Case 1: A 65-year-old man had lesions on the oral, ocular, and laryngopharyngeal mucous membranes and IgG and IgA autoantibodies reactive with the epidermal side of the basement membrane zone (BMZ). An enzyme-linked immunosorbent assay demonstrated anti-BP180NC16a antibodies. His condition improved with oral prednisolone (PSL) and betamethasone inhalation and eyedrops. Case 2: A 72-year-old woman presented with the oral, ocular, and laryngopharyngeal mucous membranal lesions as well as skin lesions. She had IgG autoantibodies reactive with the dermal side of the BMZ. She was treated with oral PSL and gargling with cyclosporine oral solution. Case 3: A 74-year-old woman had double-headed pterygium and symblepharons in the conjunctiva of both eyes. Her condition improved with oral PSL and amniotic membrane transplantation. Because the incidence of mucous membrane pemphigoid is very low in autoimmune blistering diseases and it is difficult to detect the type of disease or the target antigens, it may not be diagnosed in its early stage. We should keep this disease in mind when we examine patients with mucous membrane lesions.
Treatment of pemphigus vulgaris (PV) can be a challenge. Although the standard therapy consists of a systemic corticosteroid, immunosuppressant drugs, and intravenous gammaglobulin, the possibility of severe side effects or fatal complications often prevents us from continuing these therapies. Herein, we describe a case of severe PV treated by rituximab administration. The patient was a 37-year-old female who first noticed an erosion on the oral mucosa in 2004. She was referred to our hospital, and was diagnosed as PV based on the clinical presentation, skin biopsy findings, and titers of anti-desmoglein antibody. Treatment with a systemic corticosteroid and immunosuppressant drugs briefly relieved her symptoms; however, her disease worsened in severity in 2007. Switching of the immunosuppressant drugs and plasmapheresis were not effective. Pulsed steroid therapy with high-dose methylprednisolone had a transient effect; however, soon after, the patient developed exacerbation of the systemic erosions. Administration of rituximab at a dosage of 375 mg/m2 once weekly for four weeks produced rapid clinical remission. During the 4 years of follow-up, her anti-desmoglein antibody titer has gradually increased; however, the clinical symptoms remain mild. Our experience in this case suggests that rituximab may have other clinical benefits in addition to ablating antibodies.
Anti-signal recognition particle (SRP) myopathy that is recognized as different from PM/DM is a severe, necrotizing, immune-mediated disease characterized by rapidly progressive proximal muscle weakness and markedly elevated serum creatine kinase (CK) levels. Anti-SRP myopathy does not usually associate with skin rash and has poor responsiveness to steroid therapy. Herein, we describe a case of anti-SRP myopathy overlapping with localized morphea, which was diagnosed by histopathologic features and laboratory studies. A 44-year-old man presented with a two year history of erythema on his left upper arm and both cheeks. Although his CK level was high in the laboratory data, an initial biopsy of skin and muscle showed no histological changes consistent with DM. Six Months later, new symptoms, including muscle weakness and pain, appeared; however, no Gottronʼs papules or heliotrope rash was yet observed. At that time, a skin biopsy of cheek showed features consistent with a diagnosis of morphea, and histological examination of muscle revealed necrotizing myopathy without inflammatory cell infiltration. Notably, the anti-SRP antibody was positive in the serum. Based on these findings, we diagnosed this case as localized morphea overlapping with antiSRP myopathy. The systemic corticosteroid therapy was insufficient for controlling his symptoms; therefore cyclosporine A was added to improve his muscle weakness.
We report two patients (7- and 9-year-old girls) with transverse nasal stripe accompanied by comedones and milia in the distal third of the nose between the alar and septal cartilages. We suspect that repeated upward rubbing of the nose caused the lesions. In addition, a distortion in growth that developed at the junction of the nasal cartilages may have played a role in the pathogenesis of the disorder. To our knowledge, there have been no previous reports of this disorder in the literature from Asia.