Primary Systemic (Amyloid Light-Chain) Amyloidosis Masquerading as Pseudoxanthoma Elasticum
Bibliographic record
Abstract
IMPORTANCE: Primary systemic (amyloid light-chain [AL]) amyloidosis has a variety of cutaneous manifestations. In this case, we present a novel clinicopathological pattern of AL amyloidosis. OBSERVATIONS: A woman in her 50s with a history of AL amyloidosis manifesting as macroglossia and bilateral carpal tunnel syndrome presented with skin-colored to yellow cobblestoned plaques to the neck and bilateral antecubital fossa. Although clinical similar to pseudoxanthoma elasticum (PXE), the skin changes were found to be due to amyloid deposition primarily around the pilosebaceous unit but also within the papillary and reticular dermis. Previous reports of PXE-like plaques in AL amyloidosis have been reported as part of a very rare entity termed amyloid elastosis. However, our case demonstrates several important clinical and pathological differences from this entity. Most notably, there was no dermal elastic fiber involvement, limited cutaneous and systemic involvement, and a fairly indolent course with better response to treatment. CONCLUSIONS AND RELEVANCE: Identification of this atypical presentation of AL amyloidosis has important implications for early detection and rapid treatment. The lack of elastic fiber involvement establishes the uniqueness of this case, and further study may be required to determine if this histological finding has prognostic significance.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".