Topical tacrolimus: a novel therapeutic intervention for recalcitrant labial pemphigus vulgaris

We report a case of a persistent lip ulcer in a patient with mucocutaneous pemphigus vulgaris, recalcitrant to various topical and systemic corticosteroids and immunosuppressants, which resolved following the administration of topical tacrolimus.     

Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease)

Familial benign pemphigus is a chronic, recurrent, autosomal dominant blistering disease that may significantly affect quality of life. Surgical methods are often needed to control flares in difficult cases. We describe the use of photodynamic therapy (PDT) with topical 5-aminolevulinic acid (ALA) followed by irradiation with incoherent light (ALA-PDT) in 2 patients with chronic, recalcitrant familial benign pemphigus.     

Minocycline is a useful adjuvant therapy for pemphigus

Pemphigus is an autoimmune blistering disease with high mortality if untreated. The cases of 10 patients who had minocycline 100 mg daily added as adjuvant therapy are reported. Prior to the use of minocycline, all patients had active disease, nine were on prednisolone (10–40 mg) and five were on azathioprine (100–200 mg). The response was assessed on clinical improvement and reduction of immunosuppressive (IS) drugs. It was graded into four categories: major, minor, equivocal and no significant response. A major response was seen in four patients, minor in two, equivocal in one and no improvement in three patients. The prednisolone dose in the six responders was reduced to 0–6 mg (0 mg in three patients), with an average decrease of 21 mg. The average time to respond was 8 months. Of the six responders, three were on azathioprine, which was ceased in two patients and reduced by two-thirds in the other patient. No patient ceased minocycline because of side effects. In conclusion, minocycline 100 mg daily is a simple, safe and well tolerated treatment that should be tried in patients with pemphigus to reduce disease activity and/or the dose of potent IS agents.     

Atypical herpes simplex infection masquerading as recalcitrant pemphigus vulgaris

A 57-year-old woman presented with refractory genital erosive disease. One year earlier she experienced gingival fragility; direct immunofluorescence resulted in the diagnosis of cicatricial pemphigoid, and prednisone therapy led to initial improvement. Initial skin biopsy of her genital erosions demonstrated full-thickness ulceration with viral cytopathic change and a re-epithelializing subepidermal separation. Indirect immunofluorescence revealed intercellular IgG staining on monkey oesophagus at a titre of 1:320 consistent with pemphigus, leading to the diagnoses of pemphigus vulgaris with herpetic superinfection. Immunosuppressive treatment initially led to improvement; however, disease subsequently recurred as extensive genital erosions. We diagnosed atypical herpes simplex virus infection and oral candidiasis, discontinued all immunosuppressive medications, and initiated antiviral and antifungal therapy. Dramatic resolution was observed and the patient has remained free of disease for 13 months while taking only prophylactic famciclovir.     

Gold therapy for pemphigus vulgaris.

Six patients with proven pemphigus vulgaris have been treated with gold. Serious toxicity did not occur but “pseudotoxicity” was common. During the initial phase of treatment two patients developed a rash that cleared quickly. They both had a long history of mild minima disease and refused further treatment. Although the natural history of pemphigus vulgaris has not definitely been documented it would seem that gold has beneficially influenced disease activity in these patients.     

Corticosteroids as an adjunct to propranolol for infantile haemangiomas complicated by recalcitrant ulceration

A small subset of patients with infantile haemangiomas (IHs) can present with serious complications, the most common of which is ulceration. Ulcerated IHs can be extremely painful and always result in scarring. Numerous studies support the efficacy and reduced side‐effects of propranolol relative to systemic corticosteroids, which led to the adoption of propranolol as the mainstay of IH treatment. However, in certain cases of IH with complex ulceration, propranolol monotherapy may not be sufficient. In this case report, we present two cases that illustrate the effectiveness of the adjunctive use of oral corticosteroids for the treatment of select IHs with recalcitrant painful ulceration, which were refractory to conservative wound care, laser therapy and oral propranolol. We suggest a continuing niche role for the brief use of corticosteroids as an effective adjunct to oral propranolol in managing a subset of complex IHs complicated by intractable ulceration.     

