Agminated Spitzoid Naevi after Remission of Langerhans Cell Histiocytosis: First Italian Case and Literature Review.

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Lipoid Proteinosis: A Previously Unrecognized Mutation and Therapeutic Response to Acitretin.

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Frequent Occurrence of Aplasia Cutis Congenita in Bullous Dermolysis of the Newborn.

Bullous dermolysis of the newborn (BDN) is a subtype of dystrophic epidermolysis bullosa characterized by rapid improvement in skin fragility within the first months of life, associated with typical immunofluorescence and ultrastructural features. Inheritance can be autosomal dominant or recessive. We report here 4 cases of BDN, 2 of which presented with aplasia cutis congenita of the lower extremities. All patients improved rapidly and blister formation ceased by the third month of life in 3 cases. In these patients only residual milia, nail dystrophies and atrophic scarring at sites of aplasia cutis were visible by one year. Family history indicated dominant inheritance in 2 cases, confirmed by identification of COL7A1 mutation. Molecular analysis also revealed recessive inheritance in the 2 sporadic cases. A literature search identified several patients with BDN born with skin defects localized to the lower extremities. In conclusion, these findings indicate that aplasia cutis congenita is not an infrequent manifestation of BDN

Bosentan treatment of digital ulcers related to autoimmune disorders

Endothelin 1, a powerful endogenous vasoconstrictor and mitogen, may be causal to pulmonary hypertension. Evidence also suggests that endothelin (ET) may have a fundamental role in scleroderma pathogenesis, including pulmonary arterial hypertension (PAH), a leading cause of death in patients with scleroderma. The development of a new class of drug, ET receptor antagonists, provides an improved outlook for patients with scleroderma and related diseases. Increasing vigilance toward early detection of PAH in scleroderma and a multidisciplinary approach to diagnosis and treatment may improve the clinical outcome for these patients. We describe the efficacy and safety of bosentan, an orally active dual ET-receptor antagonist, in patients with digital ulcers. Bosentan increases exercise capacity and improves hemodynamics in patients with pulmonary hypertension, suggesting that ET has an important role in pulmonary hypertension but in our experience also for refractory skin ulcers. Drug Dev Res 72:750755, 2011. (C) 2011 Wiley Periodicals, Inc

Treatment of erythrodermic psoriasis in HCV+ patient with adalimumab

Erythrodermic psoriasis is a severe and disabling variant of psoriasis. The authors present the case of a 48-year-old man with psoriasis and hemophilia presented with a history of hepatitis C virus (HCV) infection treated with pegylated interferon alpha-2a and ribavirin therapy. At the end of antiviral therapy, skin manifestation progressively worsened, becoming erythrodermic, with lack of efficacy of steroid therapy. The authors decided to start biological therapy with induction dose of adalimumab (Humira, Abbott Laboratories, Abbott Park, Chicago, IL) 80 mg at Week 0 and 40 mg weekly. In our case, this resulted in a highly effective and safe treatment

Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations

Abstract Background Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement. Case presentation We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years. Conclusions Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder

Skin Ulcers In a Patient Afflicted With Microscopic Polyangiitis

Systemic vasculitis is a group of heterogeneous diseases characterized by inflammation and blood vessel walls necrosis. Usually the skin is one of the first organs involved, especially with damage of small to medium size vessels. The cutaneous patterns may help clinicians to diagnose these diseases at the beginning of their course, preventing complications due to internal organ involvement. The following case presents a patient with a microscopic polyangiitis that started with several skin ulcerations localized on the inferior limbs

Morphea, an unusual side effect of anti-TNF-alpha treatment

[No abstract available

Safety and efficacy of Adalimumab in the treatment of moderate to severe palmo-plantar psoriasis: an open label study

Introduction. Psoriasis of the hands and feet is highly debilitating and difficult to treat. Lesions are very painfull, disabilitating and impair quality of life of patients. Most treatment options have limited efficacy, short duration of response and several adverse events. Objective. To investigate the safety and efficacy of Adalimumab in the management of palmo-plantar psoriasis. Patients and methods. Adults patients with moderate to severe palmoplantar psoriasis were enrollend in this trial. They received a 6 courses of Adalimumab 40 mg 1 vial every 2 weeks. The study consisted of treatment period of 12 weeks (Weeks 1-12). Safety and efficacy were assessed at weeks 0.6 and 12. PGA (Physician's Global Assesment) and DLQI were used to measure the efficacy. Primary end point of the study was to evaluate patients who achieved a reduction in PGA at week 12. The secondary end point was to evaluate patients who achieved a 50% reduction in PGA at week 12. The tertiary end point evaluated patients who achieved a PGA rating of clear or almost clear. Results. Of 11 patients enrolled 6 showed overall improvement of at least one point of PGA at week 12; 4 of them obtained a PGA of 0 while 5 patient of 11 a >= 50% improvement from the beginning of the study. 8 patients showed an increase in quality of life score while receiving the drug at week 12. No serious adverse events were reported during the study. Conclusion. Continuous treatment with Adalimumab for 12 weeks was safe and efficacious in this open-label clinical trial of patients with palmoplantar psoriasis. Clin Ter 2012; 163(2):e61-6