J.M. Mascaró

Roaccutane Treatment Guidelines: Results of an International Survey

BACKGROUND Oral isotretinoin (Roaccutane) revolutionized the treatment of acne when it was introduced in 1982. METHODS Twelve dermatologists from several countries with a special interest in acne treatment met to formally review the survey of their last 100 acne patients treated with oral isotretinoin. The primary purpose of the survey was to identify the types of acne patients who were prescribed oral isotretinoin and how the patients were managed. RESULTS Of the 1,000 patients reviewed, 55% of those who received oral isotretinoin had those indications treated historically, i.e. severe nodular cystic acne or severe inflammatory acne, not responding to conventional treatment. Forty-five percent of patients who were prescribed oral isotretinoin however had either moderate or mild acne. Most patients in this group had moderate acne (85%). However, 7.3% had mild acne on physical examination. The criteria for prescribing oral isotretinoin in this less severe group of patients included acne that improves < 50% after 6 months of conventional oral antibiotic and topical combination therapy, acne that scars, acne that induces psychological distress and acne that significantly relapses during or quickly after conventional therapy. Treatment is usually initiated at daily doses of 0.5 mg/kg (but may be higher) and is increased to 1.0 mg/kg. Most of the physicians aimed to achieve a cumulative dose of > 100-120 mg/kg. Mucocutaneous side-effects occur frequently but are manageable while severe systemic side-effects are rarely problematic (2%). The teratogenicity of oral isotretinoin demands responsible consideration by both female patients and their physicians. Significant cost savings when treating acne patients with oral isotretinoin as compared to other treatment modalities were further proven in this study. CONCLUSIONS Our recommendation is that oral isotretinoin should be prescribed not only to patients with severe disease but also to patients with less severe acne, especially if there is scarring and significant psychological stress associated with their disease. Acne patients should, where appropriate, be prescribed isotretinoin sooner rather than later.

Subacute cutaneous lupus erythematosus: Clinicopathologic findings in thirteen cases

The clinicopathologic and serologic findings of thirteen patients with subacute cutaneous lupus erythematosus are reported. The clinical and immunologic features are similar to those described by most other authors. From a histologic point of view, however, two aspects could be emphasized: the presence of a larger number of epidermal colloid bodies and severe epidermal necrosis (more than 60% of cases). All patients with this microscopic picture have a similar clinical and serologic pattern: annular lesions, anti-Ro antibodies and human leukocyte antigen-DR3.

Eosinophilic ulcer of the oral mucosa: another histological simulator of CD30+ lymphoproliferative disorders

Eosinophilic ulcer of the oral mucosa (EUOM), also known as traumatic ulcerative granuloma with stromal eosinophilia or Riga–Fede disease, is an uncommon benign self‐limited lesion poorly described in the dermatological literature. It probably includes a spectrum of related disorders presenting as an ulcer with elevated indurated borders affecting the tongue, oral mucosa or lip. Histopathological findings are characteristic and consist of eosinophil‐rich mixed infiltrates accompanied by a population of large mononuclear cells whose origins have been a matter of debate. Immunohistochemical studies of these cells have suggested a myofibroblastic or histiocytic origin. We present a 93‐year‐old woman with two episodes of self‐healing ulcers on the upper lip and on the lingual mucosa, respectively. Histopathological findings on both biopsies were consistent with EUOM and showed the presence of large atypical CD30+ lymphocytes. Some recent reports have also shown positivity for the CD30 antigen, raising the possibility that a subset of EUOM could be included within the spectrum of CD30+ lymphoproliferative disorders. This finding most likely suggests that EUOM can represent another histological simulator of CD30+ lymphoproliferative disorders.

The Role of Fcγ Receptor Polymorphisms in the Response to Anti–Tumor Necrosis Factor Therapy in Psoriasis: A Pharmacogenetic Study

