CD8‐positive lymphomatoid papulosis (type D): Some lesions may lack CD30 expression and overlap histologically with mycosis fungoides

Abstract

Background CD8+ lymphomatoid papulosis is frequently indistinguishable histopathologically from primary cutaneous aggressive epidermotropic CD8+ T‐cell lymphoma except for the expression of CD30. However, absent or weak expression of CD30 has been rarely reported in cases of CD8+ LyP. Objective We aim to study the clinical and pathologic features of cases of CD8+ LyP with no or minimal expression of CD30. Material and Methods We identified all cases of CD8+ LyP diagnosed in our institution over a period of 10 years. Blinded comparison of clinical and histopathologic features of cases with and without CD30 expression was performed. Results Among seven cases (four patients) with definitive clinical and histopathologic diagnosis of CD8+ LyP, two cases (29%) had no expression of CD30. These two cases had more prominent epidermotropism, less epidermal ulceration, and less vascular damage relative to cases with CD30 expression and therefore resembled mycosis fungoides and type B LyP. CD5 and CD7 were frequently lost regardless of the CD30 status. Expression of cytotoxic markers was not different between the two groups. In the two cases with lack of CD30 expression, subsequent biopsies showed classic features of CD8+ LyP with strong expression of CD30. Conclusion CD8+ LyP with lack of expression of CD30 may have distinct histopathologic features that resemble mycosis fungoides and LyP type B. Clinically, they are indistinguishable from their CD30+ counterparts, signifying the importance of clinical correlation to avoid the erroneous diagnosis of lymphoma. Interval biopsies may be needed to establish a definitive diagnosis.

Two Histologic Patterns of Lymphomatoid Papulosis Occurring in a Child: A Matter of Timing?

“…The CD30 negativity in these cases has been reported to be transient, with subsequent biopsies demonstrating CD30 positivity. 13,14 This is similarly demonstrated in our case, with an earlier lesion showing CD30 negativity and an older lesion showing CD30 positivity. Interestingly, such protean histopathologic findings have also been described in biopsies taken from different sites concurrently have been described in MF before.…”

“…However, type B subtype, and less commonly, type D subtype, have been reported to be CD30 − . The CD30 negativity in these cases has been reported to be transient, with subsequent biopsies demonstrating CD30 positivity 13,14. This is similarly demonstrated in our case, with an earlier lesion showing CD30 negativity and an older lesion showing CD30 positivity.…”

Lymphomatoid papulosis types D and E: a multicentre series of the French Cutaneous Lymphomas Study Group

“…Since the first description of LyP D in 2010, < 50 cases have been described, in patients with an age range of 5–91 years 9,12–19 . In our series, the clinical presentation was similar to classic LyP types.…”

“…11 Since the first description of LyP D in 2010, < 50 cases have been described, in patients with an age range of 5-91 years. 9,[12][13][14][15][16][17][18][19] In our series, the clinical presentation was similar to classic LyP types. The lesions seem to exist on a spectrum ranging from small erythematous scaly PLC-like papules to lesions developing central erosion, and finally to large papulonodules with central necrosis.…”

How I treat primary cutaneous CD30+ lymphoproliferative disorders

“…Type D LyP is a CD8 1 variant that closely mimics CD8 1 MF or primary cutaneous aggressive epidermotropic CD8 1 cytotoxic T-cell lymphoma, 10 particularly when there is low CD30 expression. 11 In addition to the CD8 1 variant, LyP can exhibit a wide variety of immunoprofiles, including expression of other cytotoxic markers like TIA-1, granzyme B, and perforin, 12 as well as CD56 13 and T cell receptor g, 14 all without any difference in clinical behavior. A definitive histologic diagnose of LyP is not possible without (at a minimum) good clinical history, and all pathology reports of LyP (and CD30 1 LPDs, for that matter) should be subject to a healthy dose of skepticism until the patient is evaluated in a specialized center with expertise in diagnosing and treating CTCL.…”