Rare & Orphan Lab · DeCure for X

DeCure for Thiel-Behnke corneal dystrophy

DeCure's autonomous Rare AI scientist is researching a drug-repurposing hypothesis for Thiel-Behnke corneal dystrophy — screening already-approved drugs against its 2-gene Open Targets disease module to publish open-access research. Research is fast; the path to publication is funded in milestone stages.

Disease module2 genesLead labRare & Orphan
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Rare & OrphanDOID:0060455$DeCureRare

The disease map

Disease moduleThiel-Behnke corneal dystrophy maps to a 2-gene Open Targets module — the target space DeCure's AI scientist screens approved drugs against.
DeCure.ai methodSignature reversal (LINCS) plus network proximity (STRING) rank already-approved drugs likely to perturb this module — the same engine that produces DeCure.ai's repurposing hypotheses.
Repurposing thesisScreening approved medicines against this disease module, then publishing the evidence for the strongest candidate. Known pharmacology and human exposure data make the first question sharper — they do not establish safety or efficacy in a new indication.

Research record

01
ResearchComing soon
Candidate research + dossier — target rationale, drug-repurposing thesis and evidence pack.proof: Published dossier + on-chain hash
02
ValidationComing soon
In-vitro biological validation at a contract research org (CRO).proof: CRO contract + in-vitro report
03
Peer review & paperComing soon
Peer-reviewed paper published open-access (preprint + journal).proof: DOI + open-access link + on-chain hash

Current lead

No approved-drug candidate for thiel-behnke corneal dystrophy is corroborated in the literature DeepSearch retrieved. Some conditions are managed with non-pharmacological care — a device, surgery or physical therapy — rather than a medicine; that may be the case here, or the literature we found may simply be too sparse yet to support a drug-repurposing angle.

Molecular view

tubulin folding cofactor D (TBCD)TBCD is one of the genes genetically linked to this disease in Open Targets — shown as context, not as a drug target we're pursuing: no approved-drug candidate for this disease is yet corroborated in the literature we found.

Loading structure…
helix sheet gdpdrag to rotate · scroll to zoom

RCSB Protein Data Bank · entry 9M1M · 2.21 Å · ligand GUANOSINE-5'-DIPHOSPHATE (GDP). Experimental structure, not a prediction.

What the evidence adds up to

In a retrospective study of 4 patients (8 eyes) who underwent phototherapeutic keratectomy and 1 patient (2 eyes) who underwent penetrating keratoplasty for Thiel-Behnke corneal dystrophy linked to the 10q23-q24 locus, all 10 eyes showed biomicroscopic evidence of central recurrence over a follow-up ranging from 8 months to 25 years (mean 9.7 years). Despite universal recurrence, functional central visual acuity was maintained in all eyes, ranging from 20/25 to 20/80. Phototherapeutic keratectomy provided improved vision for a mean of 3.7 years (range 8 months to 8 years). The peripheral-to-central progression of lesions suggested an epithelial origin for the dystrophy.

Electron microscopy has been used to confirm Thiel-Behnke dystrophy after recurrence, and the condition is now classified as corneal dystrophy of Bowman’s layer type II (honeycomb pattern), distinct from Reis-Bucklers dystrophy. A 2025 case report described a woman in her early 50s with progressive vision loss and a history of childhood recurrent corneal erosions, diagnosed by anterior segment OCT showing a sawtooth pattern in Bowman’s layer. She was managed conservatively for the dystrophy and underwent cataract surgery in the left eye, which significantly improved her vision. The report emphasised that Thiel-Behnke dystrophy with cataract can severely impair vision and requires early diagnosis and monitoring.

A 2018 study of a method combining descemetorhexis and accelerated collagen crosslinking for endothelial corneal dystrophy reported corneal transparency restoration in 66.6% of primary cases, attributed to peripheral endothelial cell migration. The method was ineffective for secondary endothelial dystrophy, and the reasons for failure were not clear. This study did not address Thiel-Behnke dystrophy, which is an epithelial-stromal TGFB1 dystrophy, not an endothelial condition.

