Acquired cutis laxa

Introduction

Cutis laxa is a heterogenous group of disorder characterised by wide spread laxity of the skin resulting in a premature aged appearance.[1] The basic defect is generalised elastolysis. Cutis laxa can be congenital or acquired. The disease is usually progressive and there is no definite treatment.

Case Report

A 13-year-old male patient presented to our OP with history of recurrent episodes of urticaria and one episode of angioedema for the past 2 years. There were no constitutional symptoms and the urticaria was not associated with any specific food or drug intake. For the past 1 year the patient had noticed gradual progressive wrinkling and laxity of the skin which initially started on the face and later spread to involve the trunk. There was no parental consanguinity or family history of similar condition. On examination the patient had an "old man" appearance. The skin over the face, axilla and abdomen was wrinkled and thrown in loose folds [Figure - 1]. The skin could be easily stretched and showed slow recoiling. There was minimal involvement of the extremities. The oral cavity, genitalia, hair, nails were normal. The systems were within normal limits.

The blood haemogram, urinalysis, liver function tests and renal function tests were within normal limits. Serological test for syphilis and HIV were negative. Chest X-ray and USS abdomen were normal. Thyroid function tests, immunoglobulin profile and complement assays were within normal limits. Allergy test showed the patient allergic to spinach, cockroach and mosquito. Skin biopsy with special stains showed clumping of elastic fibres in the dermis with scanty non specific mononuclear infiltrate consistent with cutis laxa.

The patient was treated with systemic steroids and antihistamines for the urticaria. The Plastic surgeon performed a "face lift" operation for the patient which considerably improved the appearance of the patient.

Discussion

Cutis laxa, other synonyms being generalised elastolysis, elastorrhexis or dermatochalasis is characterised clinically by lax pendulous skin and histologically by loss of elastic tissue in the dermis. The easy stretching of the skin with tardy recoil is the clinical hallmark of the patient in contrast to Ehler-Danlos syndrome where the skin recoils rapidly after stretching. Cutis laxa can be congenital or acquired. Congenital cutis laxa can be autosomal dominant or recessive.[2] The autosomal dominant variety primarily affects the skin. The affected adult may have systemic abnormalities like inguinal hernia, pulmonary emphysema and bronchiectasis. The autosomal recessive variety is severe presenting with fatal cardiopulmonary disease, gastrointestinal and urinary tract diverticula and skeletal abnormalities. Acquired cutis laxa occurs secondary to a variety of conditions like inflammatory skin diseases, hypersensitivity reactions, complement deficiency, multiple myeloma and other plasma cell dyscrasias, syphilis, sarcoidosis and drugs like penicillamine.[3] The histopathological hallmark of cutis laxa is short, fragmented, clumped and even absent elastic fibres. Electron microscope studies has shown abnormalities in the fibrillin component of elastic fibres.[4] The underlying defect may be in the altered rate of transcription in the elastic mRNA.

Previously acquired cutis laxa following an attack of measles has been reported.5 Cutis laxa is a progressive disorder usually worsening with age. The physical appearance can be improved with plastic surgery procedures like repeated "face-lift" operations. The systemic abnormalities may need specific treatment depending upon the condition. Cutis laxa is a condition which can be easily diagnosed clinically without elaborate investigations. Hence the clinician should be aware of the condition. This case is being reported for the rarity of the condition. The aetiology being urticaria and angioedema in our case report being even more rare.