Evaluation of Th-1 and Th-2 Immune Responses in the Skin Lesions of Patients with Blau Syndrome

Immunohistochemical Evidence of Specific Iris Involvement in Blau Syndrome

“…Granulomas obtained from skin, lymph tissue, spleen, and bone marrow of patients with documented Blau mutations demonstrate increased CD4 + /CD8 + T-cell ratio. 7,12,13 Granulomas were absent in this study sample, as described in a previous report. 9 This observation was also not unexpected, as the patient had been treated for a prolonged period with immunosuppressive medications and had long-standing disease quiescence.…”

Widespread Granulomatous Dermatitis of Infancy

“…52 They tend to be arranged in clusters or linear arrays but can become confluent, and a perifollicular distribution may be evident. 3,4,14,24,27,32,53,54 Intermittent episodes of widespread skin disease with spontaneous resolution usually occur during a period of years (in contrast to the more persistent skin lesions of sarcoidosis). Poikiloderma has been observed in older patients, 13,27 and pitted scars due to follicular atrophoderma are sometimes apparent at sites of previous inflammatory papules.…”

“…3,4,14,24,27,32,53,54 Intermittent episodes of widespread skin disease with spontaneous resolution usually occur during a period of years (in contrast to the more persistent skin lesions of sarcoidosis). Poikiloderma has been observed in older patients, 13,27 and pitted scars due to follicular atrophoderma are sometimes apparent at sites of previous inflammatory papules. 2,3,27,[54][55][56][57] Ichthyosiform skin changes characterized histologically by a hyperkeratotic epidermis with underlying dermal granulomas have also been described in EOS/BS.…”

“…Poikiloderma has been observed in older patients, 13,27 and pitted scars due to follicular atrophoderma are sometimes apparent at sites of previous inflammatory papules. 2,3,27,[54][55][56][57] Ichthyosiform skin changes characterized histologically by a hyperkeratotic epidermis with underlying dermal granulomas have also been described in EOS/BS. 2,4,27,30,34,55 Histologically, the naked noncaseating granulomas of EOS/BS involve primarily the upper dermis and often have a perifollicular distribution.…”

Papel das células T reguladoras no desenvolvimento de dermatoses

“…2,3 The skin is an immunological organ that has antigens for T cells and produces many types of cytokines and inflammatory mediators. 4,5 The complex relationships between cells and inflammatory mediators in skin diseases are starting to be unveiled. Several diseases are defined by the production of antibodies and lymphocytes against infectious and environmental antigens.…”