Porokeratotic eccrine ostial and dermal duct nevus associated with an 11 megabase 3p deletion.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare eccrine hamartoma; the etiology is incompletely understood. A patient presented with congenital, widespread PEODDN. Clinical assessment, histopathologic, cytogenetic, and molecular genetic investigations on affected cells were pursued. Histopathology confirmed PEODDN, and chromosomal microarray on affected tissues identified a mosaic 3p26.3p25.3 deletion in affected tissues. This 11Mb deletion encompasses 47 OMIM genes. We propose that this and other chromosomal deletions may be implicated in some cases of PEODDN, suggesting locus heterogeneity and underscoring the importance of incorporating cytogenetic and molecular investigations into the multidisciplinary care of individuals with suspected mosaic genetic skin disorders.

Topical rapamycin (sirolimus) for the treatment of uncomplicated tufted angiomas in two children and review of the literature.

The mTOR inhibitor rapamycin is used systemically for the treatment of vascular lesions. We report the first use of topical rapamycin for the successful treatment of two cases of tufted angioma. The evidence for the use of topical rapamycin in other dermatologic conditions is summarized to aid in clinical decision making on preparations and anticipated side effects.

Pediatric Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Halted by Etanercept.

We report a case of an 11-year-old female with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap, most likely triggered by sulfamethoxazole-trimethoprim, who was treated with the combination of methylprednisolone, cyclosporine, and etanercept. Her condition stabilized and her skin involvement did not progress after the addition of etanercept. To our knowledge, this is the first report of etanercept for pediatric SJS/TEN.

Derivation, Evaluation, and Validation of Illustrations of Key Counselling Points for a Pediatric Eczema Action Plan.

BACKGROUND: Current eczema action plans (EAP) are based on written instructions without illustrations. Incorporating validated illustrations into EAPs can significantly improve comprehension and usability. OBJECTIVE: To produce and validate a set of illustrations for key counselling points of a pediatric EAP. METHODS: Illustrations were developed using key graphic elements and refined by subject experts. Illustrations were evaluated during one-on-one structured interviews with parents/caregivers of children ages 9 and younger, as well as with children ages 10 to 17 years between September 2015 and June 2016. The concepts of transparency, translucency, and short-term recall were assessed for validation. RESULTS: Of 245 participants, 81.3% were parents and/or caregivers of children 0 to 9 years old, and 18.7% were children between 10 and 17 years old. A total of 15 illustrations and 2 storyboards were evaluated; 9 illustrations and 2 storyboards were redesigned to reach the preset validation targets. Overall, 13 illustrations and 2 storyboards were validated. CONCLUSION: A set of illustrations for use in an EAP was prospectively designed and validated, achieving acceptable transparency, translucency, and recall, with input from patients and a multidisciplinary medical team. The incorporation of validated illustrations into eczema action plans benefits patients with limited health literacy. Future studies should evaluate if illustrations improve understanding of eczema management and translate into improved clinical outcomes.

Usability, Satisfaction, and Usefulness of an Illustrated Eczema Action Plan.

BACKGROUND: An eczema action plan (EAP) is an individualized tool to help caregivers and patients self-manage eczema. While novel illustrated EAPs have been developed and validated, there is limited literature examining the value of EAPs from patient and caregiver perspectives. OBJECTIVES: The objective of this study was to test the usability, satisfaction, and usefulness of our validated EAP from the perspective of patients and caregivers. METHODS: Consecutive patients from the pediatric dermatology clinic of a tertiary children's hospital from July 2016 to July 2017 were offered enrolment in a prospective survey study; informed consent was obtained from participants. The illustrated EAP was explained to the participant by a trained research assistant. Participants were sent electronic postvisit surveys using Likert scale questions via REDCap on EAP usability and satisfaction (9 items) as well as on usefulness (3 items). RESULTS: Of 233 consecutive clinic patients, 192 participants (82%) were enrolled, and 112 (58%; 85 caregivers and 22 patients) completed the postvisit surveys. Characteristics were similar between responders and nonresponders. Overall, participants rated the usability (96%), satisfaction (85%), and usefulness (78%) of the EAP positively. Education level, experience with eczema, previous dermatology consultation, and participant type (caregiver vs patient) did not significantly affect the usability or usefulness ratings. However, caregivers' overall EAP ratings were significantly higher ( P = .02) than the patients'. CONCLUSION: The caregivers and participants demonstrate that the EAP is a useful and highly usable tool. Future research should examine the effectiveness of EAP use on objective atopic dermatitis outcomes using a pragmatic clinical trial design.

Primary Cutaneous Histoplasma capsulatum Infection in a Patient Treated With Fingolimod: A Case Report.

Fingolimod is an immune-modulating drug used in the treatment of multiple sclerosis. Histoplasma capsulatum is a dimorphic fungus that can infect humans. Infection with the pathogen typically affects the lungs, but it is usually asymptomatic and self-limited. However, immunocompromised patients infected with the pathogen can present atypically, including the development of primary cutaneous lesions. We describe an interesting clinical case of a cutaneous H capsulatum infection in a patient treated with fingolimod.

