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.
Atopic dermatitis, commonly known as eczema, is a common chronic, relapsing skin disease characterized by pruritus, disrupted epidermal barrier function, and immunoglobulin E-mediated sensitization to food and environmental allergens. Atopic dermatitis is a complex disease that arises from interactions between genes and the environment. Loci on several chromosomes have been identified, including a family of epithelium-related genes called the epidermal differentiation complex on chromosome 1q21. Mutations in filaggrin, a key protein in epidermal differentiation, have also been identified in early-onset and severe atopic dermatitis. There are 3 classical stages of eczema: infantile, childhood, and adulthood. The spectrum of eczema presentation varies widely from a variant that only affect the hand to major forms where a patient presents with erythroderma. The acute and subacute lesions of atopic dermatitis are often characterized by intensely pruritic, erythematous papules and vesicles with excoriations and a serous exudate. Chronic atopic dermatitis is exemplified by lichenified plaques and papules with excoriations. Atopic dermatitis patients are also at higher risk for skin infections, including bacterial and viral superinfections. Conventional therapy includes avoidance of irritants and potential allergens, as well as continued hydration of the skin with thick emollients. Topical corticosteroids and topical immunomodulators are often used primarily. Other therapies including phototherapy, antimicrobials, antihistamines, and systemic immunosuppressives are also options in certain situations.
[ O R I G I N A L R E S E A R C H ]
ABSTRACTObjective: To evaluate the efficacy of combination cryotherapy and imiquimod 3.75% cream versus cryotherapy alone in the treatment of hypertrophic actinic keratosis on the dorsal hand and forearm. Methods: Twenty subjects with at least three hypertrophic actinic keratoses on each dorsal hand or forearm underwent cryotherapy treatment to hypertrophic actinic keratoses. Following cryotherapy, subjects were randomized to have either their right or left dorsal hand or forearm treated with imiquimod 3.75% cream to begin on the same day as cryotherapy treatment. Subjects then utilized the two weeks on, two weeks off, two weeks on regimen of imiquimod 3.75% cream application. Local skin reactions were also assessed. Results: For the cryotherapy/imiquimod 3.75% arm, the median total hypertrophic actinic keratosis reduction was -5.12 and for the cryotherapy alone arm, the median total hypertrophic actinic keratosis reduction was -2.24 (P<0.0094). Limitations: Local skin reactions unblind the investigator. Conclusion: Cryotherapy plus imiquimod 3.75% cream resulted in a statistically significant improvement in the reduction of hypertrophic actinic keratoses than cryotherapy alone at 14 weeks. (J Clin Aesthet Dermatol. 2013;6(2):36-43.)
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%.
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.