Clinical features

•Very poor prognosis

•Exceptionally rare & is only seen in stage IV disease (I have only seen three cases in 52 years!)

•Bimodal age distribution for Hodgkin lymphoma- teenagers & over 50’s

•Nodular sclerosis>mixed cellularity>lymphocyte depleted

•Nodal disease extending to the skin, retrograde lymphatic spread andmetastatic disease

•Single or multiple pink to red papules, nodules & plaques

•Chest, head & neck, inguinal region, axilla but can present anywhere

Histological features

•Hodgkin lymphoma- association with EBV, HIV & infectious mononucleosis

•Diffuse or nodular infiltrate, sometimes with ulceration 

•Hodgkin & Reed-Sternberg cells- large cells with bilobed or multilobed nuclei containing a prominent nucleolus (Owl’s eye cells)

•Background population of lymphocytes, plasma cells, histiocytes, neutrophils & eosinophils

•Variable necrosis

•Variable fibrosis

•CD30, CD15, pax 5, MUM1 +ve

•Variable weak CD20 +ve

•CD3-ve

•EMA & ALK1 –veVariable LMP1

Differential diagnosis

The main differential diagnsois with with lymphomatoid papulosis and cutaneous anaplastic large cell lymphoma. Other conditions including EBV+ mucocutaneous ulcer, diffsue large B cell lymphoma, post transplant & other immunosuppression-associated lymphoproliferative disorder. Given that secondary skin involvement only occurs in stage IV Hodgkin lymphoma, the correct diagnosis should not be in doubt.

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