family history of elastolytic disorder, and the patient was not taking any medication other than that described above. On questioning, there were no symptoms suggesting systemic involvement of this elastolytic disorder. On physical examination, the patient's whole body skin seemed redundant, nonresllient, and wrinkled, this being most obvious in the axillae, inguinal region, antecubital fossa, neck, and trunk, where loose and pendulous skin folds could be seen (Fig. 1). Also, the patient's face was excessively wrinkled and sagging and he had bilateral ectropion. At the superior and internal aspect of both thighs, large, poorly circumsoribed, but distinct, round patches of 1-5 cm in diameter were observed; they were atrophic and whitish, with an uneven surface, and could be depressed on palpation. Apart from 1 cm, mobile, but firm, axillary and inguinal adenopathy and three subcutaneous nodules on the arm and trunk, the rest of the physical examination was unremarkable. Routine blood tests were normal, as was a chest X-ray. Sezary cells were absent on blood smear, and borrelia and syphilis serology were negative. Thyroid function tests, angiotensin converting enzyme, and immunoelectrophoresis were within normal limits, and antinuclear antigen was negative. Two cutaneous biopsies were performed: one in an atrophic herniating lesion of the right thigh and the one on the abdomen. Both showed, on hematoxylin-phloxin-safran staining, a mild dermal perivascular inflammatory infiltrate with a predominance of mononuclear cells. On Weigert staining, both were marked by a net reduction in elastic fibers throughout the dermis.     

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.