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Published on: December 15, 2011
Granulomatous eccrinotropic lymphomatoid papulosis
A Neil Crowson1, Dmitry Y Baschinsky, Al Kovatich
1Central Medical Laboratories, Winnipeg, Canada.
Insights
This study identifies a new type of lymphomatoid papulosis with distinctive eccrine coil involvement. This condition, characterized by granulomatous inflammation, does not appear to progress to lymphoma in patients.
Area of Science:
- Dermatopathology
- Immunohistochemistry
- Molecular Biology
Background:
- Lymphomatoid papulosis is a rare condition with diverse clinical and histological presentations.
- A novel variant characterized by eccrine coil involvement and granulomatous inflammation is described.
Purpose of the Study:
- To characterize a novel variant of lymphomatoid papulosis.
- To differentiate this variant from other cutaneous conditions that may cause diagnostic confusion.
Main Methods:
- Histopathological examination including light microscopy and immunohistochemistry (CD2, CD3, CD4, CD5, CD7, CD8, CD30).
- T-cell receptor gamma chain gene rearrangement analysis using polymerase chain reaction-single-stranded conformational polymorphism.
- Clinical follow-up of patients.
Main Results:
- Two cytomorphologic patterns were identified: large cell dominant and small cell lymphocyte-rich.
- Clonal T-cell receptor gamma chain gene rearrangement was detected in 4 out of 5 lymphocyte-dominant cases.
- No lymphomas developed during an average 6-year follow-up period.
Conclusions:
- This variant of lymphomatoid papulosis is defined by eccrine coil and cutaneous nerve involvement, granulomatous inflammation, and a predominantly lymphocytic infiltrate.
- Clinical features such as pruritus, younger age at diagnosis, and an indolent course are suggestive.
- Distinguishing this entity from discoid lupus erythematosus, Jessner lymphocytic infiltrate, subcutaneous T-cell lymphoma, and arthropod bite reactions is crucial.
Abstract:
We describe 9 patients with a novel variant of lymphomatoid papulosis characterized by prominent localization of the infiltrate around the eccrine coil, resulting in nodular expansion of the coil accompanied by variable granulomatous inflammation. Light microscopy, immunohistochemical analysis using antibodies to CD2, CD3, CD4, CD5, CD7, CD8, and CD30 in 6 cases, and polymerase chain reaction--single-stranded conformational polymorphism analysis for T-cell receptor gamma chain gene rearrangement in 5 cases revealed 2 cytomorphologic patterns (large cell dominant with polymorphous inflammation, small cell lymphocyte--rich with an inconspicuous large cell component [phenotypes, CD30+/CD3+/CD4+/CD7-for large atypical cells; reactive for small lymphocytes]) and clonal restriction in 4 and polyclonality in 1 of the lymphocyte-dominant cases. During an average 6-year follow-up, no lymphomas developed. Recognition of this variant is important--accentuation of the infiltrate around the eccrine coil and cutaneous nerves, presence of granulomatous inflammation, dominance of small lymphocytes in the dermis, and variable extension into the panniculus may lead to diagnostic confusion with entities such as discoid lupus erythematosus, Jessner lymphocytic infiltrate of skin, subcutaneous T-cell lymphoma, and persistent arthropod bite reaction. Our findings suggest that pruritus, a younger age at diagnosis, and a more indolent course are defining clinical features.