Pachydermodactyly in a patient with Tourette syndrome

Pachydermodactyly in a patient with Tourette syndrome

Omalizumab, a therapeutic option for delayed pressure urticaria Parameatal cysts: A case presentation and review (Poster reference number 5075) (Po...

41KB Sizes 3 Downloads 143 Views

Omalizumab, a therapeutic option for delayed pressure urticaria

Parameatal cysts: A case presentation and review

(Poster reference number 5075)

(Poster reference number 4782)

Noelia Hernandez, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain; Federica Liuti, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain; Javier Hernandez, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain; Leopoldo Borrego, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain; Yeray Pe~ nate, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain; Zaida Hernandez, MD, Hospital Universitario Insular Materno Infantil de Gran Canaria, Las Palmas de Gran Canaria, Spain Background: Omalizumab is a recombinant humanized monoclonal anti-IgE antibody. Recently, it has been used for different forms of chronic urticaria with a good response. We present a patient with refractory delayed pressure urticaria with omalizumab. Case report: A 62-year-old woman with a 7-year history of delayed pressure urticaria with no significant findings in the analytic and serologic study and an increase in IgE levels. The patient was treated with antihistamines in combination with systemic corticosteroid, doxepin and sulfona with a poor response. Cyclosporine was successfully introduced, but the dose had to be reduced due to hyperkalemia, and a new flare-up of lesions appeared. Because of the effectiveness of previous treatments, we started with omalizumab 150 mg per month. The patient is asymptomatic after 1 year of treatment. Discussion: Delayed pressure urticaria is a physical urticaria that occurs at sites of sustained pressure on the skin after a delay of several hours, and presents in 40% of patients with chronic idiopathic urticaria. The pathogenesis depends on the mast cell activation. The treatment is generally unsatisfactory, with negative impact on the quality of life. Although omalizumab has been approved for the treatment of severe uncontrolled allergic asthma, it is also recommended as a fourth-line treatment for physical, chronic urticaria and angioedema. Recently, omalizumab has been used in cases of refractory delayed pressure urticaria. Fewer than 10 cases have been reported. Conclusion: In the case of refractory delayed pressure urticaria, omalizumab might be considered a therapeutic option.

Alison Galatian, MD, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States; Carlos Garcia, MD, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States; Robert Fischer, MS, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States Parameatal (median raphe) cysts of the male genitalia are an uncommon congenital anomaly that may present to the dermatology clinic. Median raphe cysts may also be found along the frenulum of the penis or present on the scrotum or perineum. The lesion may be noted in infancy, though usually presents later in childhood. We describe a recent case of a 14-year-old boy that presented to the University of Oklahoma dermatology clinic with a parameatal cyst. The lesion was asymptomatic at the time of presentation. The patient subsequently underwent surgical removal of the cyst by pediatric urology without complication. In addition to case description and clinical presentation, we review the histologic findings and discuss therapeutic options. We feel it is important that dermatologists recognize this anomaly to provide appropriate information and management recommendations to patients and their families. Commercial support: None identified.

Commercial support: None identified.

Pott’s puffy tumor: A new case of a forgotten entity

(Poster reference number 5118)

Pachydermodactyly (PDD) is a rare, asymptomatic, benign fibromatosis characterized by fusiform soft tissue swelling of the proximal interphalangeal (PIP) joints of the second to fourth fingers and can result from tic-like behavior involving the hands. Tourette syndrome (TS), a neurodevelopmental disorder demonstrating motor and phonic tics, can be implicated as a cause of PDD. We report a 20-year-old woman with a diagnosis of TS and a 4-year history of swelling of the PIP joints of the fingers, most prominent on the second to fourth fingers. She demonstrates tic-like behaviors causing mechanical trauma and skin changes to her hands. We present an interesting case of PDD associated with joint stiffness and pain, which may signify a rare painful variant of PDD; however, it could also be a result of her tic-like movements associated with TS. In addition, PDD is seldom reported in female patients because it exhibits a 5:1 male to female ratio. While mental disorders have been linked to PDD, few reported cases actually have a definitive diagnosis of a neurological disorder in the patient. Therefore, PDD in a patient with TS is of particular interest and could add validity to previously reported cases in the literature.

Mar Blanes, MD, PhD, Dermatology Department, Marina Baixa Hospital, Villajoyosa, Spain; Irene Monjas, MD, Otorhinolaryngology Department, Hospital General Universitario of Alicante, Alicante, Spain; Javier Mataix, MD, Dermatology Department, Marina Baixa Hospital, villajoyosa, Spain; Juan Ramon Gras, MD, PhD, Otorhinolaryngology Department, Hospital General Universitario of Alicante, Alicante, Spain Background: Pott’s puffy tumor is a very rare but serious complication of frontal rhinosinusitis. It consists of a subperiosteal abscess that evolves to osteomyelitis of the frontal bone. Eventually a frontocutaneous fistula appears, with abscess formation in the soft tissues. Clinically it presents as a localized swelling of the frontal region. Case report: A 36-year-old otherwise healthy white man was referred to our dermatology outpatient clinic with complaints of swelling on the forehead. The lesion had appeared 1 week before and was completely asymptomatic. There was no previous history of headaches or injuries in this area. In the beginning, the patient had visited another medical center, where he had been diagnosed as suffering acute sinusitis, and received oral antibiotic and antiinflammatory treatment without any improvement. On physical examination, a tense swelling in the middle of the forehead was noticed, without erythema, local warmth, or fluctuation. The patient was afebrile and in good general condition. Ultrasound study showed an extracranial abscess intimately in contact with the frontal bone. Computed tomographic (CT) scanning was also performed and it showed a complete occupation of the right frontal sinus, with two bone defects (measuring 2 cm length and 5 mm, respectively) and extension of the inflammatory material to the forehead soft tissues. There was no communication with the anterior cranial fossa. A diagnosis of Pott’s puffy tumor was therefore made. The patient started aggressive intravenous broad spectrum antibiotic treatment and underwent surgical debriding and reconstruction procedures by the otorhinolaryngology department, with good results. Discussion: The frequency of this entity, first described by Sir Percival Pott in 1760, has been drastically reduced after the beginning of the antibiotic era. However, in the published cases, many severe associated complications have been described, including orbital cellulitis, intracranial dissemination with intracranial abscess formation and thrombosis of the cavernous sinus. In our opinion, dermatologists must be aware of this nowadays rare entity, in order to avoid in these patients diagnostic procedures as skin biopsies, that are contraindicated. Providing an early diagnosis and treatment is the best way to reduce complications.

Commercial support: None identified.

Commercial support: None identified.

Pachydermodactyly in a patient with Tourette syndrome

(Poster reference number 5084)

Marjorie F. Yang, MD, Indiana University School of Medicine, Indianapolis, IN, United States; Ingrida Ozols, MD, Indiana University School of Medicine, Indianapolis, IN, United States; Nikolajs J. Perdue, Indiana University School of Medicine, Indianapolis, IN, United States; Simon Warren, MBBS, Indiana University School of Medicine, Indianapolis, IN, United States

APRIL 2012

J AM ACAD DERMATOL

AB59