The University of Toledo Translation Journal of Medical Sciences 
Pulmonology Abstract, Department of Medicine Research Symposium UTJMS 2023 May 1; 11(1):e1-e1 

Hyperpigmentation in Systemic Sclerosis 

Gavin Kelly1*, Joshua Busken1, Ragheb Assaly, MD1, 

1Division of Pulmonology and Critical Care Medicine, Department of Medicine, The 
University of Toledo, Toledo, OH 43614 

*Corresponding author: Gavin.Kelly@rockets.utoledo.edu

Published: 05 May 2023 

Introduction: Systemic sclerosis (SSc) is a chronic multisystem autoimmune disorder that is associated 
with skin pigmentation disorders and vasculopathy. There seems to be a common origin to the 
development of vasculopathy and pigmentation disorders in SSc. A unique interplay exists between the 
potent vasoconstrictor peptide endothelin-1 and melanin synthesis. In this abstract, we present a case of 
diffuse hyperpigmentation and its relationship with recurring digital ulcers and severe Raynaud’s in an 
SSc patient. 

Methods: EMR records were gathered from the patients EMR and outpatient visits with patient consent. 
Images of the patient were taken with patient consent. The patient was physically seen throughout her 
hospital stay.   

Results: Hyperpigmentation, particularly diffuse hyperpigmentation, has previously been linked to 
increase incidence of digital ulcers in SSc patients. Endothelin-1 has been established as a key mediator 
of vasculopathy in systemic sclerosis patients by acting as a potent vasoconstrictor and by inducing 
differentiation of fibroblasts, ultimately leading to fibrosis. The clinical manifestation of this in SSc 
patients is microvascular disease. It is hypothesized that increased endothelin-1 in SSc patients play a 
role in increased melanin synthesis in these patients. This is a lesser known feature of SSc, and both 
patients and healthcare providers should be educated of this association.   

Conclusion: Hyperpigmentation is a rare feature of SSc, but when present, may indicate that the patient 
is more likely to develop future digital ulcers. This is a feature of SSc that should not be overlooked as it 
can potentially lead to earlier management of disease. 

https://dx.doi.org/10.46570/utjms.vol11-2023-777 

https://dx.doi.org/10.46570/utjms.vol11-2023-777
mailto:Gavin.Kelly@rockets.utoledo.edu