Karine Boufleur

Hypertrophic lichen planus-like atopic dermatitis: a case report

Results The lesions started at age 18 during the patient s first pregnancy and had a predominant flexural distribution pattern. At the physical exam, the patient presented with erythema and infiltrative lesions in forearm and periorbital regions, and scaly erythematous papules, especially in inferior extremities. Some of these lesions presented an ulcerous center and had linear form. The patient had high specific serum IgE levels for house dust mites (Dermatophagoides farinae and D. pteronyssinus), grass and dog; sea food, fish, soy, wheat and latex. Serum total IgE was 11,100kU/L, and serology for hepatitis B and C was negative. The patient was treated with antihistamines, topical emollients and oral corticosteroids with low improvement. Because of this atypical presentation, other differential diagnoses were considered, including hypertrophic lichen planus. Skin biopsy was performed, showing hyperkeratosis, sub-acute spongiotic dermatitis and moderate acanthosis, compatible with atopic dermatitis and an evolution to lichen simplex chronicus. Conclusions Hypertrophic lichen planus is one of the differential diagnosis of atopic dermatitis and it commonly involves the flexor surfaces of the extremities bilaterally. Adultonset AD can present with non-typical morphology and localization, therefore it is important to distinguish these two entities, since lichen planus can be associated with other diseases, such as viral hepatitis.

Hydroa vacciniforme as a differential diagnosis of atopic dermatitis.

Results Patient’s symptoms started at age 4, presenting with vesiculopapular eruptions and pruritus on photoexposed areas, evolving into crusts. She had been treated with anti-histamines and topical steroids for several years, with no improvement. Sometimes the patient referred other associated symptoms, including dizziness, dyspnea, chest pain and strong pain in the thighs. Additional treatments included oral corticosteroids, moisturizers, sunscreen filter factor 30 and cyclosporine. On physical exam, the patient presented erythematous papules, infiltrated, associated with lichenification, crusts and chafing on the surface of the exposed areas in the upper limbs, anterior chest, lumbar region and lower limbs, and erythematous plaques in the nasal and malar region. Differential diagnosis included Atopic Dermatitis, Besnier’s Prurigo, Idiopathic Prurigo, Subacute Erythematosus Lupus, Protoporphyria and Contact Dermatitis. At our hospital she underwent skin biopsy, and the results showed characteristic features of Hydroa vacciniforme. The patient now is being treated with chloroquine 250mg/day, Pimecrolimus cream twice daily on the face, mometasone once a week, hydroxyzine 25mg twice daily, and sunblock factor 30. She reports great improvement of symptoms.

Diagnostic profile among patients with chronicurticaria/angioedema attending a reference clinic in Brazil: the role of auto-immunity.

Methods Two hundred and fifty four patients aged 13-80 years (202 females,79.5%), with chronic urticaria/angioedema who receive care at the Allergy Clinic of the Clinical Hospital of Ribeirao Preto Medical School in Brazil, were evaluated prospectively. Patients were inquired about triggering of symptoms by medications and/or physical agents and presence of anaphylaxis. In addition, they were investigated by skin testing and tests for eosinophilia, IgE, and hepatitis B and C. Autoimmune basis was characterized by presence of positive autologous test, anti-thyroid or antinuclear antibodies (ANA). When indicated, a skin biopsy was performed. Use of medications for urticaria was analyzed.

Oral challenge test with nsaids: evaluation of patients attending a specialty clinic in Ribeirão Preto, Brazil

Methods Prospective study including 116 patients with positive history of hypersensitivity to NSAIDs, evaluated at the Allergy Clinic of the Clinical Hospital of Ribeirao Preto Medical School, between October 2010 and July 2014. Patients were evaluated using the European Network for Drug Allergy questionnaire, and underwent single blind, placebo controlled OCT, in a hospital-controlled environment. Patients were required to be clinically stable, and to withdraw antihistamines or corticosteroids seven days prior to the OCT. Patients were given 10%, 20%, 30% and 40% of the therapeutic NSAID dosage, selecting an NSAID different from the one implicated in the allergic reaction. Fifteen minutes after each dosage, patients were evaluated with Peak Flow and blood pressure measurements, heart and respiratory rates and general examination.

Characterization of aspirin-sensitivity in patients with asthma and chronic rhinosinusitis without a history of symptoms upon acetyl-salicylic acid (ASA) ingestion: a case series

Background Sensitivity to Non-steroidal Anti-inflammatory Drugs (NSAIDs) is a possible cause of exacerbations in patients with asthma. NSAID sensitivity associated to chronic rhinosinusitis with nasal polyps features Aspirin-Exacerbated Respiratory Disease (AERD). Patients with AERD generally require long-term treatment with inhaled corticosteroids for moderate to severe persistent asthma. Respiratory reactions may begin within minutes to hours after ingestion of NSAIDs.

DKC1 mutation causing different phenotypes in a family with X-linked Hoyeraal-Hreidarsson syndrome

Background Hoyeraal–Hreidarsson syndrome (HHS) is a rare multisystem disorder characterized by intrauterine growth retardation, microcephaly, cerebellar hypoplasia, neurological deficits, aplastic anemia, and immunodeficiency. HHS is a severe variant of Dyskeratosis Congenita (DC) that displays clinical features overlapping DC, with T, B and NK immunodeficiency. Both genetic disorders presents deficiency in maintaining telomere integrity. Mutations in the DKC1 gene are responsible for the X-linked form of the disease.