Coexistence of Lymphomatoid Papulosis, Pityriasis Lichenoides Acuta and Mycosis Fungoides – A case report.
Voudouri D,1 Nikolaou V,1 Marinos L,2 Papadavid E,1 Economidi A,1 Stratigos A,1 Antoniou C. 1
1 Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece.
2 Hemopathology Department, Evangelismos Hospital
Background. Diagnosing lymphoproliferative diseases can be a real challenge for skin experts, since a solid correlation between clinical presentation and pathological findings is mandatory for accurate classification. Furthermore, the coexistence of cutaneous lymphomas such as Lymphomatoid Papulosis (LyP) and Mycosis Fungoides (MF) has been repeatedly published. We report an interesting case of a patient in whom LyP, Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA) and MF were all, consecutively diagnosed over a 15-year period of time.
Case report. A 35-year-old woman, with a personal history of LyP, was referred to our department for phototherapy. She was diagnosed ten years ago and had been under methotrexate 20mg/weekly with partial remission. During examination, apart from typical LyP lesions, erythematosquamous patchy lesions on the breasts and hips were also noted. Incisional biopsies of both types of lesions were performed and confirmed the coexistence of LyP/type-A and MF. PCR-analysis detected the same clonal T-cell population on both biopsies. The patient was treated with PUVA with complete remission of MF and partial remission of LyP. Five years later and during pregnancy a new rash appeared, consisting of multiple papules on her trunk and extremities. The biopsy of the latter set the diagnosis of PLEVA with PCR analysis turning negative. After labour, the patient was treated with doxycycline 100mg/bid achieving partial remission.
Conclusion. This is, to our knowledge, the first report of LyP, MF and PLEVA coexistence in the same patient. After analyzing and identifying overlapping clinical patterns and pathological evidence we support that these diseases are most probably different expressions of the same pathophysiologic pathway rather than autonomous, non-related clinical entities. The over time-altering host immune responses may contribute to the development of a certain clinical expression each time. Progress inmolecular genetics may provide stronger criteria for defining these entities and any negative prognostic factors.