Segmental Darier's disease: A case report.

Segmental Darier's disease is an uncommon subtype of Darier's genodermatosis, resulting from a mutation in the ATPase type 2 during early embryogenesis. It typically presents as a persistent, pruritic papular eruption following the lines of Blaschko. Histopathology of Darier's disease demonstrates acantholysis, dyskeratosis, and corps ronds. First-line treatment includes topical retinoids, calcineurin inhibitors, and synthetic vitamin D analogues. Severe disease may require systemic therapy with oral retinoids, immunomodulators, magnesium, and low-dose naltrexone. Segmental Darier's disease is important to recognize both clinically and histologically as it may resemble other acantholytic Blaschkolinear dermatoses and should be considered in individuals presenting with a chronic localized papular eruption in a Blaschkoid distribution. Herein, we present a case of a 48-year-old male with segmental Darier's disease who improved significantly following acitretin treatment.

First Presentation of Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome in Pregnancy.

BACKGROUND: Reed's syndrome, also known as hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome, is an autosomal dominant condition in which affected individuals may develop cutaneous leiomyomas, uterine fibroids, and renal cell carcinoma. OBJECTIVE: This report describes a unique case of HLRCC because it presented in pregnancy with development of cutaneous pilar leiomyomas. METHODS: Review of the literature for previous cases of Reed's syndrome during pregnancy including PubMed and Medline search. RESULTS: Genetic testing of this patient demonstrated a mutation in the fumarate hydratase (FH) gene. Review of the literature showed only 1 previous case series that described the onset of cutaneous lesions during pregnancy. CONCLUSION: This case serves as a reminder that there may exist a correlation between pregnancy and the first manifestation of cutaneous lesions in patients with HLRCC, and thus an increased clinical suspicion is warranted during this period.

Wait times for Mohs micrographic surgery in Ottawa, Ontario.

BACKGROUND: The Ministry of Health and Long-Term Care, in coordination with Cancer Care Ontario, records and analyzes wait times for cancer surgery in the province of Ontario. However, this strategy does not include wait times for skin cancer surgery. PURPOSE: The wait times and referral patterns of patients undergoing Mohs micrographic surgery at The Ottawa Hospital (TOH) were examined to better assess the adequacy of access to skin cancer treatment in Ontario. METHOD: The records of 101 Mohs surgeries (96 patients) consecutively performed at TOH between June 14, 2010, and October 19, 2010, were reviewed. The interval between the date the referral for Mohs surgery was first received and the date of surgery was calculated for each case. The specialty of the referring physician and the postal code of each patient treated were also recorded. RESULTS: The average wait time between the date of referral and the date of surgery was 122.6 days or 17.5 weeks (median 124 days, 17.7 weeks). Over 75% of patients waited over 12 weeks (84 days) for cancer surgery. All Mohs surgery patients treated at TOH resided within postal districts in eastern and northern Ontario. CONCLUSION: The current wait time for Mohs surgery in the Ottawa region is beyond the standard for cancer treatment. Improving access to care and incorporating Mohs surgery into the Ministry of Health and Long-Term Care's Wait Time Strategy might significantly improve this.

Immediate and delayed hypersensitivity to systemic corticosteroids: 2 case reports.

BACKGROUND: Both immediate, type I reactions and delayed hypersensitivity, type IV reactions to systemic corticosteroid preparations have been reported. Type I reactions are rare, with delayed hypersensitivity reactions being slightly more common. CASES: A 33-year-old woman presented repeatedly to the emergency department with asthma attacks. She developed pruritus and hives approximately 30 minutes after the administration of parenteral corticosteroids. Her respiratory status deteriorated approximately 6 hours after she received the corticosteroids. An acute eczematous dermatitis on her face, neck, and upper body appeared 24 hours after administration of the corticosteroids. The dermatitis peaked at 72 hours. Intradermal testing to Solu-Medrol, Solu-Cortef, prednisone, and Decadron confirmed a type I, anaphylactoid reaction. The dermatitis that presented 24 hours after administration of the parenteral corticosteroids is consistent clinically with a type IV delayed hypersensitivity reaction to the corticosteroids. A second patient, a 51-year-old woman, developed urticarial lesions that lasted approximately 30 minutes, immediately after intralesional triamcinolone injections for keloid scars. Intradermal testing was performed. She showed a positive reaction to triamcinolone confirming a type I allergy to this steroid. CONCLUSIONS: It is important to consider an allergy to corticosteroids in patients with worsening anaphylactic symptoms after administration of systemic corticosteroids.

Actinic superficial folliculitis in a 29-year-old man.

BACKGROUND: Since actinic superficial follicultis was first described in 1985, only three further cases have been published. The characteristics of this disease are monomorphous, superficial, follicular pustules that appear on the back, upper chest, and shoulders annually after the first sun exposure of the year. The lesions resolve on their own within 10 days. Recurrence occurs under similar conditions after a latency period of at least 4 weeks. METHODS: A 29-year-old man presented to our clinic with a 5-year history of an intermittent follicular rash. These eruptions occurred on his back and chest only when he was exposed to the sun, with his shirt off. They developed 24 to 36 hours after the first sun exposure of the year and resolved spontaneously after 5 to 7 days. Photographs of the affected area were impressive, with follicular pustules grouped along his left flank. CONCLUSION: According to our literature search, this is the sixth reported case of actinic superficial folliculitis. This is the first case in which provocative phototesting was done. We review the clinical and pathologic attributes of actinic superficial folliculitis.

A 10-year review of p-phenylenediamine allergy and related para-amino compounds at the Ottawa Patch Test Clinic.

BACKGROUND: p-Phenylenediamine (PPD) is an important allergen; 5.0% of patients tested positive to PPD when patch-tested, according to the North American Contact Dermatitis Group. Hair dyes are the main source of exposure. OBJECTIVE: To assess the significance of PPD allergy at the Ottawa Patch Test Clinic. METHODS: We assessed the epidemiology of PPD allergies and determined the cross-reactivity with other para-amino compounds. Charts of patients visiting the Ottawa Patch Test Clinic between May 1997 and July 2009 were reviewed. RESULTS: One hundred thirty-four patients were found to have a contact allergy to PPD; 75.4% were female, 24.6% were male, 13.4% were hairdressers, 18.7% had a history of atopy, 90.3% were sensitized by hair dye, 2.2% were sensitized by henna tattoos, and 7.5% were sensitized by other sources. Positive patch-test reactions to textile dyes were seen in 24.6%, 7.5% reacted to benzocaine, 6.0% reacted to sulfa drugs, 1.5% reacted to isopropyl-para-phenylenediamine, and 1.5% reacted to para-aminobenzoic acid. CONCLUSIONS: PPD is an important source of allergic contact allergy. Our results show a significant relationship of PPD with other related para-amino compounds.