Lymphomatoid papulosis misdiagnosed as pityriasis lichenoides et varioliformis acuta: Two case reports and a literature review

Abstract

The aim of this study was to improve the level of diagnosis and differential diagnosis of lymphomatoid papulosis (LyP). Two cases of type B LyP were identified and the literature was reviewed to summarize the clinical outcomes and pathology of LyP and its treatment. The two patients exhibited symptoms with papulonodular lesions, the centers of which gradually underwent ulceration and necrosis. CD30, a helper T-cell marker specifically expressed in tumor cells was analyzed by immunohistochemical staining and the result showed that CD30-negative or only scattered CD30-positive cells were present. Therefore, a diagnosis of type B LyP was made. A fairly good curative effect was achieved following treatment with retinoic acid, glucocorticoids and immunomodulatory drugs. LyP is a type of low-level malignant lymphoma and is easily misdiagnosed as pityriasis lichenoides et varioliformis acuta and other diseases. In order to avoid under diagnosis and misdiagnosis, doctors should evaluate suspected patients by histopathological and immunohistochemical examination.

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

“…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.…”

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

“…(MF-like) cases. 12 Additionally, reviewing previous published descriptions of CD8 + LyP, a few photomicrographs appear to suggest that the rare CD30-negative cases may have morphologic overlap with mycosis fungoides or type B LyP. 6,[13][14][15][16] To complicate the matter further, there are a few cases of CD8 + mycosis fungoides with large cell transformation and much rarer cases of primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma with CD30 expression, 10,14 and these are the two most significant histopathologic differential diagnoses for CD8 + LyP.…”

“…The histopathologic spectrum of CD8 + LyP is variable and may resemble the different CD4 + subtypes, including A, B, C, E, and “F” subtypes . Although CD30 positivity has been considered a prerequisite for any diagnosis of LyP, there are several reports of CD30‐weak or CD30‐negative cases, especially of the type B (MF‐like) cases . Additionally, reviewing previous published descriptions of CD8 + LyP, a few photomicrographs appear to suggest that the rare CD30‐negative cases may have morphologic overlap with mycosis fungoides or type B LyP .…”

Uspješno liječenje pityriasis lichenoides et varioliformis acuta u pacijenta s kroničnom hiperuricemijom

“…Sličnost i moguća povezanost s malignim oboljenjima limfocita T zahtijeva dugotrajno praćenje pacijenata s ovim kožnim promjenama. Postavljanje dijagnoze PLEVA-e temelji se na razlučivanju PLEVA-e od sljedećih stanja i poremećaja: limfomatoidna papuloza, varicella, Gianotti-Crostijev sindrom, erythema multiforme, pityriasis rosea, psoriasis guttata, leukocitoklastični vaskulitis, ubod kukca i sekundarni sifilis 16,19,26,27 . Dijagnozu PLEVA-e postavljenu samo na temelju kliničkog pregleda trebalo bi u slučaju perzistiranja kožnih promjena potvrditi patohistološkim nalazom.…”