Amylynne Frankel

Familial multiple pilomatrixomas as a presentation of attenuated adenomatosis polyposis coli

Pilomatrixomas are benign follicular tumors that occur most commonly in children. Rare multiple or familial pilomatrixomas have been associated with myotonic dystrophy and other disorders. Although sporadic pilomatrixomas and hybrid cutaneous cysts with pilomatrixoma‐like features have been observed in some kindreds with Gardner syndrome, an autosomal dominant form of familial adenomatous polyposis, no definitive association has been made with multiple or familial pilomatrixomas. Here we describe two siblings with multiple pilomatrixomas who were also found to have a family history of colonic adenocarcinoma. Genetic testing revealed a mutation in the 5′ portion of the adenomatous polyposis coli (APC) gene, in a region associated with an attenuated APC phenotype. These findings show that multiple pilomatrixomas may be the presenting symptom of patients with APC gene mutations.

A Reduction in Dosage of Acitretin to 17.5 mg/day Maintains Efficacy While Improving Tolerability

Background Oral acitretin 25 mg/day combined with a phototherapy regimen is a standard treatment for severe plaque-type psoriasis. Retinoid-related adverse events include alopecia, dry mucus membranes, pruritus, photosensitivity, elevation of liver enzymes, elevation of serum triglycerides and cholesterol and decrease of HDL, arthralgias, myalgias, eye irritation, blepharitis, photophobia, conjunctivitis, headaches, nausea, and anemia. Objective This single-center open label study evaluated the efficacy and tolerability of a reduced acitretin dose in patients with severe plaque-type psoriasis undergoing phototherapy treatment who were experiencing at least one retinoid-related adverse event. Results Of patients treated with 17.5 mg/day of acitretin, 89% demonstrated improved or comparable psoriasis area-and-severity index scores at week 12; 79% showed improvement in adverse events reported at baseline through week 12. Conclusion There is a role for a lower dose of acitretin in patients who are experiencing retinoid-related side effects and who are stabilized on a phototherapy regimen.

Laser Therapy for Psoriasis

Psoriasis is a chronic disease that can affect the skin, joints and nails and seriously affect quality of life. There are many treatments available to treat, but not cure this common disorder, including topical therapies as well as systemic treatments. When limited psoriatic plaques fail to respond to traditional therapies, one option is laser therapy. Laser therapy provides the ability for targeting lesional skin and sparing non-lesional skin. In unresponsive areas such as the scalp, shins, gluteal crease and palms and soles, laser therapy is an ideal option. Laser therapy can be applied in combination with other treatment modalities to yield more complete clearance of plaques. Examples of laser therapy include Excimer laser, Pulsed dye laser, and CO2 laser.

Compatibility Study Examining the Physical and Chemical Stability of Calcipotriene Ointment 0.005% with Three Topical Corticosteroids: Clobetasol Ointment 0.05%, Desoximetasone Ointment 0.25%, and Desoximetasone Ointment 0.05%

Combination topical therapy with calcipotriene ointment and topical corticosteroids is a useful treatment strategy for plaque psoriasis. Combination therapies involve using several drugs at once to optimize their effectiveness while potentially reducing unwanted adverse effects of the corticosteroid. Clobetasol propionate ointment 0.05% and desoximetasone ointment 0.25% are high-potency corticosteroids that are used for their anti-inflammatory and antipruritic properties in a variety of skin disorders. Calcipotriene ointment is a synthetic derivative of calcitriol, or vitamin D, used in the treatment of plaque psoriasis, but it can be inactivated by certain topical medications. Clinical trials involving the combination calcipotriene with topical corticosteroids have demonstrated clinical benefit compared with corticosteroid monotherapy. This study evaluated the physical and chemical compatibility of combination of topical therapies with a new, generic formulation of calcipotriene. Calcipotriene ointment 0.005% was compatible both physically and chemically when compounded with the high-potency topical corticosteroids clobetasol ointment 0.05% and desoximetasone ointment 0.25% and 0.05%.

Treatment of Erythrodermic Psoriasis: Practical Application of the National Psoriasis Foundation Consensus Statement

The management of erythrodermic psoriasis often presents a dermatologic challenge because of the lack of adequate high-quality medical literature concerning treatment options, side effect profiles associated with available therapies, and the development of concomitant comorbidities during treatment that further limit therapeutic options. After reviewing the literature published on erythrodermic psoriasis, taking into account the quality of the evidence-based medicine, in April 2010 the medical board at the National Psoriasis Foundation released a consensus algorithm for treating the patient with erythrodermic psoriasis. We present a case of a 53-year-old man with a 20-year history of plaque psoriasis who developed an erythrodermic exacerbation involving 100% body surface area. The patient was eventually administered cyclosporine 5 mg/kg/day divided into two equal doses, and the results of this treatment were excellent. He was eventually changed to a maintenance regimen involving cyclosporine 3.5 mg/kg/day divided into two equal doses along with methotrexate 15 mg weekly given for a 1-month period followed by a 1-month drug holiday. The patient has been successfully maintained on this intermittent regimen for the past 3 years with no evidence of toxicity.