3.3.14 Pseudolymphomas (not Borrelia induced)
ICD-11
4A43.0
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EH6Y
Synonyms
Lymphocytic infiltration, lymphoid hyperproliferation.
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Lymphocytic infiltration, reactive lymphoid hyperplasia.
Epidemiology
Clear epidemiological data for borrelia lymphocytoma or other subtypes is lacking.
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Epidemiological data for Borrelia lymphocytoma are known for about 2% (see chapter 2.2.9) for other subtypes is lacking.
Definition
Reactive pseudolymphomatous infiltrate of B-, T-, and combined T/B-cell type with or without follicular structures in the dermis and subcuti, mimicking malignant lymphomas.
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Reactive pseudolymphomatous infiltrate of B-, T-, and combined T/B-cell type that mimic clinically and/or histopathologically malignant lymphomas.
Aetiology & Pathogenesis
In pseudolymphoma B - and T- lymphocytes and other inflammatory cells accumulate in the dermis and subcutis as a reaction to stimuli of different origin. Often a causative agent is missing. Cutaneous pseudolymphoma with known etiology include reactions to tattoo dyes, arthropods, systemic medications, infections or vaccinations. A specific subset of B- cell type pseudolymphoma, borrelial lymphocytoma, primarily in Europe in areas endemic for the tick Ixodes ricinus with infection by Borrelia burgdorferi subsp afzelii and garinii.
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In pseudolymphoma B - and T-lymphocytes and other inflammatory cells accumulate in the dermis and subcutis as a reaction to stimuli of different origin. Often a causative agent is missing. Cutaneous pseudolymphoma with known etiology include reactions to tattoo dyes, arthropods, medications, allergens, viral or bacterial infections (molluscum contagiosum, herpes, HIV, syphilis…) or vaccinations. A subset of pseudolymphoma is the result of an unusual systemic mixed T- and B- cell response to several drug classes.
UV light can produce a T-cell dominated actinic reticuloid and persistent contact allergens (nickel or para-phenylenediamine).
A specific subset of B- cell type pseudolymphoma, Borrelia lymphocytoma (see 2.2.9), primarily occurs in Europe in areas endemic for the tick Ixodes ricinus. Borrelial lymphocytoma is a tick bite response to infection by Borrelia burgdorferi subsp afzelius and garinii. Causative agents: Borrelia garinii and Borrelia afzelii (both in Europe), not Borrelia burgdorferi sensu strictu.
Signs & Symptoms
Different forms: localized erythematous macules sometimes confluent, nodule, plaque, disseminated; occasionally with lymphadenopathy.
In borrelia lymphocytoma lesions indolent soft blue-red nodule up to 5 cm. Post scabies lymphocytomas localized or often disseminated. T-cell pseudolymphomas occur with localized plaques, nodules and disseminated papules or annular pattern.
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Different forms: localized erythematous macules sometimes confluent, solitary or multiple nodules, plaques. Occasionally disseminated; rarely with lymphadenopathy.
In Borrelia lymphocytoma lesions are often indolent soft blue-red nodules up to 5 cm. Post scabies lymphocytomas are localized or often disseminated. T-cell pseudolymphomas occur with localized plaques, nodules and disseminated papules or annular pattern. An erythroderma may also be seen. Some lesions may be also severely itchy. Red-blue lupoid infiltrate (diascopy).
Localisation
Sites of predilection: in borrelia lymphocytoma loose skin (ear, nipple, scrotum); T- or B-/T- pseudolymphomas may be localized (facial), single or multiple all over the body incl. erythrodermas.
Classification
No international classification as compared to malignant cutaneous lymphomas.
B -, T- and B-/T_ cell dominated subtypes.
Laboratory & other workups
Borrelia IgG and IgM titers raised in Borrelia lymphocytoma. In lymphocytic infiltration immunserology to exclude lupus erythematosus and blood count for leukemic infiltrate.
No specific other test.
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Borrelia IgG and IgM titers raised in Borrelia lymphocytoma. In actinic reticuloid UV light provocation test. In lymphocytic infiltration immunoserology to exclude lupus erythematosus and blood count for leukemic infiltrate. Repeated biopsies with B or T-cell genetic rearrangement studies can help (but many benign conditions present monoclonal populations of lymphocytes, careful clinical-pathologic and molecular correlation is required).
No specific other tests.
Dermatopathology
Most important to differentiate the subtypes of pseudolymphomas and to exclude primary or secondary malignant B-or T-cell lymphomas, eosinophils and plasma cells in a polymorphous pattern suggest pseudolymphoma.
Course
Depends on subtype. In borrelia lymphocytoma after adequate 2nd stage oral doxycycline over 3 weeks slowly fading of lesion(s).
Cessation of drugs lead after weeks or months depending on persistent metabolites to resolution.
Complications
None.
Diagnosis
Clinical features, serology, histology, PCR.
Differential Diagnosis
Malignant primary and secondary B- / T- cell lymphomas, disseminated cutaneous sarcoidosis, stage II syphilis.
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Each primary cutaneous lymphoma can have a mimicker. Clinical appearance will be similar to the mimicked lymphoma.
Malignant B- / T- cell lymphomas
Disseminated cutaneous sarcoidosis
Stage II syphilis
Prevention & Therapy
Depending on subtype.
Antibiotics (doxycycline 100 mg b.i.d. for 21 days) in borrelia lymphocytoma.
Cessation of responsible drugs. Topical mid and high potency corticosteroids. Occasionally intralesional steroids.
Special
None.
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