Our study brings evidence of a higher thrombophilic risk among the patients with early onset of the CVU as they had significantly higher prevalence of multiple (≥3) thrombophilias (P=0.03), homozygous mutations (P=0.03) and family history of leg ulcer (P=0.02) when compared with patients with later onset. Thrombophilia screening is important in patients with CVU before the age of 50 in order to stratify the thrombotic risk and to allow an appropriate prophylactic and therapeutic management.
Unilateral laterothoracic exanthem is a self-limited disease that occurs most commonly in children. It is characterized by unilateral exanthem, often in axillary region. The etiology is unknown, but a viral agent is suspected. We report a 1-year-old white girl with unilateral laterothoracic exanthem associated with Epstein Barr virus infection, suggesting this virus has a possible etiologic role.
EditorGranuloma annulare (GA) is a common non-infectious granulomatous didease. 1,2 Although no clear aetiology has been established, trauma, insect bite reactions, tuberculin skin testing, sun exposure, PUVA therapy, adverse reaction to drugs and viral infections have been proposed as inciting factors. 1 Its pathogenesis is probably immunologic. Evidence suggest that GA represents a cell-mediated immune response to an unknown antigen, in which granuloma formation by macrophage-histiocytes is mediated by interaction with T-helper lymphocytes. It is a Th1-driven inflammatory reaction, with interferon γ-producing lymphocytes eliciting matrix degradation. [3][4][5][6] Nearly 15% of patients with GA have the generalized subtype characterized by a later age of onset, increased prevalence of the HLA-Bw35 allele, protracted course, with only rare spontaneous resolution, and alterations in the lipid profile in about 45% of patients. 1,2,5 A 52-year-old Caucasian woman was observed for asymptomatic erythematous-violaceous annular plaques, with raised borders, symmetrically distributed over the neck and subsequently over the trunk and limbs, evolving for 6 months (Fig. 1). Besides rosacea, the patient was otherwise healthy. Laboratory findings, including autoimmune and hormonal screening, tumoral markers, viral, Borrelia burgdorferi and Treponema pallidum serologies and Mycobacterium polymerase chain reaction were all negative. Skin lesion biopsy showed a granulomatous dermatitis pattern with necrobiotic areas and lymphocytic infiltrate, supporting the diagnosis of GA (Fig. 2).
Fabry disease (FD) is a rare X-linked lysosomal storage disorder resulting from the deficient activity of the enzyme alpha-galactosidase A. Angiokeratomas (AKs) are a frequent manifestation of this disease. They usually become apparent during childhood and can cause important cosmetic disability. Current treatment of this feature in the setting of FD has been mainly based on the application of laser systems, namely the argon laser, the variable pulse width 532-nm Nd:YAG laser, the 578-nm copper vapor laser and the flashlamp-pumped pulsed dye laser. We report the case of a 31-year-old Caucasian woman with a clinical and molecular (GLA p.R118C) diagnosis of FD, presenting multiple AKs scattered over the buttocks and thighs. She was treated with 10 sessions of intense pulsed light (IPL), with a 4-8-week interval between them. An almost complete clearance of the lesions was obtained, with no scars or significant complaints. No recurrence occurred during a 12-month follow-up period. The IPL source can be considered a suitable, effective and safe treatment modality for these cutaneous lesions that typically affect patients with FD, with no need for local anesthesia and with very satisfactory cosmetic results. To our best knowledge, there are no reports in the literature of Fabry's AKs treated with IPL.
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