raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:137814

Macular amyloidosis

Type
Disease
Group
Disorder
Status
active

Macular amyloidosis (MA) is a rare chronic form of cutaneous amyloidosis, a skin disease characterized by the accumulation of amyloid deposits in the dermis, clinically characterized by pruritic hyperkeratotic gray-brown macules that give a rippled or reticulated pattern of pigmentation usually in the upper back and extensor sites of arms, forearms and legs, and histologically by the deposition of amyloid in the upper dermis and close to the basal cell layer of the epidermis. MA is commonly associated with other skin diseases, such as atopic dermatitis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enMacular amyloidosis
frAmylose maculeuse
deAmyloidose, makuläre
esAmiloidosis macular
itAmiloidosi maculare
nlMaculaire amyloïdose
ptAmiloidose macular
plAmyloidoza plamki
csMakulární amyloidóza
ukМакулярний амілоїдоз
zh斑状淀粉样变性
trMaküler amiloidoz

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10E85.4+NTBTinclusionvalidated
ICD-10L99.0*NTBTinclusionvalidated
ICD-115D00.0NTBTindexvalidated
MONDO0015303Evalidated
UMLSC0544839Evalidated
GARD19885Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card