What is still missing are prospective trials comparing phototherapeutic keratectomy with other surgical approaches specifically for Thiel-Behnke dystrophy, studies testing topical or systemic therapies that might delay recurrence, and any investigation of genetic or molecular targets given the known TGFB1 mutation. No drug treatment for Thiel-Behnke corneal dystrophy has been tested in a controlled trial, and the evidence base remains limited to small retrospective surgical series and single case reports.

Evidence

Retrieved by DeepSearch across 234,678,978 indexed works and resolved on OpenAlex — ranked by citations, including the results that did not work.

Cornea · 2004 · 24 citations

Recurrence of Chromosome 10 Thiel-Behnke Corneal Dystrophy (CDB2) After Excimer Laser Phototherapeutic Keratectomy or Penetrating Keratoplasty

AbstractPURPOSE: To evaluate the recurrence of Thiel-Behnke dystrophy (linked to the 10 q23-q24 locus) after phototherapeutic keratectomy or penetrating keratoplasty. METHODS: This is a retrospective study of 4 patients (8 eyes) who underwent phototherapeutic keratectomy and 1 patient (2 eyes) who underwent penetrating keratoplasty. Best corrected visual acuity was assessed, and biomicroscopic examinations for evidence of recurrent dystrophy were documented and photographed. The location, lesion distribution, and lesion pattern of any recurrence was noted. RESULTS: Follow-up ranged from 8 months to 25 years (mean +/- SD 9.7 +/- 7.97 years). All 10 eyes showed biomicroscopic evidence of central recurrence. Six eyes showed an intermediate zone of honeycomb opacities as well as a peripheral zone of focal and geographic lesions. Despite the high incidence of recurrence, functional central visual acuity was maintained. All eyes maintained functional best corrected visual acuity (ranging from 20/25 to 20/80) despite the postoperative recurrence. CONCLUSION: Recurrence of Thiel-Behnke corneal dystrophy is extremely high after either phototherapeutic keratectomy or penetrating keratoplasty. Despite the high incidence of recurrence, the central cornea is the last to be affected. The peripheral-to-central progression of the lesions points to an epithelial origin for the pathogenesis of the dystrophy. Phototherapeutic keratectomy in the treatment of Thiel-Behnke corneal dystrophy offers a safe and effective treatment modality, providing patients up to 8 years of improved vision ranging from 8 months to 8 years (mean +/- SD 3.7 +/- 2.7 years) and delaying or circumventing the need for more invasive intraocular surgical intervention.

https://doi.org/10.1097/01.ico.0000126442.61088.a8
British Journal of Ophthalmology · 2005 · 7 citations · open access

Recurrence of Thiel-Behnke corneal dystrophy: an electron microscopic study

AbstractThe hereditary Thiel-Behnke corneal dystrophy, first described in 1967,1 is frequently confused with Reis-Bucklers dystrophy. Both conditions affect Bowman’s layer and can be difficult to differentiate both clinically and histopathologically. Previously, electron microscopic studies of corneal dystrophies affecting Bowman’s layer identified characteristic “curly fibres,” but it was unclear whether these were diagnostic of Thiel-Behnke or Reis-Bucklers.2 More recently, Kuchle et al have attempted to clarify the situation, identifying these corneal dystrophies as two separate disease entities, and proposing the less confusing nomenclature of corneal dystrophy of Bowman’s layer (CDB) type 1 (geographic or “true” Reis-Bucklers dystrophy) and CDB type II (honeycomb or Thiel-Behnke dystrophy).3 In this report we present a case of Thiel-Behnke dystrophy diagnosed by electron microscopy after recurrence in the corneal …

https://doi.org/10.1136/bjo.2004.051029
Ophthalmology Reports · 2018 · 1 citations · open access

Comparative assessment of the efficacy of primary and secondary corneal endothelial dystrophy treatment by isolated descemetorhexis and accelerated collagen crosslinking method

AbstractThe article examines the efficacy of the authors method of endothelial corneal dystrophy treatment, inclu ding descemetorhexis and accelerated collagen crosslinking. In primary endothelial dystrophy, corneal state improvement and restoration of its transparency were observed in 66.6% of cases (due to migration of endothelial cells from the periphery to the сentral zone). In secondary endothelial dystrophy, the treatment method described in the present article is ineffective, and the reasons for failures are not quite clear and require further investigation.

