Cutaneous Immunocytoma

Background

Cutaneous immunocytomas are low-grade malignant B-cell lymphomas that arise in the skin as solitary or multiple red to brownish red papules, plaques, or nodules. Secondary dissemination to other organs or the bone marrow may occur.

DISEASE ASSOCIATIONS CHARACTERIZATION

Borrelia borgdorferi infection
J Am Acad Dermatol 1991;24:584-90
J Cutan Pathol 1997;24:457-61

Either within skin lesions of acrodermatitis chronica atrophicans, or as multiple tumors concominant with acrodermatitis

LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION

Laboratory Markers

Immunoglobulins
In most of the described cases, immunoglobulin kappa-light chains have been detected

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION

General

VARIANTS

Cutaneous immunocytoma presenting with multiple infiltrated macules and papules
J Am Acad Dermatol 2001;44:324-9

Multiple, brown-red to bluish macules and papules on the left thigh

Proliferation was monoclonal as evaluated by positive staining of the plasma cells with antibodies against IgG heavy chain and lambda-light chain and with polymerase chain reaction showing immunoglobulin heavy-chain gene rearrangement

Neither paraproteinemia nor involvement of bone marrow was present.

HISTOLOGICAL TYPES CHARACTERIZATION

General Accumulation of lymphocytes and lymphoplasmocytoid cells in the mid dermis. Numerous lymphoplasmacytoid and plasma cells
Oval to spindled hyperchromatic fibroblasts and histiocytes, some multinucleated between irregular, thickened collagen bundles

Cutaneous immunocytomas: a clinicopathologic study of 26 cases.

Rijlaarsdam JU, van der Putte SC, Berti E, Kerl H, Rieger E, Toonstra J, Geerts ML, Meijer CJ, Willemze R.

Department of Dermatology, Free University, Amsterdam, The Netherlands.

Histopathology 1993 Aug;23(2):117-25 Abstract quote

The clinical and histological features of 16 patients with a primary cutaneous immunocytoma and 10 patients with a secondary cutaneous immunocytoma are reported. In all cases the diagnosis was based on the presence of monotypic plasma cells or lymphoplasmacytoid cells.

Our data show that primary cutaneous immunocytomas are a distinct type of cutaneous lymphoma, characterized by (a) the presence of solitary or localized skin lesions (13 of 16 cases); (b) preferential localization on arms and legs (15 of 16 cases); (c) excellent response to local treatment (15 of 16 cases) and (d) a favourable prognosis.

Histologically, these primary cutaneous immunocytomas are characterized by the presence of nodular or diffuse infiltrates with monotypic lymphoplasmacytoid/plasma cells located at the periphery of the infiltrates. Important clinical and histological differences were noted between primary and secondary immunocytomas. In the latter group more widespread skin disease was seen, often in the presence of paraproteins and/or autoimmune diseases. In contrast with the peripheral localization of the monotypic cells in primary cutaneous immunocytomas the monotypic lymphoplasmacytoid/plasma cells in secondary immunocytomas formed diffuse infiltrates or these cells were found dispersed throughout the infiltrate.

There were no differences in clinical presentation or course between the different subtypes of cutaneous immunocytomas (lymphoplasmacytic, lymphoplasmacytoid and polymorphic immunocytomas).

The differential diagnosis between primary cutaneous immunocytomas and cutaneous plasmacytomas, primary follicular centre cell lymphomas and cutaneous 'pseudolymphomas' is discussed.

VARIANTS

Primary cutaneous marginal zone B-cell lymphoma: a report of 9 cases.

de la Fouchardiere A, Balme B, Chouvet B, Sebban C, Perrot H, Claudy A, Bryon PA, Coiffier B, Berger F.

Service d'Anatomie-Pathologique, Hopital Edouard-Herriot, 69437 Lyon Cedex 3, France.

J Am Acad Dermatol 1999 Aug;41(2 Pt 1):181-8 Abstract quote

BACKGROUND: Primary cutaneous B-cell lymphoma is a heterogeneous group among which marginal zone B-cell lymphoma (MZL) appears to be the most common subtype.

OBJECTIVE: We analyze clinical presentation, histologic aspects, and outcome of patients with primary cutaneous MZL.

METHODS: All samples classified as primary cutaneous lymphoma over the past 10 years were reviewed, and cases of primary MZL were identified. RESULTS: Nine cases of MZL were analyzed, all from the upper body region, with a predominance in elderly women. Histologic aspects included a dense, nodular, deep-seated infiltrate containing various proportions of small cells displaying a centrocyte-like, plasmacytoid or monocytoid appearance. Surface expression of CD5, CD10, and CD23 was negative. Long survival was noted but relapses in the skin, nodes, orbit, salivary glands, and breast were observed.

