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
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
Reviewers 2022
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 Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Letter

Scleromyxedema: A rare case report with dermoscopic findings

Department of Dermatology, Government Medical College, Bhavnagar, Gujarat, India
Department of Dermatology, Dermatopathology Research Center, Vyara, Tapi, Gujarat, India
Corresponding author: Dr. Arjun Prakashey, Department of Dermatology, Government Medical College, Bhavnagar, Gujarat, India.
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: Prakashey A, Mehta H, Bharti AH, Agharia R. Scleromyxedema: A rare case report with dermoscopic findings. Indian J Dermatol Venereol Leprol doi: 10.25259/IJDVL_740_2022

Dear Editor,

Cutaneous mucinosis is a heterogenous group of disorders which are characterised by an abnormal mucin deposition in skin. Scleromyxedema, also known as generalised papular and sclerodermoid lichen myxedematosus, is a cutaneous mucinosis characterised by a generalised papular and sclerodermoid eruption, a triad of increased fibroblast proliferation, mucin deposition and fibrosis. Monoclonal gammopathy is commonly associated.

A 50-year-old Indian male presented with an insidious onset and gradually progressive eruption of erythematous papules associated with thickening of skin primarily over the face and a tender swelling in the central aspect of the face. The patient did not have a history of any systemic disease, nor did he have any other systemic complaints on enquiry.

On cutaneous examination, the patient had a diffuse erythematous, mildly edematous, tender plaque on centrofacial area involving the glabella, and root of the nose giving rise to leonine facies [Figure 1a]. The chin also had a diffuse infiltration with shiny surface and a background yellowish hue. There was infiltration and thickning of both ears with a shiny appearance of the overlying skin. Additionally, there were sheets of firm, waxy, lichenoid to brownish papules, with few making linear configuration over the back of the neck, trunk, and proximal extremities [Figures 1b and 1c]. Increased furrowing of skin on the back (Shar-Pei Sign) and abdominal areas was noted. Both proximal interphalangeal joints had tightening of skin with central depression and an elevated rim (doughnut sign).

Figure 1a:: Centrofacial erythematous plaque with infiltration of forehead with glabella (leonine facies) and chin
Figure 1b:: Papular eruption over the back with furrowing of creases visible on lower back (Shar-Pei sign)
Figure 1c:: Central depression over proximal interphalangeal joint with elevated rim (doughnut sign)

Dermoscopic examination of the glabella (Dermlite DL4 dermatoscope–3 Gen Inc., San Juan Capistrano, CA, USA–×10 magnification, polarised mode) [Figure 2] revealed localised yellowish brown and yellowish white roundish structureless areas with interspersed linear irregular vessels and few out of focus vessels and accentuated pigmentary network at few areas. There was a relative alopecia with vellus hair showing peripilar scales and few white and black dots.

Figure 2:: Dermoscopy of the glabella showing localised yellowish brown and yellowish white structureless area (black arrow), ill focused linear irregular vessels (yellow arrow) accentuated pigmentary network (green arrow), clustered whitish round homogenous areas leading to sago grain like appearanced due to eccrine openings (Black circles)

Dermoscopy of lesion on neck [Figure 3] showed glistening yellowish white round to oval areas with loss of pigmentary network (few coalescing into a linear array with accentuation of pigmentation in the periphery).

Figure 3:: Dermoscopy from papular lesions over the neck showing glistening yellowish white, round to oval areas (coalescence into a linear array is prominent with accentuation of pigmentation in the periphery)
Figure 3:: Dermoscopy from papular lesions over the neck showing glistening yellowish white, round to oval areas (coalescence into a linear array is prominent with accentuation of pigmentation in the periphery)

Systemic examination of the patient including the sensorimotor examination was normal.

Serum protein electrophoresis with immunofixation showed an IgG monoclonal gammopathy. Chest X-ray showed mediastinal lymph node enlargement. Spirometry revealed a mild (30–50%) restrictive pattern but high resolution commuted tomography (HRCT) of chest did not show any fibrotic changes. Electrocardiogram was low voltage with a mild left axis deviation.

Histopathological examination of the glabellar skin was suggestive of scleromyxedema, as shown [Figures 4a and 4b].

Figure 4a:: White arrow displays epidermal acanthosis and hyprpigmentation of the basal layer which corresponds to the accentuation of pigment network. The red arrow displays presence of dilated capillaries which correspond dermoscopically to the telangiectasia and linear curved vessels. The yellow arrows suggest areas of deposition of mucinous material. (Haematoxylin and eosin stain, × 10 magnification)
Figure 4b:: There is presence of fibroplasia and bluish material in the mid and deep dermis denoted by yellow arrows, which on alcian blue stain displays bluish discoloration suggestive of mucin depositions. (Alcian blue stain at pH 2.5, × 10 magnification)

On the basis of the above findings, a provisional diagnosis of scleromyxedema was considered. Opinion from a hemato-oncologist as well as a rheumatologist was sought and the patient was advised a bone marrow aspiration and biopsy.

Strikingly, our patient did not show any signs or symptoms of systemic involvement at the time of diagnosis, but long term follow-up is still essential.

Previously, Mendes Bastos P et al. had described clusters of round white-ivory homogenous areas resembling rice grains, which they reported to correlate well to dermal fibroblast proliferation and collagen accumulation.1 Dermoscopically, we were also able to notice yellowish-white round structureless areas which seem to correlate well with mucin deposition on histopathology along with pigmentary and vascular changes [Table 1].

Table 1:: Correlation between the dermoscopic and histopathological features of scleromyxedema
Sr Dermoscopy Histopathology
1 Yellowish white structureless areas. Interstitial fibrosis and mucin deposition.
2 Linear and irregular vessels. Dilated capillary in the papillary dermis.
3 Accentuated pigmentary network. Irregular epidermal acanthosis and basal layer hypermelanisation.
4 Erythema in the periphery. Superficial peri vascular, peri appendageal infiltrate.

Scleromyxedema is a chronic and unpredictable disease associated with monoclonal gammopathy and severe systemic implications including neurologic, haematologic and cardiologic involvement leading to a guarded prognosis. However, our patient had no clinically evident systemic involvement with presence of classic cutaneous lesions.

Declaration of patient consent

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

Financial support and sponsorship


Conflict of interest

There are no conflicts of interest.


  1. , , , . Dermoscopy and reflectance confocal microscopy for the diagnosis of scleromyxedema. JAAD Case Rep. 2019;5:451-3.
    [CrossRef] [Google Scholar]

Fulltext Views

PDF downloads
View/Download PDF
Download Citations
Show Sections