Pagetoid reticulosis in a 13‐year old female. A unique immunohistochemical profile

Abstract

Pagetoid reticulosis (PR) is a rare lymphoproliferative disorder with indolent behavior considered a variant of mycosis fungoides. It is characterized by marked epidermotropism of the neoplastic lymphocytes. Since its original description, five cases have been reported in children. We report a new case of PR with an immunohistochemical profile not previously described in children. K E Y W O R D S epidermotropic lymphoproliferative disorder, immunophenotype, mycosis fungoides, pagetoid reticulosis

Solitary patch on the thigh of a young boy

“…Clinically, PR presents as an indolent, solitary scaly lesion on an extremity with no evidence of systemic involvement and is often misdiagnosed as psoriasis, an eczematous process, or a bacterial or fungal infection. 3,5 In most of the reported pediatric cases, the lesion is not present at birth, instead appearing in childhood and expanding slowly until diagnosis. While we could not corroborate that our patient's initial lesions was indeed congenital, the recollection of the mark as present since birth reflects that it was likely longstanding and emphasizes PR's indolent nature.…”

“…) is an aggressive lymphoma with a poor prognosis, which classically presents as an ulcerated lesion with a rapid clinical course. 2,5 PR must also be distinguished from classic MF. Clinically, MF tends to be multifocal, involves the trunk, buttocks and proximal extremities, contrasted with PR, which is focal and typically on an extremity.…”

Primary cutaneous T‐cell lymphomas in childhood and adolescence

“…Pagetoid reticulosis presents clinically as a solitary, erythematous slowly progressive plaque on the distal extremities (usually hands and feet). Histologically, a hyperplastic epidermis with epidermotropism and small to medium sized nests of pagetoid cells may be the evident finding [39]. Immunohistochemistry of the reported pediatric cases has shown a CD3 + , CD4 – and CD8 + phenotype [39–43].…”

“…Histologically, a hyperplastic epidermis with epidermotropism and small to medium sized nests of pagetoid cells may be the evident finding [39]. Immunohistochemistry of the reported pediatric cases has shown a CD3 + , CD4 – and CD8 + phenotype [39–43]. Treatment modalities include surgery of the solitary lesions [41], radiotherapy [42, 43] and less common, photodynamic therapy [40].…”

Pagetoid reticulosis

“…До сих пор не полностью понятен патогенез болезни, неясно, является ли болезнь Ворингера -Колоппа истинной лимфомой или доброкачественным лимфопролиферативным процессом, подобным лимфоидному папулезу, который в некоторых случаях может трансформироваться в грибовидный микоз [2,3].…”