Search

Background Sweet's syndrome (SS) is a neutrophilic dermatosis characterized by the abrupt onset of cutaneous, systemic and histopathological alterations in response to different stimuli. Objectives The aim of this study was to assess the epidemioclinical, histological, and therapeutic features and outcomes of SS. Methods A retrospective study of all patients diagnosed with SS over a 20-year period (1993–2012) was conducted. Data were analyzed using a level of significance of 5%. Results Ninety patients (mean age: 46.5 years) fulfilled the inclusion criteria. The ratio of women to men was 5. Significant associations emerged between dermohypodermic nodes and location on the lower limbs (P = 0.042), and vesiculobullous lesions and location on the legs (P = 0.030), dorsum of the hand (P = 0.015), and forearms (P = 0.003), and paraneoplastic forms (P = 0.012). The upper extremities were involved in the majority of patients (83.3%). Correlations were found between edema of the superficial dermis and vesiculobullous lesions and between leukocytoclastic vasculitis and atypical targetoid lesions. Sweet's syndrome was associated with cytomegalovirus infection (n = 1), inflammatory bowel disease (n = 4), neoplasm (n = 6), and pregnancy (n = 3). First-line treatment consisted of colchicine. Conclusions To the best of the present authors' knowledge, this is the largest series of SS to be reported. Clinical presentations are similar to those described in the literature. Colchicine was efficient and facilitates the reduced use of corticosteroids. The association between SS and neoplasms should be considered in the context of vesiculobullous lesions.

Background Aplasia cutis congenita (ACC) is a rare malformation characterized by absent or scarred areas of skin at birth. Although most commonly found on the scalp, ACC can also involve other locations. Its etiology and pathogenesis remain unclear. Objective To describe the epidemiologic, clinical, therapeutic, and evolutionary aspects of ACC through a hospital series. Methods We conducted a retrospective study from 1995 to 2012 and reported all cases of ACC. Results We enrolled 22 cases (14 girls and eight boys) of ACC during 18 years. The mean age at diagnosis was 5.7 years. Sixteen ACC involved the scalp, five the trunk, and one the left buttock. ACC was oval-shaped in 20 cases, triangular in one case, and linear in one case. The mean size was 4 cm. ACC was associated with bone defects in two cases, various malformations in eight (37.1%), and with syndromic malformation in three (Adams–Olivier syndrome: two cases; Goltz syndrome: one case). Conservative treatment consisting of wound dressing with vaseline was indicated in six cases. Bone reconstruction was performed in two cases. Regular follow-up and no treatment was recommended in 14 cases. Conclusion Our study emphasizes the frequent association of ACC with malformations (37.1%) and bone defects (9%).