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Special Article |

Cutaneous T-Cell Lymphoid Dyscrasia A Unifying Term for Idiopathic Chronic Dermatoses With Persistent T-Cell Clones

Joan Guitart, MD; Cynthia Magro, MD
Arch Dermatol. 2007;143(7):921-932. doi:10.1001/archderm.143.7.921.
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It often takes several years before the diagnosis of mycosis fungoides is clearly established. During this period preceding the diagnosis of cutaneous T-cell lymphoma (CTCL), some patients experience a variety of distinct skin conditions of ambiguous origin. With refinement in the methods of T-cell clonal detection in skin biopsy specimens, we can now establish that many of these preceding entities are characterized by a T-cell clone. Therefore, we studied the association of these clonal conditions with CTCL. We identified 8 distinct clinicopathological conditions characterized by the frequent detection of T-cell clonality, a chronic course often recalcitrant to topical therapy, lack of a known triggering event, and lack of morphologic evidence of a T-cell lymphoma with potential for progression to CTCL. We propose the term cutaneous T-cell lymphoid dyscrasias to unify these entities. Defining the preceding stages of CTCL is important to properly categorize these conditions and to help us understand and redefine the evolution of idiopathic clonal dermatoses and their potential for lymphoma progression.

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Figure 1.

Hypopigmented interface variant of cutaneous T-cell lymphoid dyscrasias in a 12-year-old. A, Asymmetrical hypopigmented patches resistant to topical corticosteroids. B, Lymphoid infiltrate composed of small to intermediate cells along the basal cell layer. The immunophenotype was overwhelmingly dominated by CD8 expression (hematoxylin-eosin, original magnification ×10).

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Figure 2.

Pigmented purpuric dermatosis. A, Long-standing lesion of pigmented purpuric dermatosis involving the lower leg. B, Skin biopsy specimen showing a superficial interstitial and perivascular lymphocytic infiltrate with erythrocyte extravasation and hemosiderin deposits (hematoxylin-eosin, original magnification ×20). C, Golden brown poikilodermatous patches involving the abdomen that developed a few years later in the same patient. D, Biopsy specimen obtained at the time of C showing an atypical lymphoid infiltrate along the dermoepidermal junction with characteristic features of the poikilodermatous presentation of mycosis fungoides (hematoxylin-eosin, original magnification ×4).

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Figure 3.

Atypical lymphocytic lobular panniculitis. A, Numerous confluent deep and tender nodules involving the thighs. There is no evidence of ulceration or drainage. Suture line corresponds to a recent biopsy site. B, Atypical lymphocytic lobular panniculitis without significant destructive alterations of the fat (hematoxylin-eosin, original magnification ×40). C, Higher-power magnification of B showing the composition of the mononuclear cell infiltrate as one of histiocytes and small to intermediate lymphocytes and some erythrocyte extravasation. The characteristic internal rimming of the adipocytes seen in panniculitislike T-cell lymphoma is not present (hematoxylin-eosin, original magnification ×100). D, Capillaroscopy (GeneScan assay; Applied Biosystems, Foster City, California) of lesion biopsied in 2004 shows a polyclonal background profile with a dominant clone (187 and 257 base pairs [bp]). One year later, the new profile showed a single dominant monoclonal peak. PCR indicates polymerase chain reaction; TCRβ, T-cell receptor β.

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Figure 4.

Syringolymphoid hyperplasia with alopecia. A, Patient with a single patch of alopecia on the abdomen. Some xerosis and follicular-based keratotic papules were noted in the forearm. B, Initial biopsy specimen showing a lymphoid infiltrate centered around the eccrine coil. There was no significant atypia or epidermotropism (hematoxylin-eosin, original magnification ×10). C, Punctate erythematous papules involving the soles and torso that developed 4 years later. D, Biopsy specimen at the time of C showing hyperplastic changes of the glandular epithelium with an atypical infiltrate of CD4 hyperconvoluted lymphocytes. The diagnosis of syringotropic mycosis fungoides was made (hematoxylin-eosin, original magnification ×40).

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Figure 5.

Idiopathic follicular mucinosis. A, Single indurated plaque on the left cheek with prominent follicular-based papules. The lesions were resistant to topical treatments including corticosteroid creams. B, Biopsy specimen at low magnification showing mucinous degeneration of the pilosebaceous unit associated with a prominent lymphoid infiltrate (hematoxylin-eosin, original magnification ×4). C, Specimen showing no significant lymphoid atypia, but a T-cell clonality was identified on polymerase chain reaction analysis (hematoxylin-eosin, original magnification ×40).

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Figure 6.

Pityriasis lichenoides. A, Classic changes of pityriasis lichenoides as defined by epithelial hyperplasia and striking migration of lymphocytes to involve all layers of the epidermis. Extensive red blood cell extravasation is seen amid a superficial interstitial and perivascular lymphocytic infiltrate (hematoxylin-eosin, original magnification ×20). B, Molecular studies of samples from a 56-year-old woman with long-standing pityriasis lichenoides chronica obtained at different times showing an oligoclonal process with similar molecular profiles. bp indicates base pairs; PCR, polymerase chain reaction; and TCRβ, T-cell receptor β.

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