Postinflammatory Cutis Laxa: A Case Report
Derbali F1,, Hajji R1, Mohamed M2, Mnafgui K3, Hammedi F4, Elleuch M1, Kammoun N1, Zribi S1
1Internal Medicine Department, Sidi Bouzid Regional Hospital, Sidi Bouzid, Tunisia
2Dermatology Department, Monastir University Hospital, Monastir, Tunisia
3Pathophysiology department, FSS, Sfax, Tunisia
4Pathology Department, Monastir University Hospital, Monastir, Tunisia
Abstract
Cutis Laxa (CL) is a rare disease in which the skin loses its elasticity and hangs in large folds. It is an inherited or acquired connective tissue disorder. We report the case of a 29 year-old woman followed up since 4 years for a dermatomyositis treated with glucocorticosteroids and methotrexate. She was hospitalized in February 2012 for fever, arthralgia, pelvic and shoulder muscle weakness with myalgia, malar rash, thrombocytopenia, leucopenia and lymphocytopenia Immunological tests showed Antinuclear Antibodies (ANA) (+) to 1/640, AC anti-DNA (+) and AC anti-SSA (+). Histology of the salivary glands showed grade III lymphocytic sialadenitis. The systemic lupus erythematosus and secondary Sjögren’s syndrome were diagnosed. The patient was treated with glucocorticostroids, methotrexate, nivaquine and bissolvon. One year later, the patient presented a skin aging that began in hands which has expanded rapidly in the face. The skin biopsy confirmed the diagnosis of a Cutis Laxa. The esthetic treatment is proposed.
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Keywords: Acquired cutis laxa, systemic disease, systemic lupus erythematosus, dermatomyositis, Sjögren’s syndrome
American Journal of Medical Case Reports, 2014 2 (11),
pp 259-261.
DOI: 10.12691/ajmcr-2-11-10
Received October 30, 2014; Revised November 13, 2014; Accepted November 20, 2014
Copyright © 2013 Science and Education Publishing. All Rights Reserved.Cite this article:
- F, Derbali, et al. "Postinflammatory Cutis Laxa: A Case Report." American Journal of Medical Case Reports 2.11 (2014): 259-261.
- F, D. , R, H. , M, M. , K, M. , F, H. , M, E. , N, K. , & S, Z. (2014). Postinflammatory Cutis Laxa: A Case Report. American Journal of Medical Case Reports, 2(11), 259-261.
- F, Derbali, Hajji R, Mohamed M, Mnafgui K, Hammedi F, Elleuch M, Kammoun N, and Zribi S. "Postinflammatory Cutis Laxa: A Case Report." American Journal of Medical Case Reports 2, no. 11 (2014): 259-261.
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1. Introduction
Cutis laxa (CL) is also known as dermatolysis, dermatomegaly, chalazoderma, pachydermatocele, dermatochalasia, and elastolysis [1].
CL represents a heterogeneous group of connective tissue disorders that may be acquired or inherited. It can have a generalized or local form.
It is often preceded by cutaneous inflammatory eruption (ie, urticaria, eczema, erythema multiforme). Frequently, there is an important internal organ involvement: the gastrointestinal, pulmonary and cardiovascular systems can be concerned.
Redundant skin is often most noticeable on the neck, hands, and groin, but can also be seen on the face, creating a premature aging appearance.
Clinically, there is redundant skin with a wrinkled and loose appearance. Histologically, degenerative changes in the dermal elastic fibers were observed. Light microscopy reveals few or absent elastic fibers in the dermis [2].
Of the few reports on this rare disorder, authors have speculated about an immune-mediated destruction of elastic fibers, and monoclonal gammapathies, such as multiple myeloma or heavy chain deposition disease have a recognized association with CL.
We report through one observation an exceptional association: Acquired CL and mixed connective tissue disease.
2. Case Report
A 29-year-old Tunisian woman having the history of dermatomyositis diagnosed 4 years ago was treated with glucorticosteroids and methotrexate. Her disease was stable with a clinical and biological remission until 2012. In February 2012, she was admitted in the internal medicine department for fever, arthralgia, proximal muscle weakness, diffuse myalgia, eyelid erythema, a "butterfly" rash.
