Postinflammatory Cutis Laxa: A Case Report

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 antiDNA (+) 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.

Full text

American Journal of Medical Case Reports, 2014, Vol. 2, No. 11, 259-261
Available online at http://pubs.sciepub.com/ajmcr/2/11/10
© Science and Education Publishing
DOI:10.12691/ajmcr-2-11-10
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
*Corresponding author: derbalifatma@yahoo.com
Received October 30, 2014; Revised November 13, 2014; Accepted November 20, 2014
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.
Keywords: Acquired cutis laxa, systemic disease, systemic lupus erythematosus, dermatomyositis, Sjögren’s
syndrome
Cite This Article: Derbali F, 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, vol. 2, no.
11 (2014): 259-261. doi: 10.12691/ajmcr-2-11-10.
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.

260 American Journal of Medical Case Reports
Thus, she was treated with glucocorticosteroids,
methotrexate, nivaquine and bissolvon. The evolution was
marked by the regression of disease activity signs.
Figure 1. Loose and pendulous skin of all fingers
Figure 2. Loose, pendulous skin of the face; the patient appeared to be
much older than her real age
Figure 3. Pathological examination of the biopsy material showing
fragmentation and destruction of elastic fibers (HE x 100; Ocrein x 100)
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].
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].
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].

American Journal of Medical Case Reports 261
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.
[3] Fitzsimmons JS, Fitzsimmons EM, Guibert PR, Zaldua V, Dodd
KL. Variable clinical presentation of cutis laxa. Clin Genet 1985;
28 (4): 284-95.
[4] Harris RB, Heaphy MR, Perry HO. Generalized elastolysis (cutis
laxa). Am J Med 1978; 65 (5): 815-22
[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.
[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.
[8] Chun SI, Yoon J. Acquired cutis laxa associated with chronic
urticaria. J Am Acad Dermatol 1995; 33 (5): 896-9.
[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.
[10] Kerl H, Burg G, Hashimoto K. Fatal, penicillin-induced,
generalized post-inflammatory elastolysis (cutis laxa). Am J
Dermatopathol 1983; 5: 267-76.
[11] Koch SE, Williams ML. Acquired cutis laxa: case report and
review of disorders of elastolysis. Pediatr Dermatol 1985; 2 (4):
282-8.
[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.
[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.
[19] Reed WB, Horowitz RE, Beighton P. Acquired cutis laxa. Primary
generalized elastolysis. Arch Dermatol 1971; 103 (6): 661-9.
[20] Goltz RW, Hult AM, Goldfarb M, Gorlin RJ. Cutis laxa. A
manifestation of generalized elastolysis. Arch Dermatol 1965; 92
(4): 373-87.
[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.
[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.
[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
[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
[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.
[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.
[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.
[30] Paulsen I F, Bredgaard R. Acquired cutis laxa: Diagnostic and
therapeutic considerations. J Plast Reconstr Aesthet Surg 2014; 67
(10): 242-3.
[31] Mehta B, Amladi S. Acquired localized cutis laxa of the face: a
rare presentation. Pediatr Dermatol. 2011; 28 (4): 421-3.