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. 2017 May;30(5):761-772.
doi: 10.1038/modpathol.2016.240. Epub 2017 Jan 27.

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas

Joan Guitart  1 , M Estela Martinez-Escala  1 , Antonio Subtil  2 , Madeleine Duvic  3 , Melissa P Pulitzer  4 , Elise A Olsen  5 , Ellen Kim  6 , Alain H Rook  6 , Sara S Samimi  6 , Gary S Wood  7 , Michael Girardi  2 , Jacqueline Junkins-Hopkins  8 , Doina S Ivan  3 , M Angelica Selim  5 , Kimberly A Sable  1 , Pooja Virmani  5 , Laura B Pincus  9 , Michael T Tetzlaff  3 , Jinah Kim  10 , Youn H Kim  10
Affiliations

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas

Joan Guitart et al. Mod Pathol. 2017 May.

Abstract

Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αβ T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αβ/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.

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Figures

Figure 1
Figure 1. Clinical presentation of PCAECyTCL in three different patients
a) Tumor presentation with exophytic hemorrhagic and necrotic nodules resembling pyogenic granuloma (Case 24). b) Extensive targetoid necrotic macules and plaques resembling severe pityriasis lichenoides acuta or erythema multiforme (Case 27). c) Ulcerated plaques involving genitalia (Case 23).
Figure 2
Figure 2. Clinical presentation of PCAECyTCL in two different patients
a) Oral and nasal ulcerated macules (Case 25). b) Extensive annular erosive plaques some with hemorrhagic features (Case 25). c) Annular and arcuate ulcerated plaques and scars at sites of resolved lesions (Case 25). d) Annular erythematous plaques at presentation (Case 26). e) Same patient as 2D now with extensive erosive confluent plaques a few months later (Case 26).
Figure 3
Figure 3. Histopathological features of PCAECyTCL
a) H&E stains showing extensive full-thickness pagetoid exocytosis of medium sized lymphocytes without significant keratinocytes necrosis or dermal involvement (Case 25). b) Atrophic epidermis with pagetoid intraepidermal atypical small/medium lymphocytes mostly involving the lower levels of the epidermis. Occasional necrotic keratinocytes are noted (Case 34). c) Tumor lesion with a predominantly dermal infiltrate (Case 29). d) Same as C in higher magnification showing extensive pagetoid exocytosis in addition to a dense dermal infiltrate (Case 29). e) Marked lymphoid infiltration of eccrine coils on the left side and pilosebaceous unit on the right side (Case 26). f) High power of image e demonstrating atypical small lymphocytes in the eccrine coil (Case 26).
Figure 4
Figure 4. Histopathological features of PCAECyTCL
Immunohistochemistries of case 30 illustrate in 3a with strong CD8 expression (a) and TIA-1 (b). Immunochemistries of case 27 illustrated in figure 1b including CD8 (c), granzyme B (d), and coexpression of γδ marker (e) and βF1 (f).
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