ICD-11 classes
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A43 — Overlap or undifferentiated nonorgan specific systemic...
4A43.0 — IgG4 related disease
EK91.2 — Primary cutaneous plasmacytosis
ICD-11 EK91.2 — Primary cutaneous plasmacytosis
A skin disorder resulting from focal or multifocal dense infiltration of the skin by plasma cell aggregates. It may be associated with high levels of serum IgG4. It typically presents as widespread reddish-brown papules, nodules and pigmented indurated plaques involving the trunk and limbs but may present as a single nodule or plaque. The majority of patients with this uncommon skin disorder are of East Asian descent. There is a risk of progression to systemic lymphoproliferative malignancy.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere:
- IgG4 related disease #15846
- Dermatoses which may presage cutaneous lymphoma #17414
- IgG4 related disease #18381