Physical examination revealed skin rash, metacarpophallangeal joints arthritis and motor deficit of the shoulder and pelvic muscles. Ophthalmologic examination confirmed eye dryness: Shirmer’s test, performed without anesthesia (under than 5 mm in 5 minutes) and Tear Break up Time (less than 10 seconds).
Biological tests confirmed the lymphopenia and thrombopenia and showed increased inflammatory biological parameters (CRP, ESR).
Immunological tests showed a high rate (1/640) of ANA and positive anti-DNA, anti-SSB and anti-SSA antibodies. The biopsy of the accessory salivary glands showed grade III lymphocytic sialadenitis according to Chisholm classification.
The systemic lupus erythematosus and secondary Sjögren’s syndrome associated to dermatomyositis confirm the diagnosis of mixed connective tissue disease. Thus, she was treated with glucocorticosteroids, methotrexate, nivaquine and bissolvon. The evolution was marked by the regression of disease activity signs.
One year after, the patient developed skin loosening, deep wrinkles aging that began in hands which quickly spread to the face. Physical examination revealed loose, pendulous skin of all fingers, hands and the face (Figure 1, Figure 2). She appeared to be much older than her real age (Figure 2). Pathological examination of the biopsy material showed the fragmentation and destruction of elastic fibers (Figure 3).
The diagnosis of "Cutis Laxa" was chosen because of the clinical and histological data. Physical and morphological examination (chest X-ray; echocardiography; gastroscopy …) didn’t show any visceral affection. Protein electrophoresis ruled out gammapathy particularly multiple myeloma. There was no history of drug ingestion. Botox injection was proposed.
3. Discussion
CL is a rare connective tissue disorder characterized by wrinkled skin with loss of elasticity. It can be hereditary or acquired, generalized or localized [3, 4]. Our patient is suffering from acquired and localized CL.
Its pathophysiology is not well understood. Hypotheses include abnormal copper metabolism or copper deficiency, decreased serum elastase inhibitor (α 1-antitrypsin), low lysyl oxidase activity, and immune-mediated mechanisms. In one case, anti-elastin antibodies were detected [5, 6].
Acquired CL has been described in association with drug ingestion, inflammatory skin disorders, and neoplasms. Reviewing the literature, we noted that diseases that have caused acquired cutis laxa include chronic urticaria, erythema multiforme, erythema perstans, vesicular eruptions, eczema, and Sweet’s syndrome [7, 8, 9]. Drugs such as penicillin, penicillamine, and isoniazid have also been implicated [10, 11].
Reports of Acquired CL associated with multiple myeloma, plasmacytoid lymphoma, systemic lupus erythematosus, nephritic syndrome with and without sarcoidosis, necrobiosis lipoidica, syphilis, and Lyme disease can be found in the literature [12-17][12].
The basic histological lesion is localized in the connective tissue related to electively elastic fibers. However, other lesions in the dermis can be observed. In our case, pathological examination showed the fragmentation and destruction of elastic fibers (Figure 3).
The lesions of elastic fibers may also affect other organs than the skin and cause vascular or visceral lesions. Our patient showed no extra-cutaneous affection.
Acquired CL is more common in adults. It progresses in a cephalocaudal direction [18, 19, 20]. In our case, symptoms began in hands which quickly spread to the face (Figure 1- Figure 2).
The disease most commonly found in association with CL is monoclonal gammapathy particularly multiple myeloma. Since 1976, a dozen cases of this association was reported [21-25][21].
Lewis FM et al. and Vegnil S et al. reported, respectively in 1993 and 2003, a patient with acquired CL in association with sarcoidosis [26, 27].
In 2002, Rongioletti F et al. described the first case of the acral localization of the acquired form of CL associated with severe rheumatoid arthritis [28].
In our knowledge, our case represents the first reporting the association mixed connective tissue disease /CL.
In all the reported sightings, skin aging is preceded by erythematous scaly lesions. This was not the case of our patient.
Acquired CL is not transmitted genetically. However, one of the areas of research conducted by Siefring ML et al is whether certain individuals may have a genetic predisposition to develop CL after certain exposures [29].
