Fatal subcutaneous panniculitis‐like T‐cell lymphoma with interface change and dermal mucin, a dead ringer for lupus erythematosus

Abstract

We report a 48-year-old man who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3(+) CD4(-) CD8(+/-) CD56(+). Monoclonal rearrangements of the T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is one amongst the rare reports of subcutaneous panniculitis-like T-cell lymphoma with systemic involvement.

Primary Cutaneous B-Cell Lymphoma, Leg Type Restricted to the Subcutaneous Fat Arising in a Patient With Dermatomyositis

“…However, these have universally been described as T-cell processes not limited to the subcutis. [22][23][24][25] Our case is unique in its B-cell phenotype and in its restriction to the subcutaneous fat.…”

Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

“…The HLH triggered by HHV-8 is extremely rare but has been reported in associated lymphoproliferative disorders as well as in immunocompromised patients. Conditions rarely reported in association with HLH include acute T-cell or NK leukemia [64,65].…”

Lupus erythematosus

“…In weiterer Krankheitsfolge traten, wie histologisch verfolgt werden konnte, atypische Lymphozyten auf, die schließlich im Rearrangement eine Monoklonalität aufwiesen, sodass die Knoten schließlich als malignes Lymphom identifiziert wurden. Weitere differenzialdiagnostisch schwierige Fälle wurden in der Literatur beschrieben [76,77,78]. Bei 9 Fällen eines Lupus profundus konnten histologisch epidermale Veränderungen in 73%, in 45% Muzinablagerungen und Infiltrate mit 91% Plasmazellen und Lymphfollikeln in der Dermis nachgewiesen werden.…”