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July 2022     Volume 6 Issue 4 
 

(c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 341 

SHORT COMMUNICATION 
 

 

Clinicopathologic Correlation: Differentiating Between Localized 
Lichen Myxedematosus And Scleromyxedema 
 

Kamran K. Harper, MD1, Lauryn M. Falcone MD, PhD1, Kevin T. Savage, MD1, Alaina J. James, 
MD, PhD1 
 
1 University of Pittsburgh Department of Dermatology/UPMC, Pittsburgh, PA  
 

 
 

Scleromyxedema, also known as 
sclerodermoid or generalized lichen 
myxedematosus, is a rare type of primary 
cutaneous dermal mucinosis associated with 
monoclonal gammopathy, systemic 
symptoms, and normal thyroid studies.1 
Clinically, scleromyxedema can have a 
variable presentation with potential for 
relapse. Histologically, scleromyxedema can 
be indistinguishable from localized lichen 
myxedematosus (LM).2 Unlike patients with 
LM, patients with scleromyxedema may 
develop severe complications such as 
dermato-neuro syndrome, which is a 
potentially fatal condition characterized by a 
flu-like prodrome followed by fever, seizures, 
and coma.3 

 
It is important to differentiate 
scleromyxedema from LM early due to the 
risk of progressive systemic disease. IVIG is 
generally regarded as first-line therapy for 
scleromyxedema.  Second line-agents 
include thalidomide or lenalidomide paired 
with systemic glucocorticoids. Melphalan, 
Bortezomib with dexamethasone, or 
autologous hematopoietic stem cell 
transplantation can be utilized for severe or 
refractory disease.4 

 
We report the case of a 38-year-old female 
with chronic arthritis who presented with an 

11-month history of biopsy-proven LM that 
was recalcitrant to treatment with topical 
steroids and methotrexate. On physical 
exam, she had numerous, 2mm, 
monomorphic, dome-shaped papules on the 
lateral cheeks, upper dorsolateral arms, and 
anterior legs. Repeat punch biopsy of the 
right upper arm demonstrated increased 
mucin deposition separating thickened 
collagen fibers and increased fibroblasts in 
the superficial dermis consistent with 
“scleromyxedema/lichen myxomatous.”  
 
Upon further review of her medical records, 
our patient had a history of epilepsy and 
monoclonal IgG gammopathy. Given the 
patient’s constellation of symptoms and prior 
medical history, she was diagnosed with 
scleromyxedema. We referred her to 
oncology and began treatment with IVIG, 
after which she experienced an improvement 
in symptoms and complete resolution of her 
skin lesions.  
 
Within 5 months of discontinuing IVIG, our 
patient’s skin disease relapsed. On repeat 
examination, she had scattered 2mm dome-
shaped papules on the dorsal hands similar 
to her prior presentation. She also had 
scattered 5-8mm faintly violaceous macules 
on the upper and lower dorsal extremities. 
Biopsies of both lesion types revealed mildly 



SKIN 
 

July 2022     Volume 6 Issue 4 
 

(c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 342 

increased fibroblasts and dermal mucin, 
reconfirming the diagnosis of 
scleromyxedema. Our patient is scheduled to 
reinitiate IVIG therapy with the goal of 
achieving clearance of her skin lesions.  
 
In the absence of long-term, continuous 
treatment, scleromyxedema has the potential 
to progress and evolve clinically.4 

Scleromyxedema can be distinguished from 
LM through careful history and physical 
exam. Earlier correlation of this patient’s 
dermatologic presentation with her systemic 
signs including monoclonal gammopathy, 
epilepsy, and arthritis may have prompted 
sooner initiation of the appropriate treatment 
for scleromyxedema. Given that prognosis 
and management of LM and 
scleromyxedema differ, patients with 
histologic evidence of a cutaneous mucinosis 
should be screened for evidence of systemic 
involvement.  
 
Conflict of Interest Disclosures: None 
 
Funding: None 
 
Corresponding Author: 
Alaina J. James, MD, PhD 
University of Pittsburgh  
Department of Dermatology/UPMC 3601  
Fifth Avenue Suite 5A, Pittsburgh, PA, 15213 
Email: jamesaj@upmc.edu

 
 
References: 
1. Rongioletti F, Rebora A. Updated classification of 

papular mucinosis, lichen myxedematosus, and 
scleromyxedema. J Am Acad Dermatol. 
2001;44(2):273-281. 
doi:10.1067/mjd.2001.111630 

2. Rongioletti F, Merlo G, Carli C, et al. 
Histopathologic characteristics of 
scleromyxedema: A study of a series of 34 cases. 
J Am Acad Dermatol. 2016;74(6):1194-1200. 
doi:10.1016/j.jaad.2015.12.021 

3. Fleming KE, Virmani D, Sutton E, et al. 
Scleromyxedema and the dermato-neuro 
syndrome: case report and review of the literature. 
J Cutan Pathol. 2012;39(5):508-517. 
doi:10.1111/j.1600-0560.2012.01882.x 

4.  Knobler R, Moinzadeh P, Hunzelmann N, et al. 
European dermatology forum S1-guideline on the 
diagnosis and treatment of sclerosing diseases of 
the skin, Part 2: Scleromyxedema, scleredema 
and nephrogenic systemic fibrosis. Journal of the 
European Academy of Dermatology and 
Venereology. 2017;31(10):1581-1594. 
doi:10.1111/jdv.14466 

 

 

mailto:harperkk@upmc.edu