Generalized acquired cutis laxa treated with facial plastic surgery

ARTICLE

Auteur(s) : Yanning XUE¹, Hao CHEN², Xuesi ZENG², Yiqun JIANG², Jianfang SUN² fangmin5758@yahoo.com.cn

¹ Jiangsu Province hospital of traditional Chinese medicine, 155 Han-zhong Road, 210029 Nanjing, Jiangsu Province, China

² Institute of Dermatology, Chinese Academy of Medical Sciences, 12 Jiang-wang-miao Road, Nanjing city, 210042 Jiangsu Province, China

Cutis laxa (dermatochalasis, generalized elastolysis) is an uncommon disorder characterized by laxity of the skin, which hangs in loose folds, producing the appearance of premature aging. It can be inherited or acquired, both forms being generalized or localized [1]. The acquired generalized form is very uncommon and is usually preceded by inflammatory dermatosis. We report the case of aquired cutis laxa successfully treated with surgical repair.

A 33-year-old woman attended our hospital for gradual but progressive premature aging during the last 2 years (figure 1A). Many lax papules and plaques arose on her back without subjective symptoms 2 years previously. These lesions spread slowly to her chest, abdomen (figure 1B), face, neck and proximal extremities. The patient's face was loose and sagging. She had had episodes of wheal-like lesions on her trunk and extremities for 6 years (figure 1C). The lesions were painful rather than pruritic. After 48 hours the wheal-like lesions became healing hyperpigmentation. She had no fever, arthralgia or abdominal pain. The patient had no history of prior penicillin or penicillamine use or complement deficiency. Her family history included no evidence of inherited cutis laxa. Routine laboratory tests and immunologic findings yielded normal results. The histopathology of the lax skin showed minimal changes, with only a sparse lymphocytic infiltrate, perivascularly. On elastic tissue stain, the elastic fibers were diffusely diminished in the dermis (figure 1D). The histopathology of a wheal-like lesion revealed a relatively dense infiltrate of neutrophils and lymphocytes around the blood vessels and with fragmentation of nuclei but without fibrinoid necrosis. The elastic fibers became short and thin around the small vessels (figure 1E). Both clinical and histological findings confirmed the diagnosis of aquired cutis laxa. No systemic involvement was found. The patient was sent for surgical repair, with satisfactory results to date (figure 1F).

In 1966, Marshall et al. first proposed a classification for cutis laxa, which is widely accepted in the literature; essentially they divided it into two categories: inherited and acquired forms [1]. Congenital cutis laxa mainly occurs in neonates or young children; it may have severe systemic involvement that can even cause early death [2, 3]. Acquired cutis laxa has a more benign course, affecting mainly young adults and with minor systemic involvement. Men and women are equally affected. There is no racial predilection. The onset of disease may be insidious. It may be generalized or localized. Lesions are commonly described as pendulous or redundant coarsely wrinkled areas of skin. Classically, the face and particularly the earlobes are involved and lesions typically spread in a generalized direction. Localized cases have also been reported. Systemic involvement can affect the pulmonary, vascular, and gastrointestinal tracts. The diagnosis is strongly suggested clinically, but requires histopathologic confirmation by the loss of dermal elastic tissue. Our patient had typical clinical and histopathological characteristics.

Acquired cutis laxa has been associated with many conditions, including inflammatory dermatoses, hypersensitivity reactions, neoplastic disorders [4, 5]. Urticaria and urticarial-like lesions are the most common diseases associated with cutis laxa. Our patient is peculiar in that she had urticarial vasculitis-like lesions, which were different from other patients reported before.

Surgical repair seems to be the only therapeutic choice and can provide long-term success. It is important to realize that facelift surgery and blepharoplasty are major procedures with a significant recovery period. Our patient was submitted to rhytidectomy and blepharoplasty for repair, with satisfactory results to date. Dapsone may improve inflammatory dermatoses [6]. But there is no therapy to prevent the progression of cutis laxa. How to hold back progression of the disease is still a challenge.

Disclosure

Financial support: none. Conflict of interest: none.

References

1 J Marshall, T Heyl, H.W. Weber Postinflammatory elastolysis and cutis laxa. A report on a new variety of this phenomenon and a discussion of some syndromes characterized by elastolysis S Afr Med J 1966; 40: 1016-1022.

2 WO Thomas, MH Moses, RD Craver et al. Congenital cutis laxa: a case report and review of loose skin syndromes Ann Plast Surg 1993; 30: 252-256.

3 S de Schepper, B Loeys, A de Paepe et al. Cutis laxa of the autosomal recessive type in a consanguineous family Eur J Dermatol 2003; 13: 529-533.

4 CJP Riveros, MFB Gavilán, LFS Francxa et al. Acquired cutis laxa confined to the face: case report and review of the literature Int J Dermatol 2004; 43: 931.

5 KG Lewis, L Bercovitch, S Dill et al. Acquired disorders of elastic tissue: part II. Decreased elastic tissue J Am Acad Dermatol 2004; 51: 165-185.

6 BK Fisher, E Page, W. Hanna Acral localized acquired cutis laxa J Am Acad Dermatol 1989; 21: 33-40.