Lymphomatoid papulosis with 6p25.3 rearrangement: a further case of the newly described variant.

Abstract

Funding sources: none. Conflicts of interest: none declared. Dear Editor, A 67‐year‐old woman presented with a 6‐month history of recurrent erythematous papules on her face and scalp, persisting for approximately 6 weeks before resolving spontaneously without scarring. Examination revealed two nonspecific excoriated erythematous papules on the vertex of her scalp (Fig. 1a,b) and a subtle, poorly defined, nonscaly erythematous patch on the anterolateral aspect of her neck (Fig. 1c). She was systemically well with no B symptoms, lymphadenopathy or hepatosplenomegaly. There was no personal history of skin disease, malignancy or immunosuppression and no family history of note. Her full blood count, lymphocyte subsets, lactate dehydrogenase, and HIV and human T‐lymphotropic virus type I serology were all normal or negative. A skin biopsy of a facial papule demonstrated a nodular dermal lymphoid infiltrate. The epidermis was nonulcerated, hyperplastic and peppered with atypical small‐ to medium‐sized mononuclear cells with irregular nuclei in a pagetoid reticulosis pattern. Occasional intraepidermal nests of lymphocytes, similar to the Pautrier microabscesses of mycosis fungoides, were also noted (Fig. 2a). The atypical cells in the dermis were medium to large in size with hyperchromatic, irregular nuclei and abundant finely granular cytoplasm (Fig. 2b). The atypical cells had a CD3+, CD4−, CD8−, CD2−, CD5+ immunophenotype and showed diffuse strong expression of CD30 (Fig. 2c). ALK‐1, epithelial membrane antigen (EMA), T‐cell intracytoplasmic antigen 1 (TIA‐1), CD56, granzyme B and Epstein–Barr virus‐encoded RNA were negative and Ki‐67 was > 95%. T‐cell receptor gene analysis confirmed nonidentical clonal rearrangements in the skin and blood. Fluorescence in situ hybridization (FISH) analysis of the DUSP22‐IRF4 locus demonstrated separation of centromeric (green) and telomeric (red) fluorescent signals confirming a translocation involving 6p25.3 (Fig. 2d), and a diagnosis of the primary cutaneous CD30‐positive lymphoproliferative disorder, lymphomatoid papulosis (LyP) with 6p25.3 rearrangement, was made.