Journal of Plastic, Reconstructive & Aesthetic Surgery (2014) 67, e242ee243
CORRESPONDENCE AND COMMUNICATION Acquired cutis laxa: Diagnostic and therapeutic considerations Dear Sir, Cutis laxa (CL) is a rare, inherited or acquired connective tissue disorder that may appear either as a localized or generalized disease.1,2 It is characterized by a reduced number and abnormal properties of elastic fibers throughout dermis, which creates a characteristic clinical appearance with loose, hypoelastic skin and premature aging with distinct furrows, wrinkles, and inelastic and sagging skin. This is illustrated in the case history described below. Acquired CL is a rare disease, which most often gets symptomatic in early adulthood. Many different causes have been reported to trigger the first attack of the disease. It is often associated with inflammatory dermatoses or more rarely with a neoplastic disease. The pathogenesis is considered to be related to a hypersensitivity reaction to bacterial or other antigens, and an acute attack of urticaria often precedes the disease. Certain drugs like penicillin, penicillamin, and isoniazide appear to act as releasing factors.2,3 The pathogenesis of acquired CL is largely unknown but different hypotheses have been described, including excessive elastase activity from neutrophils and macrophages, an immune-mediated mechanism, or a dysfunction in elastase inhibitors.2,4 It affects the elastic fibers resulting in the loss of elasticity. The changes in the elastic fibers depend on the stage and severity of the disease. Reduction of elastic fibers may either be observed throughout all the layers of dermis, or confined to part of the dermis. The remaining elastic fibers are often short and fragmented, while the collagen fibers usually are normal. Although the diagnosis may appear obvious from clinical symptoms, a definitive diagnosis requires histological confirmation in order to differentiate from other conditions such as mid-dermal elastolysis, Ehlers-Danlos syndrome, postinflammatory elastolysis, anetoderma, Marfan’s syndrome, and pseudoxanthoma elasticum. Unlike most other connective tissue disorders patients with CL will generally heal well after surgery.2,5
Worldwide, only few patients have been described with acquired CL. Information regarding effect of treatment options is virtually absent and therefore highly needed. There is currently no effective medical treatment, but prophylactic treatment of urticaria, which may trigger new attacks, is supposed to reduce the rate of disease progression. Future therapeutic approaches may include immunosuppressant drugs or specific agents that are able to block elastase activity or induce elastin synthesis. The effect and duration of plastic surgical intervention is also uncertain. Surgical interventions can have a cosmetic effect, but the duration is hardly long-term. Nevertheless, cosmetic surgical intervention may be of major psychological significance for the patients, with a significant impact on a more gradual adaptation to an abnormal look in relation to the chronological age.
Case history A 30-year old Caucasian woman was referred to an outpatient clinic of dermatology because of obvious signs of aging that had suddenly appeared in her face and neck (Figures 1 and 2). The patient was otherwise healthy except for an allergic reaction to penicillin five years ago, causing a generalized attack of urticaria. Since then the patient had suffered from chronic urticaria. Before referral to the clinic of plastic surgery, bilateral blepharoplasty operations were performed twice, but both with rapid recurrences. Shortly after the first attack of urticaria signs of premature aging of the skin appeared in the face and neck. There were no symptoms from internal organs or skin changes on the body or extremities. No family members had a history of skin diseases. Beyond contraceptives the patient received no medication. When admitted to the clinic of plastic surgery physical examination revealed a lax and deeply wrinkled facial skin, pendulous earlobes, and drooping eyelids. She looked like a person twice her biological age. Otherwise physical examination was normal including normal extensibility of the joints. Echocardiogram and chest xray were normal. A DEXA scan suggested osteopoenia with a mildly reduced T-score. Laboratory blood tests were normal except for moderate leukocytosis, explained by an acute cystitis.
http://dx.doi.org/10.1016/j.bjps.2014.06.010 1748-6815/ª 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
Correspondence and communication
e243 Skin biopsies from her face and neck showed an almost total absence of elastic fibers in the dermis. Neutrophil granulocytes were observed in epidermis in one of the biopsies. Biopsies taken from normal skin in the arm showed normally looking elastic fibers in the upper part of the dermis, almost none in mid-dermis, and a slightly decreased number in the deep dermis. It was concluded that histology supported the clinical diagnosis of an acquired CL, triggered by urticaria. The patient was treated for her chronic urticaria with daily, oral antihistamines, and every second week she received an injection with anti-IgE antibodies. Addition of immunosuppressants may be started after reconstructive plastic surgery of the face.
