Elastoderma

Elastoderma is characterized by the specific and unusual collection of elastic fiber tissues in the dermis, a condition that, while rare, is further complicated by a lack of effective intervention, mostly because the cause is not clearly established. Protheragen specializes in the preclinical development of novel therapies for rare connective tissue disorders, including elastoderma.

Introduction to Elastoderma

Elastoderma affects the skin in a very specific way. It is an acquired condition of the skin known colloquially as cutis laxa. Balanced stretching of the skin is observed alongside a slow return to the original state, which occurs in specific regions including the neck, arms, and torso. Different from hereditary cutis laxa forms, this condition does not present alongside other systemic conditions or genetic disorders linked to the elastin gene. While most skin disorders in the medical field are well documented, elastoderma's scarcity only underlines further the need to not only understand its underlying mechanisms but create effective therapeutic interventions.

Fig.1 Histological and ultrastructural findings of elastic tissue abnormalities. (de Waal et al., 2022)

Pathogenesis of Elastoderma

Pathogenesis of elastoderma stems from the disruption of the equilibrium of elastic tissue maintenance. The main changes are the pathological increase of grotesque and disorganized elastic fibers within the reticular dermis. This may be the result of excess elastin synthesis by dermal fibroblasts or an abnormal breakdown of these fibers by enzymes, such as elastase. The skin undergoes changes in its topography, structural support, and loss of the normal elastic recoil leading to skin sagging and laxity. As of now, the precise mechanisms leading to these changes remain uncertain.

Therapeutics Development for Elastoderma

Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.

Our Services

Protheragen offers a comprehensive suite of preclinical solutions to accelerate therapeutic development for elastoderma. Our team of specialists in dermatology, connective tissue disorders, and molecular biology will work closely with your project leaders to advance therapeutic development and disease model development specifically tailored to this disorder, leveraging our state-of-the-art technologies.

Therapeutic Development Platforms for Elastoderma

Protheragen's platforms integrate small molecule therapeutics targeting fibroblast activity, biologics aimed at regulating extracellular matrix homeostasis, and specialized formulations for dermal delivery. Our approach is designed to address the multifaceted pathogenesis of elastoderma, from the molecular level of elastin regulation to the observable skin phenotype.

• Small molecule drug
• Cell therapy
• Gene therapy

• Therapeutic antibody
• Therapeutic peptide
• Therapeutic protein

Disease Model Development for Elastoderma

Protheragen has significant expertise in developing and utilizing preclinical models for elastoderma, which include 2D cell models, 3D skin models, as well as animal models. These models are designed to recapitulate key pathophysiological features of the disease, such as excessive elastin accumulation and disorganized dermal architecture, aiding in the evaluation of potential therapies.

• Patient-derived elastoderma fibroblasts
• Elastin cross-linking deficient dermal constructs
• 3D matrix deposition co-cultures

• Fibulin-5 deficient mouse
• LOXL1 transgenic rat
• Elastase-induced elastosis rabbit

DMPK and Drug Safety Evaluation Services for Elastoderma

Protheragen's DMPK and drug safety evaluation services provide critical insights into how your drug is absorbed, distributed, metabolized, and excreted. Our services help to predict in vivo behavior and optimize dosing strategies for elastoderma therapies, with a particular focus on dermal penetration and tissue distribution of drug candidates.

Protheragen's expertise in rare skin disorders and connective tissue pathologies ensures a seamless translation from target discovery to IND-enabling studies. We can provide end-to-end services from discovery through  disease modeling to DMPK and drug safety evaluation services.

If you are interested in our services, please feel free to contact us.

References

• de Waal, A. C., et al. "Elastoderma: An Uncommon Cause of Acquired Hyperextensible Skin." Acta Derm Venereol 92.3 (2012): 328-9.
• Mansour, A., et al. "Giant Diaphragmatic Hernia in Elastoderma." Isr Med Assoc J 24.2 (2022): 121-22.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.