Nowadays, there is no yet aneffective therapy for CL. Its treatment based on plastic surgery such as cosmetic surgical procedure (rhytidectomy) [30, 31].
4. Conclusion
CL is a rare connective tissue disease, the first obvious symptom is a skin slackening. The late appearance of CL should suggest the acquired form which makes the prognosis worse.
References
[1] | James WD, Berger TG, Elston DM. Andrews’ diseases of the skin: clinical dermatology. Philadelphia: Saunders 2006. p. 515 | ||
![]() | |||
[2] | George S, Jacob M, Pulimood S, Chandi SM. Cutis laxa. Clin Exp Dermatol 1998; 23 (5): 211-3. | ||
![]() | CrossRef | ||
[3] | Fitzsimmons JS, Fitzsimmons EM, Guibert PR, Zaldua V, Dodd KL. Variable clinical presentation of cutis laxa. Clin Genet 1985; 28 (4): 284-95. | ||
![]() | CrossRef | ||
[4] | Harris RB, Heaphy MR, Perry HO. Generalized elastolysis (cutis laxa). Am J Med 1978; 65 (5): 815-22 | ||
![]() | CrossRef | ||
[5] | Kuivaniemi H, Peltonen L, Kivirikko KI. Type IX Ehlers-Danlos syndrome and Menkes syndrome: the decrease in lysyl oxidase activity is associated with a corresponding deficiency in the enzyme protein. Am J Hum Genet 1985; 37 (4): 798-808. | ||
![]() | |||
[6] | Lewis PG, Hood AF, Barnett NK, Holbrook KA. Postinflammatory elastolysis and cutis laxa: A case report. J Am Acad Dermatol 1990; 22 (1): 40-8. | ||
![]() | CrossRef | ||
[7] | Tsuji T, Imajo Y, Sawabe M, Kuniyuki S, Ishii M, Hamada T, et al. Acquired cutis laxa concomitant with nephrotic syndrome. Arch Dermatol 1987; 123 (9): 1211-6. | ||
![]() | CrossRef | ||
[8] | Chun SI, Yoon J. Acquired cutis laxa associated with chronic urticaria. J Am Acad Dermatol 1995; 33 (5): 896-9. | ||
![]() | CrossRef | ||
[9] | Harpey JP, Jaudon MC, Clavel JP, Galli A, Darbois Y. Cutis laxa and low serum zinc after antenatal exposure to penicillamine. Lancet 1983; 2: 858. | ||
![]() | CrossRef | ||
[10] | Kerl H, Burg G, Hashimoto K. Fatal, penicillin-induced, generalized post-inflammatory elastolysis (cutis laxa). Am J Dermatopathol 1983; 5: 267-76. | ||
![]() | CrossRef | ||
[11] | Koch SE, Williams ML. Acquired cutis laxa: case report and review of disorders of elastolysis. Pediatr Dermatol 1985; 2 (4): 282-8. | ||
![]() | CrossRef | ||
[12] | Delisle BR, Schanne R, Gilbert M. Generalized post-inflammatory cutis laxa associated with lupic panniculitis. Ann Dermatol Venereol 1990; 117 (11): 841-4. | ||
![]() | |||
[13] | Ozkan S, Fetil E, Gunes AT, Bozkurt E, Sahin T, Erkizan V, et al. Cutis laxa acquisita: is there any association with Borrelia burgdorferi? Eur J Dermatol 1999; 9 (7): 561-4. | ||
![]() | |||
[14] | McCarty MJ, Davidson JM, Cardone JS, Anderson LL. Cutis laxa acquisita associated with multiple myeloma: a case report and review of the literature. Cutis 1996; 57 (4): 267-70. | ||
![]() | |||
[15] | Gupta A, Helm TN. Acquired cutis laxa associated with multiple myeloma. Cutis 2002; 69 (2): 114-8. | ||
![]() | |||
[16] | Machet MC, Machet L, Vaillant L, Estève E, de Muret A, Arbeille A, et al. Acquired localized cutis laxa due to cutaneous lymphoplasmacytoid lymphoma. Arch Dermatol 1995; 131 (1): 110-1. | ||