Conflict of interest/funding None. Figure 1 At the age of 23 years the patient’s appearance is normal for age.
References 1. Riveros CJ, Gavilan MF, Franca LF, Sotto MN, Takahashi MD. Acquired localized cutis laxa confined to the face: case report and review of the literature. Int J Dermatol 2004;43:931e5. 2. Banks ND, Redett RJ, Mofid MZ, Manson PN. Cutis laxa: clinical experience and outcomes. Plast Reconstr Surg 2003;111: 2434e42. 3. Mehta B, Amladi S. Acquired localized cutis laxa of the face: a rare presentation. Pediatr Dermatol 2011;28:421e3. 4. Berk DR, Bentley DD, Bayliss SJ, Lind A, Urban Z. Cutis laxa: a review. J Am Acad Dermatol 2012;66:842.e1e842.e17. 5. Ozsoy Z, Gozu A, Dayicioglu D, Mete O, Buyukbabani N. Localized cutis laxa and blepharoplasty. Dermatol Surg 2007; 33:1510e2.
I.F. Paulsen R. Bredgaard Clinic for Plastic Surgery, Burn Treatment and Breast Surgery, Copenhagen University Hospital, Rigshospitalet, Denmark E-mail address: [email protected] B. Hesse Clinic of Clinical Physiology, Nuclear Medicine and PET, Copenhagen University Hospital, Rigshospitalet, Denmark Figure 2 At the age of 30 years the same patient shows obvious, premature aging with loose and deeply wrinkled face and drooping eyelids in spite of two blepharoplasty operations, performed 1 and 2 years earlier.
T. Steiniche Clinic of Pathology, Aarhus University Hospital, Denmark T.F. Henriksen Clinic for Plastic Surgery, Burn Treatment and Breast Surgery, Copenhagen University Hospital, Rigshospitalet, Denmark 20 December 2013
CORRESPONDENCE AND COMMUNICATION Acquired cutis laxa: Diagnostic and therapeutic considerations Dear Sir, Cutis laxa (CL) is a rare, inherited or acquired connective tissue disorder that may appear either as a localized or generalized disease.1,2 It is characterized by a reduced number and abnormal properties of elastic fibers throughout dermis, which creates a characteristic clinical appearance with loose, hypoelastic skin and premature aging with distinct furrows, wrinkles, and inelastic and sagging skin. This is illustrated in the case history described below. Acquired CL is a rare disease, which most often gets symptomatic in early adulthood. Many different causes have been reported to trigger the first attack of the disease. It is often associated with inflammatory dermatoses or more rarely with a neoplastic disease. The pathogenesis is considered to be related to a hypersensitivity reaction to bacterial or other antigens, and an acute attack of urticaria often precedes the disease. Certain drugs like penicillin, penicillamin, and isoniazide appear to act as releasing factors.2,3 The pathogenesis of acquired CL is largely unknown but different hypotheses have been described, including excessive elastase activity from neutrophils and macrophages, an immune-mediated mechanism, or a dysfunction in elastase inhibitors.2,4 It affects the elastic fibers resulting in the loss of elasticity. The changes in the elastic fibers depend on the stage and severity of the disease. Reduction of elastic fibers may either be observed throughout all the layers of dermis, or confined to part of the dermis. The remaining elastic fibers are often short and fragmented, while the collagen fibers usually are normal. Although the diagnosis may appear obvious from clinical symptoms, a definitive diagnosis requires histological confirmation in order to differentiate from other conditions such as mid-dermal elastolysis, Ehlers-Danlos syndrome, postinflammatory elastolysis, anetoderma, Marfan’s syndrome, and pseudoxanthoma elasticum. Unlike most other connective tissue disorders patients with CL will generally heal well after surgery.2,5
Worldwide, only few patients have been described with acquired CL. Information regarding effect of treatment options is virtually absent and therefore highly needed. There is currently no effective medical treatment, but prophylactic treatment of urticaria, which may trigger new attacks, is supposed to reduce the rate of disease progression. Future therapeutic approaches may include immunosuppressant drugs or specific agents that are able to block elastase activity or induce elastin synthesis. The effect and duration of plastic surgical intervention is also uncertain. Surgical interventions can have a cosmetic effect, but the duration is hardly long-term. Nevertheless, cosmetic surgical intervention may be of major psychological significance for the patients, with a significant impact on a more gradual adaptation to an abnormal look in relation to the chronological age.