IMPORTANCE Variability in genes encoding proteins involved in the immunological pathways of biological therapy may account for the differences observed in outcomes of anti–tumor necrosis factor (TNF) treatment of psoriasis. OBJECTIVE To assess the role of 2 Fcγ receptor (FcγR) polymorphisms in the response to anti-TNF therapy in psoriasis. DESIGN Retrospective series of patients with psoriasis who received anti-TNF therapy(infliximab, adalimumab, or etanercept) from January 1, 2007, through December 31, 2010. Patients were followed up for 12 weeks. SETTING Two psoriasis referral centers. PARTICIPANTS Seventy treatment-naive patients with moderate to severe psoriasis who received anti-TNF agents. INTERVENTION Patients underwent FcγRIIA-H131R and FcγRIIIA-V158F polymorphism genotyping. MAIN OUTCOMES AND MEASURES The Psoriasis Area and Severity Index and the body surface area were assessed at baseline and at treatment weeks 6 to 8 and 12. The polymorphism genotypes were correlated with the treatment outcomes. RESULTS Bivariate analysis showed a nonsignificant association between FcγR low-affinity genotypes and greater improvement in the Psoriasis Area and Severity Index and body surface area at the end of treatment. Conversely, patients harboring high-affinity alleles presented a greater reduction in body surface area at the intermediate point, which remained independent in the multivariate analysis. We also detected an additive effect of both polymorphisms in the multivariate analysis. High-affinity alleles may contribute to a quicker response owing to a more efficient removal of relevant cells expressing TNF. CONCLUSIONS AND RELEVANCE Preliminary results of this pilot study on the pharmacogenetics of FcγR and biological therapy in psoriasis suggest a role with clinical implications for FcγRIIA-H131R and FcγRIIIA-V158F polymorphisms in the outcome of anti-TNF treatment of psoriasis. These results might help dermatologists in guiding therapeutic decisions, especially in very severe cases where a quick response is needed.

Mortality of bullous pemphigoid in the first year after diagnosis: a retrospective study in a Spanish medical centre

Background  Prognosis of patients with bullous pemphigoid (BP) is controversial, with a 1‐year mortality rate ranging from 6% to 48%.

Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

Pemphigus vulgaris is an infrequent but life-threatening autoimmune blistering disease that is rare in the pediatric age. Although the mainstay of therapy for childhood pemphigus vulgaris (CPV) is steroids, adjuvant immunosuppressive drugs are often needed to control the disease. Thus, an important part of CPV morbidity can be related to treatment. We report the youngest CPV patient described in the English literature, an 18-month-old boy, who has been followed up for 16 years. During this period, the patient has received several immunosuppressive therapies with variable response. He has finally achieved a long-lasting and complete remission with rituximab. Successful treatment with rituximab has previously been reported in 6 cases of CPV, in whom rituximab presented a good side effect profile. Our patient has experienced a chronic and severe clinical course with multiple flares eventually developing vegetative lesions. Since he presented refractoriness to multiple therapies, we administered rituximab. The introduction of this drug led to a dramatic clinical response and a long-term clinical remission. Based on the experience of this case and the data reported in the literature, we believe that rituximab may be a safe and efficacious agent for the treatment of severe CPV.

Human irritant response to qualities and concentrations of cocoamidopropylbetaines: a possible model of paradoxical irritant response

Cocoamidopropylbetatnes arc surfactants frequently used in cosmetics. We have evaluated the irritant capacity of 3 different qualities of cocoamidopropylbetaine, using the following method; patch tests have been carried out with 5 different dilutions in 67 patients and the results read at 2 days by noninvasive methods (direct visualization, transepidermal water loss (TEWL). laser Doppler flowmetry (LDF)). The results with the 3 methods were concordant. However, the results with the different concentrations were paradoxical, as irritancy did not increase at higher concentrations We have tried to explain this by the fact that these substances contain by‐products (free amidoamine and sodium monochloroacetate), the concentrations tested all being above the critical micelle concentration and therefore containing both micelles and monomer. Finally, we believe that noninvasive methods such as TEWL and LDF could be of treat use in the evaluation of irritant contact dermatitis

Papuloerythroderma of Ofuji: A Report of 2 Cases Including the First European Case Associated with Visceral Carcinoma

Papuloerythroderma (PE) is a rare type of erythroderma of the elderly which results from the coalescence of sheets of papules that spare skin folds, with peripheral eosinophilia in most cases. Skin biopsy shows a nonspecific eczematous pattern with a mature T cell lymphocytic and eosinophilic infiltrate with Langerhans cells in the dermis. We report 2 cases of PE. The 1st case, in which no underlying malignancy could be found, responded partially to oral etretinate, topical steroid creams and tar derivates. The 2nd case did not respond to high-dose oral steroids and was associated with adenocarcinoma of the colon, with a fatal outcome. PE has been associated with lymphoma and carcinoma. We report the first European case of PE associated with visceral carcinoma and review the cases reported in the western literature.

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.

Pegylated interferon alfa-induced sarcoidosis: two sides of the same coin.

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