![]() | CrossRef | ||
[17] | Dennis P K, Peter A K. Acquired Cutis Laxa in a 55-year- old female with multiple myeloma and serologic evidence of systemic lupus erythematosis. Dermatol Online J 2011; 15; 17 (7): 8. | ||
![]() | |||
[18] | Harris RB, Heaphy MR, Perry HO. Generalized elastolysis (cutis laxa). Am J Med 1978; 65 (5): 815-22. | ||
![]() | CrossRef | ||
[19] | Reed WB, Horowitz RE, Beighton P. Acquired cutis laxa. Primary generalized elastolysis. Arch Dermatol 1971; 103 (6): 661-9. | ||
![]() | CrossRef | ||
[20] | Goltz RW, Hult AM, Goldfarb M, Gorlin RJ. Cutis laxa. A manifestation of generalized elastolysis. Arch Dermatol 1965; 92 (4): 373-87. | ||
![]() | CrossRef | ||
[21] | Frémont G, Kérob D, Prost-Squarcioni C, Lièvre N, Rivet J, Tancrède E et al. Cutis laxa acquise généralisée associée à un myélome : découverte de grandes cellules vacuolisées dermiques. Ann Dermatol Venereol 2007; 134: 548-51. | ||
![]() | CrossRef | ||
[22] | Turner MD, Haynes HA, Granter SR, Miller MD. Acquired cutis laxa following urticarial vasculitis associated with IgA myeloma. J Am Acad Dermatol 2009; 60 (6): 1052-7. | ||
![]() | CrossRef | ||
[23] | New HD, Callen JP. Acquired cutis laxa and granuloma annulare-like lesions secondary to multiple myeloma. J Am Acad Dermatol 2010: AB43. | ||
![]() | |||
[24] | Urbanski G, Dib M, Simon A, Croué A, Arbeille B, Callewaert B et al. Cutis laxa acquise associée à une gammapathie monoclonale chez un patient porteur d’une mutation du gène de l’élastine. Ann Dermatol Venereol. 2012; 139 (12 Suppl), B202-B203 | ||
![]() | CrossRef | ||
[25] | Combes E, Gaudy-Marqueste C, Archier E, Mallet S, Monestier S, GrobJJ, et al. Cutis laxa acquise révélant une gammapathie monoclonale à IgGcompliquée d’une néphropathie. Ann Dermatol Venereol. 2013; 140 (12Suppl 1): S604-S605 | ||
![]() | CrossRef | ||
[26] | Lewis FM, Lewis-Jones S, Gipson M. Acquired cutis laxa with dermatitis herpetiformis and sarcoidosis. J Am Acad Dermatol 1993; 29: (5 Pt 2):846-8. | ||
![]() | CrossRef | ||
[27] | Vegnil S, Vignali P, Aitken G, Grossin M, Vinceneux P. Cutis laxa acquise aprés huit ans d'évolution d'une sarcoidose. Rev Med Int 2003; 24 Suppl 4. | ||
![]() | |||
[28] | Rongioletti F, Cutolo M, Bondavalli P, Rebora A. Acral localized acquired cutis laxa associated with rheumatoid arthritis. J Am Acad Dermatol 2002; 46: 128-30. | ||
![]() | CrossRef | ||
[29] | Siefring ML, Lawrence EC, Nguyen TC, Lu D, Pham G, Lorenchick C, Levine KL, Urban Z.. A Novel Elastin Gene Mutation in a Vietnamese Patient with Cutis Laxa. Pediatr Dermatol 2014; 31 (3): 347-9. | ||
![]() | CrossRef | ||
[30] | Paulsen I F, Bredgaard R. Acquired cutis laxa: Diagnostic and therapeutic considerations. J Plast Reconstr Aesthet Surg 2014; 67 (10): 242-3. | ||
![]() | CrossRef | ||
[31] | Mehta B, Amladi S. Acquired localized cutis laxa of the face: a rare presentation. Pediatr Dermatol. 2011; 28 (4): 421-3. | ||
![]() | CrossRef | ||