CONCLUSION: MZL is the predominant primary cutaneous lymphoma of our study. It has distinctive histologic and clinical features as well as outcome.

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION

Immunoperoxidase Monoclonal light chain restriction

Am J Surgical Pathol 1994;18:969-78
Am J Surg Pathol 1997;21:1307-15

Commonly Used Terms

Skin Lymphomas

Last Updated 10/19/2001

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Accumulation of lymphocytes and lymphoplasmocytoid cells in the mid dermis. Numerous lymphoplasmacytoid and plasma cells Oval to spindled hyperchromatic fibroblasts and histiocytes, some multinucleated between irregular, thickened collagen bundles
Cutaneous immunocytomas: a clinicopathologic study of 26 cases. Rijlaarsdam JU, van der Putte SC, Berti E, Kerl H, Rieger E, Toonstra J, Geerts ML, Meijer CJ, Willemze R. Department of Dermatology, Free University, Amsterdam, The Netherlands.	Histopathology 1993 Aug;23(2):117-25 Abstract quote The clinical and histological features of 16 patients with a primary cutaneous immunocytoma and 10 patients with a secondary cutaneous immunocytoma are reported. In all cases the diagnosis was based on the presence of monotypic plasma cells or lymphoplasmacytoid cells. Our data show that primary cutaneous immunocytomas are a distinct type of cutaneous lymphoma, characterized by (a) the presence of solitary or localized skin lesions (13 of 16 cases); (b) preferential localization on arms and legs (15 of 16 cases); (c) excellent response to local treatment (15 of 16 cases) and (d) a favourable prognosis. Histologically, these primary cutaneous immunocytomas are characterized by the presence of nodular or diffuse infiltrates with monotypic lymphoplasmacytoid/plasma cells located at the periphery of the infiltrates. Important clinical and histological differences were noted between primary and secondary immunocytomas. In the latter group more widespread skin disease was seen, often in the presence of paraproteins and/or autoimmune diseases. In contrast with the peripheral localization of the monotypic cells in primary cutaneous immunocytomas the monotypic lymphoplasmacytoid/plasma cells in secondary immunocytomas formed diffuse infiltrates or these cells were found dispersed throughout the infiltrate. There were no differences in clinical presentation or course between the different subtypes of cutaneous immunocytomas (lymphoplasmacytic, lymphoplasmacytoid and polymorphic immunocytomas). The differential diagnosis between primary cutaneous immunocytomas and cutaneous plasmacytomas, primary follicular centre cell lymphomas and cutaneous 'pseudolymphomas' is discussed.
VARIANTS
Primary cutaneous marginal zone B-cell lymphoma: a report of 9 cases. de la Fouchardiere A, Balme B, Chouvet B, Sebban C, Perrot H, Claudy A, Bryon PA, Coiffier B, Berger F. Service d'Anatomie-Pathologique, Hopital Edouard-Herriot, 69437 Lyon Cedex 3, France.	J Am Acad Dermatol 1999 Aug;41(2 Pt 1):181-8 Abstract quote BACKGROUND: Primary cutaneous B-cell lymphoma is a heterogeneous group among which marginal zone B-cell lymphoma (MZL) appears to be the most common subtype. OBJECTIVE: We analyze clinical presentation, histologic aspects, and outcome of patients with primary cutaneous MZL. METHODS: All samples classified as primary cutaneous lymphoma over the past 10 years were reviewed, and cases of primary MZL were identified. RESULTS: Nine cases of MZL were analyzed, all from the upper body region, with a predominance in elderly women. Histologic aspects included a dense, nodular, deep-seated infiltrate containing various proportions of small cells displaying a centrocyte-like, plasmacytoid or monocytoid appearance. Surface expression of CD5, CD10, and CD23 was negative. Long survival was noted but relapses in the skin, nodes, orbit, salivary glands, and breast were observed. CONCLUSION: MZL is the predominant primary cutaneous lymphoma of our study. It has distinctive histologic and clinical features as well as outcome.

LABORATORY/RADIOLOGIC/OTHER TESTS	CHARACTERIZATION
Laboratory Markers
Immunoglobulins	In most of the described cases, immunoglobulin kappa-light chains have been detected

GROSS APPEARANCE/CLINICAL VARIANTS	CHARACTERIZATION
General
VARIANTS
Cutaneous immunocytoma presenting with multiple infiltrated macules and papules	J Am Acad Dermatol 2001;44:324-9 Multiple, brown-red to bluish macules and papules on the left thigh Proliferation was monoclonal as evaluated by positive staining of the plasma cells with antibodies against IgG heavy chain and lambda-light chain and with polymerase chain reaction showing immunoglobulin heavy-chain gene rearrangement Neither paraproteinemia nor involvement of bone marrow was present.

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER	CHARACTERIZATION
Immunoperoxidase	Monoclonal light chain restriction