The Effectiveness of Written Action Plans in Atopic Dermatitis.

Atopic dermatitis (AD) is a chronic dermatosis requiring a stepwise and dynamic approach to management. The use of written action plans has been shown to improve outcomes in other chronic diseases that require a similar incremental approach. A systematic review was performed to evaluate the effect of a written eczema action plan (EAP) in AD management and to identify characteristics of effective action plans in children with eczema. Only two trials were identified as eligible, which highlights the need for more research on EAPs.

Leukemia cutis and facial nerve palsy as presenting symptoms of acute lymphoblastic leukemia.

Leukemia cutis and facial nerve palsy are rare presenting symptoms of leukemia. This report describes a case of acute T-cell lymphoblastic leukemia (ALL) presenting with only these two symptoms, a presentation of ALL that, to our knowledge, has not been previously described. It serves to alert physicians to look for underlying malignancy in the setting of cutaneous findings associated with facial nerve palsy.

Segmental infantile hemangioma with milia: A case report.

Milia are common in children. They are small, keratinizing cysts that arise either as primary epidermoid cysts or secondary to other dermatoses, trauma or certain medications. In the paediatric population, milia are most frequently congenital and resolve spontaneously. Infantile hemangiomas are relatively common in neonates. They typically arise within the first few weeks of life, undergo a proliferative phase in the first 6 months, then begin to involute at around 12 months of age. After involution, residual skin changes may be observed, such as telangiectasia, fibrofatty tissue and redundant skin. However, there is a gap in the literature regarding concomitant milia and infantile hemangiomas. We report a case of a 5-months-old female who presented with a large, segmental infantile hemangioma of the posterior neck with milia.

Noninferiority and Safety of Nadolol vs Propranolol in Infants With Infantile Hemangioma: A Randomized Clinical Trial.

Importance: Propranolol for infantile hemangiomas (IH) has been shown to be effective and relatively safe. However, other less lipophilic ß-blockers, such as nadolol, may be preferable in individuals who experience propranolol unresponsiveness or adverse events. Objective: To document the noninferiority and safety of oral nadolol compared with oral propranolol in infants with IH. Design, Setting, and Participants: This double-blind noninferiority prospective study with a noninferiority margin of 10% compared propranolol with nadolol in infants aged 1 to 6 months with problematic IH. The study was conducted in 2 academic pediatric dermatology centers in Canada between 2016 and 2020. Infants aged 1 to 6 months with a hemangioma greater than 1.5 cm on the face or 3 cm or greater on another body part causing or with potential to cause functional impairment or cosmetic disfigurement. Interventions: Oral propranolol and nadolol in escalating doses up to 2 mg/kg/d. Main Outcomes and Measure: Between-group differences comparing changes in the bulk (size and extent) and color of the IH at week 24 with baseline using a 100-mm visual analog scale. Results: The study included 71 patients. Of these, 36 were treated with propranolol. The mean (SD) age in this group was 3.1 (1.4) months, and 31 individuals (86%) were female. Thirty-five infants were treated with nadolol. The mean (SD) age in this group was 3.2 (1.6) months, and 26 individuals (74%) were female. The difference in IH between groups by t test was 8.8 (95% CI, 2.7-14.9) for size and 17.1 (95% CI, 7.2-30.0) for color in favor of the nadolol group, demonstrating that nadolol was noninferior to propranolol. Similar differences were noted at 52 weeks: 6.0 (95% CI, 1.9-10.1) and 10.1 (95% CI, 2.9-17.4) for size and color improvement, respectively. For each doubling of time unit (week), the coefficient of involution was 2.4 (95% CI, 0.5-4.4) higher with nadolol compared with propranolol. Safety data were similar between the 2 interventions. Conclusions and Relevance: Oral nadolol was noninferior to oral propranolol, indicating it may be an efficacious and safe alternative in cases of propranolol unresponsiveness or adverse events, or when faster involution is required. Trial Registration: ClinicalTrials.gov Identifier: NCT02505971.

A unique combination of Rosai-Dorfman disease and mycosis fungoides: a case report.

Rosai-Dorfman disease (RDD) is a rare histiocytic condition of unknown etiology. Patients with RDD classically present with massive painless cervical lymphadenopathy. However, extra-nodal disease occurs in approximately 40% of cases, with the skin being among the most commonly involved sites. Patients with isolated extra-nodal involvement may present without adenopathy. Reports of RDD occurring in patients with Hodgkin's lymphoma, and B-cell lymphoma have been published, but there has only been one previous report of RDD in a patient with a T-cell lymphoma. This case report documents a unique combination of RDD and mycosis fungoides (MF), a cutaneous T cell lymphoma. This report also highlights diagnostic challenges in RDD due to the rarity of the condition and its variable presentation.

Treatment of Darier's disease with oral magnesium: a case report.

