1/4. parakeratosis variegata: a possible role of environmental hazards?We report 2 cases of parakeratosis variegata (PV) evolving from lesions beginning with characteristics of ashy dermatosis. Both patients presented with a reticulated, poikilodermatous and hyperpigmented eruption with bizarre coalescent lichenoid papules. histology showed lichenoid epidermotropic infiltrates, more pronounced in case No. 1, consistent with early malignancy. The course was chronic: after more than 10 years, systemic symptoms were not present. In patient No. 1, a monoclonal T-cell population was detected 12 years after the onset of the disease. Both patients had close contact with fertilizers and insecticides. In patient No. 2, the lesions spontaneously regressed within 3 years after cessation of exposure. PV may be a prelymphomatous stage of mycosis fungoides or some closely related cutaneous T-cell lymphoma and does not always evolve into overt malignancy. gene rearrangement detection techniques may be helpful in predicting the course of the disease.- - - - - - - - - - ranking = 1keywords = keratosis (Clic here for more details about this article) |
2/4. Keratosis lichenoides chronica. Successful treatment with psoralen-ultraviolet-A therapy.A man with keratosis lichenoides chronica is described who, unlike previously described patients, had had his eruption since infancy. Moreover, this patient had prominent oral involvement resembling lichen planus. Typically, keratosis lichenoides chronica is recalcitrant to all forms of treatment, but this patient responded substantially to psoralen-ultraviolet-A therapy. Several weeks after treatment was completed, he showed no signs of relapse.- - - - - - - - - - ranking = 0.4keywords = keratosis (Clic here for more details about this article) |
3/4. Cutaneous T-cell lymphoma arising from parakeratosis variegata: long-term observation with monitoring of T-cell receptor gene rearrangements.BACKGROUND: parakeratosis variegata is a rare skin disease first described in 1890. Even today, the disease entity remains confusing because various names indicating similar skin conditions have been used. Several cases of parakeratosis variegata have been reported to develop into cutaneous T-cell lymphoma, but there have been no reports describing the occurrence of lymphoma after a long-term follow-up period nor have T-cell receptor gene rearrangements been monitored in this disease. OBJECTIVE: Our purpose was to determine whether parakeratosis variegata (long-standing premycotic condition) can develop into cutaneous T-cell lymphoma. methods: We analyzed skin specimens from a patient with a 33-year history of parakeratosis variegata by Southern blotting using a T-cell receptor gene probe. RESULTS: We could detect apparent rearranged bands of T-cell receptor gene in the skin specimens taken in 1993 in contrast to the dna analysis in 1988 which featured no such rearranged band. CONCLUSION: This case represents a critical stage of parakeratosis variegata converting to lymphoma. Our results indicate that parakeratosis variegata generated monoclonality of T cells in its chronic course.- - - - - - - - - - ranking = 2keywords = keratosis (Clic here for more details about this article) |
4/4. The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl.parakeratosis variegata is a rare disorder with unknown aetiology. In a few cases it arises from benign skin diseases such as pityriasis lichenoides et varioliformis acuta (Mucha Habermann disease) or pityriasis lichenoides chronica. However, transformation into malignant diseases such as cutaneous T-cell lymphoma has been observed. We report an 11-year-old girl with a 10-year history of pityriasis lichenoides chronica now presenting with parakeratosis variegata. Analysis of skin infiltrating T cells showed clonally rearranged T-cell receptor gamma chains occurring with a frequency of more than 2%. This finding is compatible with the clinical observation of parakeratosis variegata transforming into a malignant T-cell disorder. We therefore suggest that patients suffering from parakeratosis variegata and other diseases such as pityriasis lichenoides et varioliformis acuta or pityriasis lichenoides chronica should be continuously monitored.- - - - - - - - - - ranking = 1.6keywords = keratosis (Clic here for more details about this article) |