https://doi.org/10.17816/ov11241-47
Figshare · 2025 · 0 citations · open access

Supplementary Material for: A CASE REPORT OF SUCCESSFUL CATARACT SURGERY IN THEIL-BEHNKE CORNEAL DYSTROPHY: A VISUAL REHABILITATION FOR THE PATIENT

AbstractIntroduction Thiel-Behnke corneal dystrophy is a rare inherited condition characterized by symmetrical subepithelial corneal opacities that gradually reduce vision. It is an autosomal dominant inherited epithelial stromal TGFB1 dystrophy that mainly causes visual impairment. Case Presentation This case report describes case of a woman in her early 50s with progressive vision loss in her left eye over the past three months with a history of recurrent corneal erosions in childhood. Examination on a slit lamp showed corneal dystrophy affecting both eyes, matching the features of Thiel-Behnke dystrophy. Diagnosis was confirmed with Anterior Segment - Optical Coherence Tomography (AS-OCT), revealing a distinctive sawtooth pattern in Bowman's layer. The patient was managed conservatively for her dystrophy and was also surgically treated for her cataract in the left eye which showed a significant improvement in her vision. Conclusion Despite its rarity, Thiel-Behnke dystrophy along with cataract can severely impair vision, making early diagnosis and continuous monitoring crucial for preventing further visual decline.

https://doi.org/10.6084/m9.figshare.28682711
Figshare · 2025 · 0 citations · open access

Supplementary Material for: A CASE REPORT OF SUCCESSFUL CATARACT SURGERY IN THEIL-BEHNKE CORNEAL DYSTROPHY: A VISUAL REHABILITATION FOR THE PATIENT

AbstractIntroduction Thiel-Behnke corneal dystrophy is a rare inherited condition characterized by symmetrical subepithelial corneal opacities that gradually reduce vision. It is an autosomal dominant inherited epithelial stromal TGFB1 dystrophy that mainly causes visual impairment. Case Presentation This case report describes case of a woman in her early 50s with progressive vision loss in her left eye over the past three months with a history of recurrent corneal erosions in childhood. Examination on a slit lamp showed corneal dystrophy affecting both eyes, matching the features of Thiel-Behnke dystrophy. Diagnosis was confirmed with Anterior Segment - Optical Coherence Tomography (AS-OCT), revealing a distinctive sawtooth pattern in Bowman's layer. The patient was managed conservatively for her dystrophy and was also surgically treated for her cataract in the left eye which showed a significant improvement in her vision. Conclusion Despite its rarity, Thiel-Behnke dystrophy along with cataract can severely impair vision, making early diagnosis and continuous monitoring crucial for preventing further visual decline.

https://doi.org/10.6084/m9.figshare.28682711.v1
Journal of the Foundations of Ophthalmology · 2023 · 0 citations

An Overview of Epithelial and Stromal Corneal Dystrophies

AbstractPrincipally, corneal dystrophies are a set of conditions that impact corneal transparency and distorts corneal structure. They are commonly bilateral, progressive, and differs from corneal degeneration as they are often inherited through autosomal dominant or recessive modes as well as x-linked modes. Several genes have been implicated in corneal dystrophies and will be elaborated on in the description of dystrophies in this article. Management of corneal dystrophies depend on severity of symptoms and require specialist input. Mildly symptomatic dystrophies do not warrant management. However, in those with progressive disease and/or poor vision, surgical options may be of use. Techniques commonly involved in the management of dystrophies include deep lamellar endothelial keratoplasty, penetrating keratoplasty, or the area of disease is ablated using a photo-therapeutic keratectomy.

https://doi.org/10.48089/jfo7683114

Disease module: DeepOracle (Open Targets). Structures: RDKit from PubChem SMILES. Literature: retrieved by DeepSearch across 234,678,978 indexed works (targeted per-candidate search), resolved on OpenAlex.

DeCure is a research and publication project, not medical advice and not a treatment. "DeCure for X" describes a research goal, not a claim that a cure exists. Backing a cure is a contribution to fund the research — it is not an investment, and confers no yield, royalty, equity or IP ownership. Papers are published open-access by the DeCure.ai DAO.