Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Letter
ARTICLE IN PRESS
doi:
10.25259/IJDVL_824_2023

Acquired generalised cutis laxa with visceral involvement in a patient with rheumatoid arthritis and monoclonal gammopathy of uncertain significance

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India
Department of Radiodiagnosis and Interventional Radiology, All India Institute of Medical Sciences, New Delhi, India
Corresponding author: Dr. Vishal Gupta, All India Institute of Medical Sciences, New Delhi, India. doctor.vishalgupta@gmail.com
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Manandhar K, Lal A, Manchanda S, Gupta V. Acquired generalised cutis laxa with visceral involvement in a patient with rheumatoid arthritis and monoclonal gammopathy of uncertain significance. Indian J Dermatol Venereol Leprol. doi: 10.25259/IJDVL_824_2023

Dear Editor,

Cutis laxa is a rare disorder of elastic tissue characterised by lax pendulous skin and variable systemic involvement. It may be inherited or acquired; the acquired forms being more prevalent.1,2 We report acquired generalised cutis laxa in a patient with rheumatoid arthritis, who was subsequently found to have visceral involvement and monoclonal gammopathy of uncertain significance.

A 38-year-old man consulted us for generalised ‘loosening’ of the skin. He had first noticed it on the face 3 years ago and it gradually progressed to involve other sites, particularly axillae and groins. His past medical history was relevant for rheumatoid arthritis (RA) of 9 years’ duration, being treated with intermittent use of oral complementary and alternative medicine with a waxing and waning course. The family history was non-contributory. General physical examination was unremarkable. Mucocutaneous examination was striking for generalised laxity and sagging of the skin with poor elastic recoil, particularly prominent over the chest, axillae, and groins [Figures 1a and 1b]. With a diagnosis of generalised acquired cutis laxa, the patient was evaluated for internal organ involvement and any underlying associated conditions. His routine haemogram and biochemistry were within normal limits. Serum titres for RA factor (96.9 IU/mL, reference <14 IU/mL) and CCP (58.2 U/mL, reference <5 U/mL) were raised. Chest X-ray showed emphysematous changes in bilateral lung lower lobes [Figure 2a]. High-resolution computed tomography (HRCT) of the chest showed centrilobular emphysematous changes in bilateral upper lobes, panlobular emphysematous changes in bilateral lower lobes, and fibrotic opacities in the left lower lobe [Figures 2b and 2c]. Pulmonary function test results FEV1 22%, FVC 43%, FEV1/FVC 51%) were suggestive of an obstructive lung disease. Micturating cystourethrogram and retrograde urethrogram revealed an anterior urethral diverticulum [Figures 3a3c]. Computerised tomographic (CT) enterography and echocardiography were normal. Serum electrophoresis revealed an M band (0.69g/dl, 10.2%), while serum-free light chain assay showed an elevated serum-free kappa light chain (156.29 mg/L, reference 3.3–19.4 mg/L) and lambda light chain (378.38 mg/L, reference 5.710–26.3 mg/L) but a normal kappa/lambda ratio (0.41, reference 0.26–1.65), suggestive of monoclonal gammopathy of uncertain significance. Positron emission tomography (PET) scan did not reveal any site with increased metabolic uptake. Following a rheumatology- consult for RA, the patient was started on oral weekly methotrexate (15 mg) and daily naproxen (500 mg). He was counselled about the nature and course of his skin disease and was advised an annual follow-up.

Figure 1a:
Generalised skin laxity on the trunk with loose hanging skin on the chest.
Figure 1b:
Lax skin on the axilla.
Figure 2a:
Bilateral hyperinflated lung fields with bullous emphysematous changes in bilateral lower lobes (white arrows).
Figure 2b:
Coronal reformatted lung window images of HRCT chest showing centrilobular emphysematous changes in bilateral upper lobes (*), panlobular emphysematous changes in bilateral lower lobes (arrowheads)
Figure 2c:
Coronal reformatted lung window images of HRCT chest showing l fibrotic opacities in left lower lobe (dotted arrow).
Figure 3a:
Retrograde urethrogram shows retrograde filling in the smooth tubular dilatation of the left Cowper duct (white arrow), likely a simple Cowper duct syringocele.
Figure 3b:
Micturating cystourethrogram. Full bladder spot reveals normal bladder capacity and outline (*).
Figure 3c:
Micturating cystourethrogram. Full bladder reveals normal posterior urethra (dotted arrow) and the left simple Cowper duct syringocele (white arrow).

