Loretta Fiorillo

First case series on the use of imiquimod for morphoea.

Background  Morphoea is characterized by fibrosis, which is mediated by cytokines including transforming growth factor (TGF)‐β.

Pseudomonas skin infection: clinical features, epidemiology, and management.

Pseudomonas aeruginosa is a Gram-negative bacillus that is most frequently associated with opportunistic infection, but which can also present in the otherwise healthy patient. The range of P. aeruginosa infections varies from localized infections of the skin to life-threatening systemic disease. Many P. aeruginosa infections are marked by characteristic cutaneous manifestations. The aim of this article is to provide a comprehensive synthesis of the current knowledge of cutaneous manifestations of P. aeruginosa infection with specific emphasis on clinical features and management.The ability of P. aeruginosa to rapidly acquire antibacterial resistance is an increasingly well recognized phenomenon, and the correct application of antipseudomonal therapy is therefore of the utmost importance. A detailed discussion of currently available anti-pseudomonal agents is included, and the benefits of antimicrobial combination therapy versus monotherapy are explored. Rapid clinical recognition of P. aeruginosa infection aided by the identification of characteristic cutaneous manifestations can play a critical role in the successful management of potentially life-threatening disease.

Bilateral cerebrovascular accidents in incontinentia pigmenti

Incontinentia Pigmenti is an X-linked dominant neurocutaneous disorder with central nervous system manifestations in 30% of cases, including seizures and mental retardation. Ischemic or hemorrhagic cerebrovascular accidents have been reported rarely in incontinentia pigmenti. Chart review and literature search was performed following identification of the index case. We describe a patient with incontinentia pigmenti who developed bilateral cerebrovascular accidents in the neonatal period, with resultant severe neurologic sequelae. This is the second reported case of bilateral cerebrovascular accidents in a patient with incontinentia pigmenti. This finding may be secondary to cerebrovascular anomalies, similar to those observed in the retina. Recognition of cerebrovascular accidents as a complication of incontinentia pigmenti will hopefully lead to earlier recognition and treatment.

Cutaneous squamous cell carcinoma in two pediatric lung transplant patients on prolonged voriconazole treatment

Oral voriconazole is commonly used for treatment and prophylaxis of invasive fungal disease post‐LTx. Development of cutaneous SCC has been described in adult LTx recipients, although it is extremely rare in children. We describe two Caucasian children who developed cutaneous SCC beyond three yr post‐LTx. Both developed severe photosensitivity, actinic keratosis and required curative surgical excision of the cutaneous SCC lesions. Neither patient developed metastatic lesions nor had allograft dysfunction as a result of the SCC or the change in medical treatments. The effect of voriconazole on the development of malignant skin lesions is discussed and a recommendation on dermatologic surveillance, preventive measures against phototoxicity and early treatment of SCC are provided.

Evaluation of the Efficacy and Safety of Topical Imiquimod 5% for Plaque-Type Morphea A Multicenter, Prospective, Vehicle-Controlled Trial

Background There is currently a lack of evidence-based therapies that are safe and effective for plaque-type morphea. We aimed to evaluate the therapeutic potential and safety profile of imiquimod 5% cream in plaque-type morphea. Methods We enrolled 25 adult patients from two Canadian centers with histologically confirmed plaque-type morphea. Imiquimod 5% was applied to a representative plaque, and vehicle was applied to a control plaque for 9 months. Treatment efficacy was assessed with the Dyspigmentation, Induration, Erythema, and Telangiectasias (DIET) score, histology, and ultrasound evaluation. Results and Conclusions Twenty-two patients completed the entire length of the study. Imiquimod 5% was superior to vehicle in reducing DIET scores at 3, 6, 9, and 12 months (p < .05). Induration demonstrated the greatest response. Histologic evaluation showed significant improvement or resolution of disease. However, no ultrasonographic differences were found in dermal and hypodermal thicknesses between the treatment and vehicle groups (p > .05). Adverse effects were minimal and well tolerated.

Rituximab induced psoriasis in an infant.

Rituximab is a chimeric monoclonal antibody directed against the CD20 antigen present on the surface of mature B cells. It has been widely used in the treatment of many dermatologic and rheumatologic conditions. The onset of psoriasis after rituximab therapy has not been reported in children. We report the first case of psoriasis after rituximab therapy in a 16‐month‐old boy with no other risk factors for developing psoriasis.

