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Biomedical subjects

H C Nousari

Publications and source records attributed to H C Nousari.

At least 55 records · Page 3Linked to original sources
PubMed1999

Severe nonendemic pemphigus foliaceus presenting in the postpartum period.

The new onset of pemphigus in the postpartum period is exceedingly rare with only 1 case previously reported in the literature. We describe a 31-year-old woman who developed extensive denudation of her skin shortly after delivering her second child. Histologic examination, immunofluorescence testing, and immunoprecipitation with desmoglein 1 confirmed the diagnosis of pemphigus foliaceus. The patient responded to treatment with prednisone, cyclophosphamide, and plasmapheresis. The effect of the postpartum period on the onset and exacerbation of immune-mediated bullous diseases is discussed.

Adult↗
PubMed1999

Clinical, demographic, and immunohistologic features of vancomycin-induced linear IgA bullous disease of the skin. Report of 2 cases and review of the literature.

Administration of intravenous vancomycin has been associated with the development of linear IgA bullous disease (LABD). In contrast to the idiopathic variant, vancomycin-induced LABD (VILABD) appears to be more transient and to be associated with lower morbidity. The characteristics of this entity remain undefined. Our analysis of clinical, demographic, and immunopathologic features of 2 new and 14 previously reported patients with VILABD reveals that VILABD is clinically and immunopathologically indistinguishable from its idiopathic variant. A variety of premorbid conditions and concomitant medications were observed, none of which was consistently associated with the development of VILABD. VILABD occurs independently of vancomycin trough levels, resolves promptly upon discontinuation of vancomycin, and recurs more severely and with shorter onset latency with vancomycin rechallenge. This entity should be recognized as 1 of the adverse cutaneous effects of intravenous vancomycin, and warrants prompt diagnosis through direct immunofluorescence skin examination.

Adult↗
PubMed1999

Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis.

A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.

Adult↗
PubMed1999

"Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.

We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. Review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases.

Adult↗
PubMed1999

Critical overview: adverse cutaneous reactions to psychotropic medications.

BACKGROUND: Adverse cutaneous reactions (ACRs) are common, potentially life-threatening or symptomatically and cosmetically unappealing side effects of psychotropic drugs. DATA SOURCES: A MEDLINE search of the literature was employed to cite the association of various psychotropic drugs with specific cutaneous reactions. DATA SYNTHESIS: In addition to the common exanthematous eruption, we explore several serious reactions including erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis, urticaria, angioedema, anaphylaxis, hypersensitivity syndrome, hypersensitivity vasculitis, erythroderma, and drug-induced lupus erythematosus. Other side effects such as alopecia, pigmentary disorders, photosensitivity, lichenoid lesions, fixed drug eruptions, and psoriasiform, acneiform, and seborrheic eruptions are discussed. Attention is paid to the morphology and distribution, systemic findings, diagnosis, and treatment of these conditions. CONCLUSION: Awareness of ACRs will allow psychiatrists to deter their continuation or recurrence, educate patients who have them, and diagnose serious instances of them.

Anaphylaxis↗
PubMed1998

Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. Patients with PNP develop characteristic IgG autoantibodies directed against multiple antigens, most of which have been identified as cytoplasmic proteins of the plakin family (desmoplakin I, II, BPAG1, envoplakin, and periplakin). This study identified cell surface target antigens of PNP. We focused on desmoglein (Dsg) 3 and Dsg1, the autoantigens of pemphigus vulgaris and pemphigus foliaceus. ELISA using baculovirus-expressed recombinant Dsgs (rDsg3, rDsg1) has revealed that 25 out of 25 PNP sera tested were positive against Dsg3 and 16 of 25 were positive against Dsg1. All of 12 PNP sera tested immunoprecipitated Dsg3. Removal of anti-Dsg3 autoantibodies by immunoadsorption was sufficient to eliminate the ability of PNP sera to induce cutaneous blisters in neonatal mice in vivo. Furthermore, anti-Dsg3-specific antibodies that were affinity purified from PNP sera were proven to be pathogenic and caused blisters in neonatal mice. These findings indicate that Dsg3 and Dsg1 are the cell surface target antigens in PNP and that IgG autoantibodies against Dsg3 in PNP sera play a pathogenic role in inducing loss of cell adhesion of keratinocytes and causing blister formation.

Acantholysis↗
PubMed1997

Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome.

BACKGROUND: Molecular defects in the glycoprotein fibrillin are believed to be responsible for impaired structural integrity of cardiovascular, skeletal, and ocular tissues in Marfan's syndrome (MFS). Traditionally, excellent results have been achieved with the Bentall composite graft repair of aneurysms of the ascending aorta in MFS. However, because of the potential complications associated with prosthetic valves, there is growing interest in techniques that preserve the native aortic valve. METHODS: Between May 1994 and February 1995, 15 patients with a history of concomitant or remote aortic root aneurysms or dissection underwent operation for valvular heart disease. Specimens of aortic valve, ascending aortic wall, and mitral valve were obtained specifically to observe differences in fibrillin content and architecture between patients with (n = 9) and without (n = 6) MFS. In addition, control specimens of aortic valve, aortic wall, and mitral valve were obtained from 4 patients with isolated valvular or coronary artery disease but no evidence of connective tissue disorders or other aortic pathologic conditions. Fibrillin immunostaining using indirect immunofluorescence was used. Specimens were coded and graded by a blinded observer to determine quantity, homogeneity, and fragmentation of fibrillin. RESULTS: Observed fibrillin abnormalities in MFS and control patients were limited to the midportion (elastin-associated microfibrils) of the aortic valve, aortic wall, and mitral valve tissues. Fibrillin abnormalities of aortic valve, aortic wall, and mitral valve tissues were seen in all patients with MFS and were most severe in those older than 20 years. Similar fibrillin abnormalities of aortic valve and aortic wall specimens were observed in control patients more than 60 years old. CONCLUSIONS: Even in the setting of a normal-appearing aortic valve, the current rationale for widespread use of valve-sparing repairs of aortic root aneurysms in patients with MFS and patients older than 60 years should be carefully reexamined in light of these findings.

Adolescent↗
PubMed1997

Anti-epiligrin (laminin 5) cicatricial pemphigoid.

Cicatricial pemphigoid is an autoimmune disease predominantly of elderly patients. Recently, a subtype of cicatricial pemphigoid with antibodies to epiligrin (laminin 5) was described. We report a patient in whom anti-epiligrin cicatricial pemphigoid was identified. He presented with cutaneous, oral and ocular involvement. He has shown initial improvement on corticosteroids and cyclophosphamide.

Autoantibodies↗