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Clinical, Cosmetic and Investigational... 2024Restricted pagetoid reticulosis, also known as Woringer-Kolopp disease, represents a rare cutaneous lymphoproliferative disorder categorized as an isolated variant of...
Restricted pagetoid reticulosis, also known as Woringer-Kolopp disease, represents a rare cutaneous lymphoproliferative disorder categorized as an isolated variant of mycosis fungoides. This report presents a case involving limited pagetoid reticulosis affecting the right upper extremity in a 25-year-old female. The patient had been experiencing plaques on the right upper extremity for a decade. Dermatologic examination revealed well-defined scaly plaques on the right forearm, surrounded by hyperpigmented patches. Skin histopathology demonstrated atypical mononuclear cell infiltration in the lower part of the epidermis, forming nests. Immunohistochemistry indicated CD3+, CD4+, CD5+, CD7+, CD8+, CD30+, and Ki-67-positive staining. Additionally, CD20, CD79α, and PD-1 were negative. Monoclonal rearrangement of T-cells was identified in TCR β and TCR γ through clonality assessment. The diagnosis of limited paget-like reticulocyte hyperplasia was established, leading to surgical resection. A review of the literature affirmed the variable immunophenotype of pagetoid reticulosis, with atypical cells exhibiting four types: (1) CD3+, CD4+, CD8+-type; (2) CD3+, CD4-, CD8+-type; (3) CD3+, CD4-, CD8-type; and (4) CD3+, CD4+, CD8+-type-relatively uncommon in the restrictive type. This case report details the clinical features, histologic and morphologic characteristics, immunohistochemical phenotype, diagnosis, and differential diagnosis of a rare CD3+, CD4+, CD8+ limited pagetoid reticulosis. The lesion was surgically resected, and the patient underwent a 3-year follow-up to observe its prognosis.
PubMed: 38827631
DOI: 10.2147/CCID.S462966 -
Pathology International Sep 2023DUSP22-rearranged primary cutaneous anaplastic large-cell lymphoma (pcALCL) has a biphasic histological pattern defined by large dermal atypical lymphocytes and... (Review)
Review
Constant small-cell changes and variable LEF1 expression in DUSP22-rearranged primary cutaneous anaplastic large-cell lymphoma: Analysis of the repeated biopsies of three patients.
DUSP22-rearranged primary cutaneous anaplastic large-cell lymphoma (pcALCL) has a biphasic histological pattern defined by large dermal atypical lymphocytes and epidermotropic small lymphocytes resembling pagetoid reticulosis, but the positivity rate of the biphasic pattern in DUSP22-rearranged pcALCL is unknown. Immunohistochemically, LEF1 expression in >75% of tumor cells is associated with DUSP22-rearrangement (DUSP22-R) in systemic ALCL. However, whether this association applies to pcALCL remains unclear. To analyze these pathological clues for screening DUSP22-R, we reviewed 11 skin biopsies from three patients with DUSP22-rearranged pcALCL. All specimens showed a biphasic pattern, of which three showed nonpagetoid infiltration of the epidermis. In all lesions, small-cell changes of tumor cells were observed not only within the epidermis but also under the epidermis. LEF1 positivity rates varied by lesion (range: 30%-90%, mean: 59.6%) with only three patients expressing LEF1 in more than 75% of tumor cells. In conclusion, the biphasic pattern was a constant finding in DUSP22-rearranged pcALCL, but it was not always pagetoid reticulosis-like. The recognition of small-cell change outside the epidermis may be helpful in diagnosing DUSP22-rearranged pcALCL. However, LEF1 expression was variable and its diagnostic usefulness may be limited.
Topics: Humans; Lymphoma, Large-Cell, Anaplastic; Pagetoid Reticulosis; Biopsy; Skin Neoplasms; Lymphoid Enhancer-Binding Factor 1; Dual-Specificity Phosphatases; Mitogen-Activated Protein Kinase Phosphatases
PubMed: 37530485
DOI: 10.1111/pin.13360 -
Photodermatology, Photoimmunology &... Jul 2023
Topics: Humans; Pagetoid Reticulosis; Dermoscopy; Skin Neoplasms; Melanoma; Microscopy, Confocal
PubMed: 36178448
DOI: 10.1111/phpp.12837