ABSTRACT
BACKGROUND: Poikilodermatous mycosis fungoides (pMF) is characterized by poikiloderma areas, typically involving the major flexural areas and trunk. Its presentation can be generalized or admixed with other forms of MF. Previous studies fail to correlate the clinical presentation with prognosis and laboratory findings. Some reports show pityriasis lichenoides chronica (PLC) preceding the poikiloderma. OBJECTIVES: Correlate prognostic, histopathological and molecular aspects of pMF with its clinical presentation. METHODS: Retrospective analysis of 14 cases of generalized pMF (GpMF), 22 of localized pMF (LpMF) and 17 of pMF admixed with other forms of MF (mix-pMF). RESULTS: Female predominance and lower age at diagnosis was found in all groups compared to classic MF, a high prevalence of PLC-like lesions in the GpMF group and a high rate of hypopigmented lesions in the mix-pMF group. There were 2 deaths within the GpMF group. Histology was similar to previously reported findings, as was the prevalence of CD4 T-cell infiltrate, compared to CD8. The T-cell clonality positivity was lower in the GpMF group, compared to other groups (27% GpMF, 80% LpMF and 100% mix-pMF). DISCUSSION: This is the first article to categorize the different forms of pMF and correlate them with clinical and laboratory findings. The dermatological presentation differs among the groups. There was a high frequency of PLC-like lesions within the GpMF group and of hypopigmented lesions in mix-pMF. The histological and immunohistochemical findings were similar to those previously reported. Aggressive treatments are not recommended due to the good prognosis of all pMF forms. The low positivity of T-cell clonality in the GpMF group should be investigated.
Subject(s)
Mycosis Fungoides/diagnosis , Parapsoriasis/diagnosis , Skin Neoplasms/diagnosis , Clone Cells/pathology , Female , Humans , Immunohistochemistry , Male , Middle Aged , Mycosis Fungoides/pathology , Mycosis Fungoides/therapy , Parapsoriasis/pathology , Parapsoriasis/therapy , Prognosis , Retrospective Studies , Skin Neoplasms/pathology , Skin Neoplasms/therapy , T-Lymphocytes/pathologyABSTRACT
Frontal fibrosing alopecia (FFA) is an inflammatory cicatricial alopecia, which is considered to be a variant of lichen planopilaris. In addition to follicular changes, FFA often presents with associated cutaneous manifestations in most patients, including lichen planus pigmentosus, implantation line hypochromia and facial papules. The objective of the present article is to provide a detailed overview of the non-follicular cutaneous clinical manifestations of FFA and discuss their impact on the diagnosis and treatment of patients with this condition.