Cutis laxa is characterised by loose hanging skin that can be stretched easily and returns to its original position slowly (poor elastic recoil). Systemic involvement may be present in a variable proportion of patients. The underlying defect is an abnormal elastic fibre network in the skin and internal organs. Inherited forms can have various modes of transmission with overlapping clinical expressions and occur due to mutations in elastin, fibulin 5 and 4, or ATP6V0A2 genes. Acquired forms of cutis laxa are more common and have a later onset, usually in adulthood.1,2 Diagnosis is largely clinical, based on distinctive cutaneous findings. Generalised loose easily stretchable skin is also a characteristic feature of Ehlers Danlos syndrome, but the elastic recoil is normal.3 Skin biopsy in cutis laxa typically shows loss or fragmentation of elastic fibres, though normal histopathology does not exclude the diagnosis.4

Acquired cutis laxa can be classified as type I (generalised) or type II (localised). Generalised form often starts on the face (‘old man’ appearance) and progresses cephalocaudally. Aetiology remains uncertain, but several associations have been reported, including drugs, inflammatory skin and systemic diseases, amyloidosis, infections, and malignancies. There are reports of cutis laxa in patients with inflammatory arthritis, including a report of acral localised cutis laxa in a patient with long-standing RA. About half of the cases are associated with inflammatory dermatoses.13 Marshall syndrome is a distinct type of localised cutis laxa affecting children and occurs due to post-inflammatory elastolysis after neutrophilic dermatosis.4 Some patients with acquired disease may have internal organ involvement, such as emphysema, bronchiectasis, intestinal and urinary tract diverticula, genital prolapse, and vascular dilatations.1,2 In a series of 14 cases (9 males: 5 females, mean age- 56 years) of acquired cutis laxa with monoclonal gammopathy (four had multiple myeloma, six had monoclonal gammopathy of uncertain significance and four had heavy chain deposition disease), systemic manifestations included emphysema (n = 8), gastrointestinal (n = 6) and genitourinary involvement (n = 1).5

There is no effective treatment, though excision of lax skin can produce temporary cosmetic improvement. In contrast to the Ehlers–Danlos syndrome, cutis laxa does not involve vascular fragility. Therefore, surgery is not contraindicated as it does not adversely affect wound healing. Anecdotal reports have shown benefits with procedures including rhytidectomy and blepharoplasty but the effect is mostly temporary, and repeated procedures may be needed.6

To conclude, we present this case for its rarity. Dermatologists should perform a careful screening for underlying internal organ involvement and associated conditions [Table 1], including monoclonal gammopathies.

Table 1: Evaluation and screening investigations for patients with acquired cutis laxa
Triggers and associations

Past medical history of urticarial eruption, inflammatory skin disease, or any systemic illness

History of significant weight loss, B symptoms

Drug history

Serum and protein electrophoresis, serum-free light chain assay

ANA, RA factor, anti-CCP, and alpha1 antitrypsin levels (depending on clinical clues)

Systemic involvement

Pulmonary involvement: Chest X-ray, HRCT, Pulmonary function test

Cardiac involvement: ECG, Echocardiography, Computersied tomographic (CT) angiography

Gastrointestinal involvement: Ultrasound abdomen, Colonoscopy, CT enterography

Urogenital involvement: X-ray KUB, Retrograde and micturating cystourethrography

ANA: Antinuclear antibody; RA factor: Rheumatoid factor; KUB: Kidney, ureter, bladder; Anti-CCP: Anti cyclic citrullinated peptide; HRCT: High resolution computed tomography, ECG: electrocardiography.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of Artificial Intelligence (AI)-Assisted Technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

References

  1. , , . Cutis laxa. In: statPearls. StatPearls Publishing; .
    [Google Scholar]
  2. , , , , . Cutis laxa: A review. J Am Acad Dermatol. 2012;66:842.e1-17.
    [CrossRef] [PubMed] [Google Scholar]
  3. , , , . Acral localized acquired cutis laxa associated with rheumatoid arthritis. J Am Acad Dermatol. 2002;46:128-30.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , , , . Acquired cutis laxa type II (Marshall syndrome) in a 3-month-old boy. Pediatr Dermatol. 2022;39:312-3.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , , , . Cutis laxa associated with monoclonal gammopathy: 14 new cases and review of the literature. J Am Acad Dermatol. 2018;79:945-7.
    [CrossRef] [PubMed] [Google Scholar]
  6. , , . Acquired localized cutis laxa: A case report and the role of plastic surgery. Indian J Dermatol. 2019;64:55-8.
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]

Fulltext Views
794

PDF downloads
861
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections