Degos disease

Name and code equivalence

Record source-specific use of Degos disease, Köhlmeier-Degos disease and malignant atrophic papulosis, including whether each label covers cutaneous-only disease.

1. Does the cited terminology use malignant atrophic papulosis for the full disease spectrum or specifically for systemic disease? definition
2. Which ICD-10, ICD-11 and SNOMED CT mappings are verified in a named release, and which are broader than Degos disease? provenance

Criteria and mimics

Require an attributed diagnostic framework and an explicit distinction between Degos disease and a Degos-like lesion pattern.

1. Which published criteria or expert assessment support the diagnosis, and what issuing body, publication date or revision applies? provenance
2. How were Dowling-Degos disease, lupus-associated lesions and other occlusive vasculopathies assessed as alternatives? boundary

Documented lesion pattern

Retain dated descriptions or photographs and distinguish newly appearing lesions from residual atrophic marks.

1. Which lesions show a depressed porcelain-white centre and surrounding erythema, and where are they distributed? measurement
2. How many new lesions appeared during the stated interval, and which observations document their evolution? measurement

Sample-specific support

Capture reported vascular occlusion and tissue injury without treating an isolated pathology phrase as sufficient diagnosis.

1. Which vascular and tissue changes did the pathologist report, and what lesion age and sampling depth were documented? provenance
2. Does the report support Degos disease, remain nonspecific or favour an alternative when correlated with the clinical findings? boundary

Internal involvement assessment

Prioritise gastrointestinal and neurological assessment while allowing other organ findings and competing explanations.

1. What evidence supports or argues against gastrointestinal or central nervous system involvement, and when was it obtained? measurement
2. Which internal-organ abnormalities are attributed to Degos disease by a clinician, and which remain unexplained or attributable to another condition? boundary

Time-qualified disease extent

Maintain dated extent assessments without assuming a fixed stage sequence or permanent benign course.

1. How long has disease been observed since skin onset, and which systemic assessments support the current extent label? measurement
2. What new evidence would require revising a cutaneous-only classification to systemic or indeterminate? action

Mechanism evidence status

The underlying cause remains uncertain; record proposed mechanisms as attributed hypotheses. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)

1. What evidence supports complement activation, endothelial injury or thrombosis in the cited study, and does it demonstrate cause or association? provenance
2. Are autoimmune or familial findings evidence about primary Degos disease, an associated condition or a Degos-like phenotype? boundary

Rarity with denominators

Degos disease is described as extremely rare; published case counts must not be interpreted as prevalence. [NORD](https://rarediseases.org/rare-diseases/degos-disease/)

1. Is the frequency claim a published case count, registry count, prevalence estimate or incidence estimate, and what population and year does it cover? measurement
2. How could referral patterns, diagnostic uncertainty and publication of severe cases distort the reported age distribution or systemic disease proportion? provenance

Warning-sign response

Sudden abdominal pain, gastrointestinal bleeding or new neurological symptoms warrant clinical assessment for possible systemic complications. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)

1. Are acute abdominal symptoms, gastrointestinal bleeding or new neurological changes recorded, and has urgent clinical assessment occurred? action
2. What specialist-authored follow-up plan specifies monitoring, reassessment triggers and responsibility for reviewing results? provenance

Treatment evidence and response

Reported interventions include antiplatelet drugs and experimental approaches such as eculizumab and treprostinil; reports of benefit require evidence-qualified interpretation. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)

1. For the proposed intervention, what study design, patient population, disease extent and regulatory context support its consideration? provenance
2. Which skin, organ, functional and adverse-effect outcomes will the clinician assess, over what interval, before judging response? action

Check these first

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these check these first hold for the sense of Degos disease this model covers, and on what evidence? provenance

Kinds and varieties

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these kinds and varieties hold for the sense of Degos disease this model covers, and on what evidence? provenance

Real-world use

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these real-world use hold for the sense of Degos disease this model covers, and on what evidence? provenance

Failure modes and hazards

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these failure modes and hazards hold for the sense of Degos disease this model covers, and on what evidence? provenance

Neighbouring kinds and how to tell them apart

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these neighbouring kinds and how to tell them apart hold for the sense of Degos disease this model covers, and on what evidence? provenance