Biomedical subjects
Publications and source records attributed to H C Nousari.
Fundamental advances in the fields of molecular biology and immunology have facilitated investigation of the autoimmune blistering disorders. Recently, the use of human autoantibodies has helped to identify biologically important adhesion molecules of the skin, and the pathogenic mechanisms involved in bullous skin diseases are now being precisely defined.
Generalized chronic cutaneous lupus including lupus panniculitis in childhood is rare and usually occurs in the setting of genetic complement deficiencies. The association with antiphospholipid syndrome is even more rare. We report a 13-year-old girl with extensive lupus panniculitis since the age of 8 months and no evidence of complement deficiency. She recently developed antiphospholipid syndrome characterized by anticardiolipin antibodies and digital necrosis.
The classic description of erythema marginatum is of a few asymptomatic erythematous patches or plaques with a characteristic central clearing which may coalesce and develop a polycyclic configuration. We report a boy with a urticarial eruption without the classic lesions of erythema marginatum in whom the diagnosis of rheumatic fever was subsequently made. Histology of the urticarial lesions revealed a mixed, superficial, perivascular infiltrate with prominent eosinophils. Rheumatic fever should be considered in children with fever and urticaria-like eruptions even in the absence of the classically described erythema marginatum.
Keratitis-ichthyosis-deafness (KID) syndrome is a congenital ectodermal disorder causing erythrokeratoderma, vascularizing keratitis, and neurosensory deafness. Ichthyosis hystrix is a rare cutaneous disease characterized by well-demarcated, spiky, verrucous, linear plaques that is believed to be a clinical and pathologic chimera of two autosomal dominant diseases: epidermal nevus and epidermolytic hyperkeratosis. We present a patient with the classic triad of KID syndrome with clinical and histologic features of ichthyosis hystrix. This case demonstrates that KID syndrome comprises a spectrum of ectodermal disorders which may include diseases such as hystrix ichthyosis and deafness (HID) syndrome.
Drug-induced linear immunoglobulin A bullous disease is a subepidermal blistering disorder that most commonly occurs after exposure to vancomycin. It can clinically mimic toxic epidermolytic necrolysis. We describe an 87-year-old white woman in whom linear immunoglobulin A bullous disease developed while she was taking vancomycin and phenytoin. A few days after the linear immunoglobulin A bullous disease developed, both medications were discontinued. No new bullae developed, and the eruption completely resolved within 2 weeks. The patient was treated with only topical therapy.