Localized Tetanus in a Child

The majority of physicians in practice today in developed countries have never seen a case of tetanus. The last pediatric case reported in Canada occurred in 1992. We present the case of a child who had localized tetanus despite previous partial immunization.

Xanthomata and diabetes in an adolescent with familial dysbetalipoproteinemia 9 yr after valproate-induced pancreatitis

A 14‐yr‐old girl presented with eruptive xanthomata and hypertriglyceridemia. This rare presentation led to diagnoses of diabetes and familial dysbetalipoproteinemia. Type 1 diabetes is a common childhood illness often presenting in adolescence. However, this patients past medical history revealed valproate‐induced severe acute pancreatitis with necrosis at the age of 5 yr. Diabetes, in this case, developed 9 yr later as a result of inadequate pancreatic tissue to support increasing insulin requirements during growth and adolescence. Diabetes was discovered only after the appearance of cutaneous eruptive xanthomata, which appeared due to the previously undiagnosed genetic dyslipidemia. Although the relationship between xanthomata, hypertriglyceridemia, and diabetes may be well known in adults, in children, xanthomata are very rarely the presenting feature of diabetes of any cause. The patient was treated with insulin which induced rapid resolution of hypertriglyceridemia and gradual disappearance of xanthomata. This case acknowledges the rarity of diabetes presenting with xanthomata in adolescence, highlights the importance of searching for an underlying dyslipidemia in such a case, and presents diabetes as a long‐term complication of acute pancreatitis in children.

Recurrent Gianotti-Crosti syndrome

To the Editor: Gianotti-Crosti syndrome (GCS) classically presents in children as a self-limited, symmetric erythematous papular eruption affecting the cheeks, extremities, and buttocks. While initial reports implicated hepatitis B virus as the etiologic agent, many other bacterial, viral, and vaccine triggers have since been described. A previously healthy 2-year-old boy presented with a 3-week history of a cutaneous eruption that initially appeared on his legs and subsequently progressed to affect his arms and face. Two weeks after onset of the eruption, he was immunized with intramuscular Vaxigrip influenza vaccination (Sanofi Pasteur), and new lesions appeared at the immunization site on his right upper arm. Physical examination demonstrated an afebrile child with erythematous papules on the cheeks, arms, and legs (Fig 1). He had a localized papular eruption on his right upper arm (Fig 2). There was no lymphadenopathy or hepatosplenomegaly. Laboratory investigations revealed leukocytosis (white cell count, 14,600/mm) with a normal differential, reactive thrombocytosis ( platelet count, 1,032,000/mm), a positive urine culture for cytomegalovirus, and positive IgM serology for Epstein-Barr virus (EBV). Histopathologic examination of a skin biopsy specimen from the right buttock revealed a perivascular and somewhat interstitial lymphocytic infiltrate in the superficial and mid-dermis with intraepidermal exocytosis of lymphocytes, mild spongiosis and papillary dermal edema. He was treated with 2.5% hydrocortisone cream, and the eruption resolved. Twelve months later, he presented with a similar papular eruption localized to the left upper arm at the site of a recent intramuscular influenza vaccination (Vaxigrip). Although an infection represents the most important etiologic agent, a second event involving immunomodulation might lead to further disease accentuation, thus explaining the association of GCS with vaccinations. In our case, there was evidence of both cytomegalovirus (CMV) and EBV infection as well as a recent history of immunization. Localized accentuation of papules at the immunization site was unusual, as previous cases of GCS following immunizations have had a widespread and typically symmetric eruption. It is possible that trauma from the injection or a component of the vaccine elicited a Koebner response, causing local accentuation. There are no previous reports of recurrence of vaccine-associated GCS. One report documented recurrence with two different infectious triggers. As GCS is a mild and selflimiting disease, further vaccinations are not contraindicated. Andrei I. Metelitsa, MD, FRCPC, and Loretta Fiorillo, MD, FRCPC

Branchiooculofacial syndrome and bilateral ectopic thymus: report of a family.

Branchiooculofacial syndrome (BOFS) is a rare autosomal‐dominant condition characterized by branchial cleft sinus and ophthalmologic and craniofacial abnormalities that can range from mild to severe forms. Ectopic thymus, an uncommon condition with just over 100 cases reported in the literature, is seen in BOFS. We report a family with BOFS affecting the mother and two daughters, showing the dominant inheritance and variable phenotypical expression of BOFS.