Home / Clinical cases / Primary cutaneous plasmacytoma

Primary cutaneous plasmacytoma

 

Case synopsis

Case synopsis

A 44 year old male, presented by asymptomatic erythematous papules on his forhead 6 months duration.

patient had a history of similar lesions and it was excised leaving scar ( white arrow). A biosy was taken ( black arrow)

 

Diagnosis

Diagnosis

Pathology result was consistent with ” Primary cutaneous plasmacytoma”

Primary cutaneous plasmacytoma is a rare B-cell lymphoma consisting of a monoclonal proliferation of mature plasma cells in the skin without underlying multiple myeloma.

* Localized forms of malignant proliferations of plasma cells include;

1- Solitary plasmacytoma of bone

2- Extramedullary plasmacytoma

 

* The term plasmacytoma is no longer used in the WHO-EORTC classification.

** PCP  Originally classified as a discrete entity,currently is considered to be a variant of primary cutaneous marginal zone B-cell lymphoma.

* Cases of localization to the skin are thought
to represent 2% to 4% of all primary extramedullary  plasmacytomas.

* Very few cases of PCPs ( 30) have been
reported in the literature

* Secondary cutaneous plasmacytoma occurs in the setting of multiple myeloma or extramedullary plasmacytoma.

* PCPs occur in age range of 22 to 88 years

* lesions typically present as slow-growing, reddish brown macules or plaques on the face, trunk, or extremities.

Multiple myeloma developed in   one-third of patients & the features include;

1- Bone marrow plasmacytosis of more than 10%

2- Osteolytic lesions

3- Detection of a plasma or urine paraprotein.

( all were normal in our patient)

Ddx

1- Other primary B cell lymphoma

2- Benign infiltrates of plasma cells in the skin (eg, plasma cell granuloma)

3- Infectious diseases with prominent plasma cell infiltration (eg, syphilis, borrelia infection)

Treatment

Treatment

*The prognosis for primary cutaneous plasmacytoma is greater than 90% survival at 5 years.

* Patients with solitary lesions have a better prognosis than those with multiple lesions.

* Possible treatment options include;

1- Intralesional steroids

2- PUVA

3- Topical immunomodulators

4- local radiotherapy

5- Excision.

Leave a Reply

Your email address will not be published. Required fields are marked *