Trichodysplasia spinulosa (TS) is a rare skin condition caused by trichodysplasia spinulosa-associated polyomavirus (TSPyV). It affects immunosuppressed patients, and <50 cases have been reported. The majority of these cases are seen in solid organ transplant recipients. TS often poses a diagnostic and therapeutic challenge because How to cite this article: Jose A, Dad T, Strand A, et al. Trichodysplasia spinulosa: Case reports and review of literature.
Trichilemmomas are benign cutaneous proliferations derived from the outer root sheath of the hair follicle. They most often occur on the head and neck region and show a female predominance. When multiple, they are associated with Cowden syndrome (CS), a rare disorder due to an autosomal dominant germline mutation in PTEN (phosphatase and tensin homolog on chromosome 10), a tumor suppressor gene. Trichilemmomas outside of the head and neck region are rare, and as such, the association with CS is not clear. A 28-year-old healthy female with no significant family history of cancer presented to her dermatologist with multiple erythematous papules on the left anterior ankle, starting at birth. A shave biopsy confirmed the diagnosis of trichilemmoma with focal desmoplastic features (or desmoplastic trichilemmoma). A PTEN immunohistochemical study showed patchy (but not complete) loss of staining of the lesional cells. After shave removal, the trichilemmomas recurred 1-2 months later.
Objective: to evaluate the functional properties of neutrophils in patients with IgA deficiency. Material and methods. We have studied the functional state of neutrophils (phagocytosis, ability to form active forms of oxygen and extracellular traps) in 58 patients (33 women, 25 men) with the confirmed diagnosis of partial IgA deficiency (38 patients) and general variable immune deficiency (20 patients) with a decreased content of IgA and IgG. Results. Patients with IgA deficiency reveal increased oxygen-dependent ability of blood neutrophils to form extracellular traps. The maximum degree of increase in the indices of netosis is found in patients with a lower IgA value (less than 0.3 g/l). Conclusion. Patients with a defect in the production of IgA observe disturbances of the functional properties of blood neutrophils by way of activation of their ability to form extracellular networks. This fact can be one of the prerequisites of increased patients` predisposition to develop autoimmune pathology.
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