Lymphomatoid Papulosis Development in Acute Lymphoblastic Leukemia

Abstract

Lymphomatoid papulosis (LyP) is a chronic, recurrent benign skin disease characterized by histological features of a CD 30-positive cutaneous T-cell lymphoproliferative disorder. It is rare, with an annual, worldwide incidence of 1.2 - 1.9 per million, and accounts for 16-47% of pediatric cutaneous lymphoproliferative disorders. It often occurs on the extremities or the trunk and rarely affects the face or genitals. Its onset may be triggered by irradiation therapy, immunomodulating agents, infection or atopic dermatitis. It has a benign course but is associated with certain hematological malignancies. Mycosis fungoides and primary cutaneous anaplastic large cell lymphoma are the most commonly associated hematological malignancies. The incidence of lymphoma in children with LyP has been reported to be 8.5% at most. Most patients who develop lymphomas do so within 4 years of the LyP onset; therefore, patients with LyP should be carefully followed up. Herein, we report a case in which tumors appeared in the left scrotum and under the left lip during maintenance therapy for precursor B-cell acute lymphoblastic leukemia. We needed to distinguish the tumor from extramedullary recurrence of ALL or de novo other cutaneous lymphoma; however, the histological findings of a tumor biopsy resulted in a diagnosis of LyP.

Keywords: Acute lymphoblastic leukemia; Child; Lymphomatoid papulosis.

Figure 1

Macroscopic appearance of the tumors on the left scrotum (a, arrow) and under the lip (b, arrow).

Figure 2

Histology of the scrotal skin mass showing cutaneous CD30-positive T-cell lymphoproliferative disorder (lymphomatoid papulosis). The lower (a) and higher (b, c) magnification views of hematoxylin and eosin stained histological specimens of the scrotal skin mass. There is a necrotic layer in the dermis, and dense cell infiltration throughout the dermis. The infiltrating cells are mainly small to large mononuclear cells. Large cells are positive for CD30 on immunostaining (d).