光动力疗法治疗寻常痤疮的研究进展

寻常痤疮是临床上最常见的皮肤病之一,是一种由多因素综合作用引起的,累及毛囊皮脂腺的慢性炎症性皮肤病,其发病率呈逐年上升的趋势,并且影响患者的生活质量.传统的治疗方法为局部或系统治疗,具有潜在的毒副作用,加之细菌对抗生素耐药性的不断增加.近年来,光动力疗法已广泛应用于痤疮的治疗中,并成为治疗痤疮的有效方法,尤其是针对炎症性痤疮,其疗效优于单独光照治疗.光动力疗法的光源主要有二极管激光、强脉冲光、蓝光、红光.研究结果显示,该疗法安全、有效,不良反应较小.     

Beneficial effects of topical tacrolimus on recalcitrant erosions of pemphigus vulgaris

We report a case of pemphigus vulgaris in which a recalcitrant area of erosion on the cheek cleared only when topical tacrolimus was used in addition to a regime of systemic therapy consisting of cyclophosphamide and prednisolone. Clinical improvement occurred within 10 days of applying topical tacrolimus with healing of erosions and reduction in pain and burning sensations. Topical tacrolimus may inhibit local activation of T lymphocytes through altered expression of cytokines such as interleukin-1, -4 and -5, tumour necrosis factor-alpha and interferon-gamma. Some of these cytokines may also contribute directly to increasing keratinocyte fragility in the aetiology of pemphigus vulgaris erosions. This case illustrates that topical tacrolimus may be a useful adjunct in the management of patients with pemphigus vulgaris.     

Immunopathological examination of esophagus as a useful criterion of cure in pemphigus vulgaris

BACKGROUND: We performed histopathological and immunopathological examinations of the esophagus in patients with pemphigus vulgaris during clinical remission. METHODS: In the group of 14 patients without serum antibodies, five were treated with low doses of steroids and cyclophosphamide (as maintenance treatment), while nine had already completed the therapy. RESULTS: In all five cases under maintenance treatment we found bound pemphigus antibodies in vivo. Acantholysis was present in two of these. In all nine non-treated patients, acantholysis and immunopathological findings were negative. CONCLUSIONS: Our studies reveal that the absence of bound 'pemphigus vulgaris' antibodies in the esophagus by immunological examination could be regarded as proof of complete cure and could be the decisive finding to stop pemphigus treatment.     

Two courses of rituximab (anti-CD20 monoclonal antibody) for recalcitrant pemphigus vulgaris

Background Pemphigus vulgaris (PV) is a severe autoimmune blistering disease involving the skin and mucous membranes. The response to therapy varies greatly amongst patients and treatment may be challenging. Rituximab is a chimeric monoclonal antibody that selectively targets cell surface antigen CD20, thus depleting mature B cells in vivo. Methods We report the results of rituximab treatment in two patients with severe PV. In both patients, high‐dose oral prednisolone and adjuvant therapy with intravenous immunoglobulins and mycophenolate mofetil failed to control disease activity. Consequently, the patients were treated with two courses of four weekly intravenous infusions of rituximab (375 mg/m²) with a 6‐month interval. Results Clinical improvement was already noticeable 3–6 weeks after the first infusion. After the second course, complete remission was achieved. Oral prednisolone was reduced and treatment with mycophenolate mofetil was continued. The patients remained in full remission 6 months after the last rituximab infusion. Conclusion These cases suggest that two courses rather than a single course of rituximab may be a preferable mode of treatment. Rituximab should be considered as a promising treatment option for recalcitrant PV.     

Rituximab therapy in severe juvenile pemphigus vulgaris

Juvenile pemphigus vulgaris (PV) is a rare and often misdiagnosed condition. Although PV frequently is severe in children, a substantial portion of the morbidity and mortality associated with juvenile PV has been attributed to treatment. This report demonstrates the efficacy of rituximab therapy in juvenile PV. We report 2 cases and review the literature. Rituximab treatment was effective in helping to control 2 recalcitrant cases of juvenile PV without inducing the adverse effects associated with other adjuvant therapies. Rituximab should be considered when treating resistant cases of PV in pediatric populations to avoid the long-term side effects of other immunosuppressive treatments.