Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
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
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
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 - 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
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
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
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor
doi: 10.4103/0378-6323.140310
PMID: 25201849

Acquired cutis laxa of face with multiple myeloma

Tulika A Yadav, Atul M Dongre, Uday S Khopkar
 Department of Dermatology, Seth GS Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Tulika A Yadav
Department of Dermatology, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra
How to cite this article:
Yadav TA, Dongre AM, Khopkar US. Acquired cutis laxa of face with multiple myeloma. Indian J Dermatol Venereol Leprol 2014;80:454-456
Copyright: (C)2014 Indian Journal of Dermatology, Venereology, and Leprology


Cutis laxa is a rare dermatosis characterized by diffuse laxity of skin resulting in a prematurely aged appearance. It is most commonly an inherited condition. The acquired form is rare and has been associated with various conditions, including multiple myeloma, monoclonal gammopathy of undetermined significance, and heavy chain deposition disease. Cutis laxa usually follows a progressive course and there is no definitive treatment available. [1] We describe a 44-year-old female with multiple myeloma and cutis laxa localized to the face. She developed anasarca, which subsided following treatment for multiple myeloma leaving lax and wrinkled skin all over the body. Lax skin improved spontaneously elsewhere but persisted on the face.

A 44-year-old female presented to us with a two-year history of loosening of skin over the face. Two years back, she had developed severe anaemia with associated anasarca and exertional dyspnea. She also had heavy proteinuria, hypoproteinemia and progressive renal failure. A kidney biopsy was performed which revealed mesangial cell proliferation and lobular accentuation of glomerular tufts with marked mesangial matrix deposition and eosinophilic periodic acid Schiff (PAS) positive nodules. Immunofixation electrophoresis on a 24-hour urine sample was positive for kappa light chains. Serum immunofixation electrophoresis (IFE) revealed a stable monoclonal IgG-λ M-spike (0.5 g/dL). Serum β2 -microglobulin was increased. Bone marrow examination revealed 14% plasma cells. Based on these findings, a diagnosis of early multiple myeloma with nephrotic syndrome was made. She was started on a chemotherapy regimen consisting of thalidomide (100 mg/day), oral cyclophosphamide (150 mg/day) both for the first five days in every month with weekly intravenous dexamethasone (40 mg/week) along with loop diuretics. In addition, the patient was prescribed bortezomib which was discontinued by the patient after two months. Following one year of treatment, the anasarca subsided leaving behind loose wrinkled skin all over the body. Over the next one year, the skin over the extremities and the abdomen reverted back to normal; however, loosening of skin persisted over the face.

Examination revealed a healthy-appearing woman, who appeared older than her stated age [Figure - 1]. She had loose, sagging skin on the face and neck giving her a hound-dog facies [Figure - 2]. Skin over the ear lobules was lax. On stretching of the skin over face, no elastic recoil was seen. Her systemic examination was also normal. Hematoxylin and eosin (H and E) stained sections from a skin biopsy from the face showed a mixed cell infiltrate of lymphocytes and occasional neutrophils [Figure - 3]. A special stain to demonstrate elastic tissue (Verhoff van Gieson) showed fragmented and clumped elastic fibers [Figure - 4].

Figure 1: Loose sagging folds of skin over the face
Figure 2: Loose folds of skin over the neck
Figure 3: A sparse infiltrate of lymphocytes and few histiocytes are seen in the dermis seen on H and E, (×100)
Figure 4: VVG stained sections showing clumped and fragmented elastic fibers (×400)

Based on her clinical features, a diagnosis of acquired cutis laxa localized to the face following multiple myeloma was made and she was referred to a plastic surgeon for surgical correction of redundant folds.

Cutis laxa is a rare condition characterized clinically by lax, pendulous skin and histologically by loss of elastic tissue in the dermis. It is a heterogeneous condition, which may be inherited as a dominant, recessive or X-linked recessive disease, or it may be acquired. The face and neck are often affected, which produces a ′bloodhound′ appearance of premature aging. Mild trauma can lead to purpura due to skin fragility and this may lead to formation of fibrotic nodules over the bony prominences. There may be widespread, massive folds of lax skin, or the changes may be mild and confined to a limited area. Such cases may be confused as having anetoderma. The acquired cephalic form of localized cutis laxa presents characteristically as large, hanging loose ear lobes, blepharochalasis and lax nasolabial folds. Organs other than the skin may also be involved. Internal organ abnormalities like emphysema, gastric fibromas and tracheobronchomegaly have been reported. [2]

Histologically, the skin is of normal thickness, but the elastic fibers are sparse, short, fragmented and clumped, particularly in the upper dermis, and they show granular degeneration. [3] The elastic fibers are found to be deficient in elastin, though their microfibrils appear normal. Similar changes in elastic fibers may occur in the lungs and aorta. [4],[5] There are occasional case reports of multiple myeloma and other plasma cell dyscrasias associated with cutis laxa. [6]

It is likely that paraproteinemia resulting in the deposition of immune complexes possibly leads to the release of inflammatory cytokines and subsequent destruction of elastic fibers. Surgical repair seems to be the only therapeutic choice, but the results are variable and temporary.

Ferrándiz-Pulido C, Serra M, Bel S, Ferrer B, Repiso T, Garcia-Patos V. Multiple myeloma-associated amyloidosis presenting with acrolocalized acquired cutis laxa. Arch Dermatol 2010;146:1433-4.
[Google Scholar]
Wanderer AA, Ellis EF, Goltz RW, Cotton EK. Tracheobronchomegaly and acquired cutis laxa in a child. Physiologic and immunologic studies. Pediatrics 1969;44:709-15.
[Google Scholar]
Hashimoto K, Kanzaki T. Cutis laxa. Ultrastructural and biochemical studies. Arch Dermatol 1975;111:861-73.
[Google Scholar]
Marchase P, Holbrook K, Pinnell SR. A familial cutis laxa syndrome with ultrastructural abnormalities of collagen and elastin. J Invest Dermatol 1980;75:399-403.
[Google Scholar]
Sephel GC, Byers PH, Holbrook KA, Davidson JM. Heterogeneity of elastin expression in cutis laxa fibroblast strains. J Invest Dermatol 1989;93:147-53.
[Google Scholar]
New HD, Callen JP. Generalized acquired cutis laxa associated with multiple myeloma with biphenotypic IgG- λ and IgA-κ gammopathy following treatment of a nodal plasmacytoma. Arch Dermatol 2011;147:323-8.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections