Fibrous Hamartoma of Infancy: A Firm Plaque Presenting with Hypertrichosis and Hyperhidrosis.

Fibrous hamartoma of infancy (FHI) is a rare, benign, soft tissue tumor. The lesion's clinical resemblance to more aggressive soft tissue tumors of childhood makes histologic diagnosis paramount. We report a unique presentation of a FHI associated with localized hypertrichosis and hyperhidrosis.

The use of mycophenolate mofetil for the treatment of autoimmune and chronic idiopathic urticaria: experience in 19 patients.

BACKGROUND: There is a paucity of investigation on which to base the treatment of chronic urticaria after a patient fails maximum therapy with antihistamines. One prospective, open-label, uncontrolled study suggested that mycophenolate mofetil may be a successful second-line therapy. OBJECTIVE: We sought to evaluate the efficacy and safety of mycophenolate mofetil in 19 patients with autoimmune and chronic idiopathic urticaria. METHODS: In a retrospective chart review, records of patients with autoimmune and chronic idiopathic urticaria who were evaluated between 2001 and 2009 were analyzed. RESULTS: Improvement in urticaria was observed in 89% of patients, specifically 91% of patients with autoimmune urticaria and 88% with chronic idiopathic urticaria. Time to initial improvement ranged from 1 to 9 weeks. In 59% of these patients, complete control of urticaria was achieved, which included 70% of patients with autoimmune urticaria and 43% with chronic idiopathic urticaria. Mean time to complete control was 14 weeks, with a range of less than 1 to 31 weeks. The dose of mycophenolate mofetil at complete control ranged from 1000 to 6000 mg divided twice daily. Mycophenolate mofetil was tapered in 7 of these 10 patients after an average of 7 weeks. Six of the 7 patients tapered then discontinued mycophenolate mofetil with remissions lasting between 2 and 16 weeks up to when the chart review ended. Mycophenolate mofetil was well tolerated with no serious infections or laboratory abnormalities. Gastrointestinal symptoms were most common. LIMITATIONS: This was a retrospective chart analysis. The number of patients was relatively small. CONCLUSIONS: Mycophenolate mofetil is a useful and well-tolerated second-line therapy for patients with autoimmune and chronic idiopathic urticaria in whom antihistamines and other therapeutic agents have failed.

Developments in systemic immunomodulatory therapy for psoriasis.

Psoriasis is an inflammatory skin condition that can be accompanied by joint disease. Pre-biological and biological systemic therapies are effective. Dermatologists have used systemic immunomodulators including methotrexate to treat moderate-to-severe disease for over 30 years. Pre-biological agents have toxicities and side effects that can be difficult to tolerate and require frequent monitoring. Beginning with alefacept in 2003, several biologics including cell-adhesion-molecule antagonists and cytokine antagonists such as tumor necrosis factor-blockers gained approval for psoriasis. They greatly advanced our understanding of psoriasis pathogenesis. Because they are so new, their safety is not established. Tumor necrosis factor-blockers have controversial associations with certain cancers and infections. Biologics require clinical monitoring and have specific contraindications. Scientists are exploring several new therapeutic targets.

Unilateral facial paralysis after treatment of secondary syphilis.

Bell's palsy is an acute facial paralysis of unknown etiology. Infections including syphilis have been implicated as causes for peripheral facial paresis. The Jarisch-Herxheimer reaction is an acute worsening of skin manifestations and systemic symptoms occurring after administration of antimicrobial therapy for spirochetal infections. Although rare, neurological signs can present as part of the Jarisch-Herxheimer reaction. The authors report a case of Bell's palsy experienced by a patient shortly after treatment with penicillin for secondary syphilis and propose that this acute unilateral peripheral facial paralysis was a Jarisch-Herxheimer reaction in response to therapy.

Successful treatment of Hailey-Hailey disease with acitretin.

Hailey-Hailey disease is an autosomal dominant skin condition characterized by waxing and waning painful and pruritic vesicles and plaques affecting the intertriginous areas. Its pathogenesis involves inherited abnormalities in a cutaneous calcium pump. Most patients are managed conservatively with topical corticosteroids as well as topical and oral anti-infective agents. Scarce reports in the literature describe the use of oral retinoid therapy to manage refractory cases. We present a case of Hailey-Hailey disease in a 64-year-old man who was refractory to conservative management but improved dramatically over 6 months of oral therapy with 25 mg of acitretin daily. The mechanism by which such therapy improves disease manifestations is unknown. A potential mechanism is based on the influence of retinoids on epidermal differentiation and may involve cutaneous calcium homeostasis. Hailey-Hailey disease is discussed and the use of oral retinoid treatment for Hailey-Hailey disease is reviewed.

Management and prevention of varicella-zoster virus infection in pregnancy: a case report and review of the literature.

Primary infection with varicella-zoster virus (VZV) during pregnancy can lead to devastating outcomes for both the mother and fetus. We describe a case of VZV infection in a pregnant woman who presented at 38 weeks' gestation. We also review the literature regarding management and prevention. Varicella-zoster virus-associated pneumonia in the mother is important to recognize. Outcomes in the newborn largely are dependent on gestational age at the time of infection. Prevention is paramount to management.

Experience with molluscum contagiosum and associated inflammatory reactions in a pediatric dermatology practice: the bump that rashes.

OBJECTIVE To investigate the frequency, epidemiology, clinical features, and prognostic significance of inflamed molluscum contagiosum (MC) lesions, molluscum dermatitis, reactive papular eruptions resembling Gianotti-Crosti syndrome, and atopic dermatitis in patients with MC. DESIGN Retrospective medical chart review. SETTING University-based pediatric dermatology practice. PATIENTS A total of 696 patients (mean age, 5.5 years) with molluscum. MAIN OUTCOME MEASURES Frequencies, characteristics, and associated features of inflammatory reactions to MC in patients with and without atopic dermatitis. RESULTS Molluscum dermatitis, inflamed MC lesions, and Gianotti-Crosti syndrome-like reactions (GCLRs) occurred in 270 (38.8%), 155 (22.3%), and 34 (4.9%) of the patients, respectively. A total of 259 patients (37.2%) had a history of atopic dermatitis. Individuals with atopic dermatitis had higher numbers of MC lesions (P < .001) and an increased likelihood of molluscum dermatitis (50.6% vs 31.8%; P < .001). In patients with molluscum dermatitis, numbers of MC lesions increased during the next 3 months in 23.4% of those treated with a topical corticosteroid and 33.3% of those not treated with a topical corticosteroid, compared with 16.8% of patients without dermatitis. Patients with inflamed MC lesions were less likely to have an increased number of MC lesions over the next 3 months than patients without inflamed MC lesions or dermatitis (5.2% vs 18.4%; P < .03). The GCLRs were associated with inflamed MC lesion (P < .001), favored the elbows and knees, tended to be pruritic, and often heralded resolution of MC. Two patients developed unilateral laterothoracic exanthem-like eruptions. CONCLUSIONS Inflammatory reactions to MC, including the previously underrecognized GCLR, are common. Treatment of molluscum dermatitis can reduce spread of MC via autoinoculation from scratching, whereas inflamed MC lesions and GCLRs reflect cell-mediated immune responses that may lead to viral clearance.

Hypersensitivity reactions to vaccine constituents: a case series and review of the literature.

Vaccines are composed of immunogens, preservatives, adjuvants, antibiotics, and manufacturing by-products. Components of vaccines may rarely elicit adverse reactions in susceptible individuals, thus raising concerns regarding vaccine safety. In this report, we add to the medical literature 3 cases of cutaneous delayed-type hypersensitivity to the vaccine preservative aluminum. We provide a review of major constituents in vaccines that have elicited immediate-type or delayed-type hypersensitivity reactions and describe their clinical manifestations. We include a table of the Food and Drug Administration-approved vaccines, which lists the quantities of major components including ovalbumin (egg protein), gelatin, aluminum, neomycin, 2-phenoxyethanol, thimerosal, and formaldehyde. Our goals were to inform physicians on the variety of hypersensitivity reactions to common vaccines and to provide information on the choice of vaccines in patients with suspected hypersensitivity.