Lymphomatoid papulosis

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Lymphomatoid papulosis

ORPHA:98842Disease

Also called LyP

What it is

Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL) with which it shares overlapping clinical and histopathologic features.

Key facts

Age of onset
All ages
Classified as
Disease

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)Primary cutaneous CD30+ T-cell lymphoproliferative disease

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Genes

NPM1Part of a fusion gene
TYK2Part of a fusion gene

ICD-10 codes

C86.6filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

GARD 6944MEDDRA 10056670MESH D017731MONDO 0020326UMLS C0206182

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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