Darier's disease, an autosomal dominant genodermatosis, arises from a mutation in the ATP2A2 gene that codes for sarco/endoplasmic reticulum Ca2+-ATPase in the endoplasmic reticulum and is characterized by greasy keratotic papules commonly found in seborrheic regions. Conventional treatments, including topical corticosteroids, antibiotics, antifungals and retinoids, often have limited efficacy. The present article reports the novel use of oral magnesium chloride supplementation (300 mg daily) in the treatment of Darier disease. After 5 years of limited improvement using conventional therapies, significant improvements in neck lesions were observed within 1 month of starting oral magnesium chloride. This suggests that oral magnesium chloride may be an effective therapeutic option for Darier disease, although further in vitro and clinical trials are necessary to evaluate its clinical efficacy.

Atypical presentation of congenital yellow nail syndrome in a 2-year-old female.

BACKGROUND: Yellow nail syndrome (YNS) is a rare clinical entity of unknown etiology that is characterized by a triad of yellow nails, respiratory manifestations, and lymphedema. The condition appears in the mid- to later years of life and only rarely in childhood. We describe a rare case of YNS with an atypical clinical presentation consisting of only yellow and dystrophic nails in a 2-year-old female since birth. OBJECTIVE: A case of congenital YNS with only dystrophic and yellow nails is reported. METHODS AND RESULTS: A 2-year-old female presented with yellow nails since birth. There was no positive family history. Physical examination revealed 20 thickened, dystrophic, yellow nails with onycholysis. There was no evidence of respiratory manifestations or lymphedema. CONCLUSION: Although rare, YNS can present as a congenital clinical entity and persist after birth. Pediatric patients with YNS show different clinical manifestations than the classic adult patient. The presence of yellow and dystrophic nails in the absence of respiratory and lymphatic manifestations may be the only sign of pathology and warrants close monitoring as progression to more serious complications can occur.

Dynaclose tape: a practical alternative to punch biopsy site closure.

BACKGROUND: A punch biopsy is a common therapeutic and diagnostic procedure that is routinely performed by dermatologists. A thin cylinder of tissue is removed with a biopsy punch, which creates a full-thickness wound. Sutures are used for primary closure as part of standard practice and are removed in follow-up 5 to 14 days later. The biopsy site heals with a small linear scar. In the past, punch biopsies were often left to heal by secondary intention, but given the extended healing time, this has fallen out of practice among dermatologists. OBJECTIVE: The purpose of this case report is to introduce a new quick and cost-effective approach to accelerate secondary intention healing following punch biopsies in busy dermatology clinics. RESULTS/CONCLUSION: We present a case where Dynaclose tape (Canica Design Inc, Almonte, ON) was used as an alternative to sutures for biopsy site closure. Dynaclose tape is a strip with adhesive ends and a clear center composed of silicone elastomer. The silicone elastomer promotes wound closure by pulling the opposing margins together. The tape maintains excellent adhesion and follows the natural shape of the skin to maximize patient comfort and mobility.

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.

Alopecia areata following adalimumab.

BACKGROUND: Alopecia areata is a nonscarring hair loss characterized by well-circumscribed patchy areas, most often on the scalp. The inflammatory cytokine tumor necrosis factor alpha (TNF-alpha), has been connected with the development of alopecia areata in vivo; thus, the TNF-alpha inhibitors have been cited as possible treatments for this autoimmune condition. OBJECTIVE: We report a case of alopecia areata that developed in a 52-year-old woman who was recently started on adalimumab for treatment of her psoriatic arthritis. RESULTS: We discuss the previously published cases in the literature linking alopecia areata to TNF-alpha inhibitor administration. Our case is the first report of a new-onset alopecia areata following adalimumab. CONCLUSIONS: Even though TNF-alpha is implicated in causing alopecia areata, TNF-alpha inhibitors have paradoxically been associated with new cases of alopecia areata. It is possible that TNF-alpha may not be involved in the pathogenesis of alopecia areata, as in vitro studies have suggested.

Becker nevus on the lower limb: case report and review of the literature.

BACKGROUND: Becker nevus is a hamartoma with brown hyperpigmentation and hypertrichosis that usually affects young males. It has a predilection for the upper half of the trunk and proximal upper extremity. OBJECTIVE: This is a case report of a 38-year-old male with a Becker nevus without hypertrichosis on the lower limb and a review of the literature. RESULTS: Including our case report, 12 cases of Becker nevus on the lower limb were identified in the English literature (8 males and 4 females). It has never been reported below the knee. In males, the lesion was not associated with other anomalies, and all but two showed hypertrichosis (six of eight). In 50% (two of four) of women, it was associated with localized lipoatrophy. In one woman, it presented without hypertrichosis. CONCLUSION: The presence of Becker nevi on the lower limb is unusual, and hypertrichosis is not always a feature. Larger studies are required to look into the true prevalence of this presentation and its prognostic value as a marker for other anomalies.