Case history A 30-year old Caucasian woman was referred to an outpatient clinic of dermatology because of obvious signs of aging that had suddenly appeared in her face and neck (Figures 1 and 2). The patient was otherwise healthy except for an allergic reaction to penicillin five years ago, causing a generalized attack of urticaria. Since then the patient had suffered from chronic urticaria. Before referral to the clinic of plastic surgery, bilateral blepharoplasty operations were performed twice, but both with rapid recurrences. Shortly after the first attack of urticaria signs of premature aging of the skin appeared in the face and neck. There were no symptoms from internal organs or skin changes on the body or extremities. No family members had a history of skin diseases. Beyond contraceptives the patient received no medication. When admitted to the clinic of plastic surgery physical examination revealed a lax and deeply wrinkled facial skin, pendulous earlobes, and drooping eyelids. She looked like a person twice her biological age. Otherwise physical examination was normal including normal extensibility of the joints. Echocardiogram and chest xray were normal. A DEXA scan suggested osteopoenia with a mildly reduced T-score. Laboratory blood tests were normal except for moderate leukocytosis, explained by an acute cystitis.
http://dx.doi.org/10.1016/j.bjps.2014.06.010 1748-6815/ª 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
Correspondence and communication
e243 Skin biopsies from her face and neck showed an almost total absence of elastic fibers in the dermis. Neutrophil granulocytes were observed in epidermis in one of the biopsies. Biopsies taken from normal skin in the arm showed normally looking elastic fibers in the upper part of the dermis, almost none in mid-dermis, and a slightly decreased number in the deep dermis. It was concluded that histology supported the clinical diagnosis of an acquired CL, triggered by urticaria. The patient was treated for her chronic urticaria with daily, oral antihistamines, and every second week she received an injection with anti-IgE antibodies. Addition of immunosuppressants may be started after reconstructive plastic surgery of the face.
Conflict of interest/funding None. Figure 1 At the age of 23 years the patient’s appearance is normal for age.
References 1. Riveros CJ, Gavilan MF, Franca LF, Sotto MN, Takahashi MD. Acquired localized cutis laxa confined to the face: case report and review of the literature. Int J Dermatol 2004;43:931e5. 2. Banks ND, Redett RJ, Mofid MZ, Manson PN. Cutis laxa: clinical experience and outcomes. Plast Reconstr Surg 2003;111: 2434e42. 3. Mehta B, Amladi S. Acquired localized cutis laxa of the face: a rare presentation. Pediatr Dermatol 2011;28:421e3. 4. Berk DR, Bentley DD, Bayliss SJ, Lind A, Urban Z. Cutis laxa: a review. J Am Acad Dermatol 2012;66:842.e1e842.e17. 5. Ozsoy Z, Gozu A, Dayicioglu D, Mete O, Buyukbabani N. Localized cutis laxa and blepharoplasty. Dermatol Surg 2007; 33:1510e2.
I.F. Paulsen R. Bredgaard Clinic for Plastic Surgery, Burn Treatment and Breast Surgery, Copenhagen University Hospital, Rigshospitalet, Denmark E-mail address: [email protected] B. Hesse Clinic of Clinical Physiology, Nuclear Medicine and PET, Copenhagen University Hospital, Rigshospitalet, Denmark Figure 2 At the age of 30 years the same patient shows obvious, premature aging with loose and deeply wrinkled face and drooping eyelids in spite of two blepharoplasty operations, performed 1 and 2 years earlier.
T. Steiniche Clinic of Pathology, Aarhus University Hospital, Denmark T.F. Henriksen Clinic for Plastic Surgery, Burn Treatment and Breast Surgery, Copenhagen University Hospital, Rigshospitalet, Denmark 20 December 2013
