Congenital hemangioma.

Congenital hemangiomas (CHs) are fully developed hemangiomas that are present at birth. There are 2 possible types: rapidly involuting CHs (RICHs) and noninvoluting CHs (NICHs). We conducted a retrospective study (2008-2012) of 6 patients (2 females, 4 males) with CHs (mean age, 16 days). We analyzed the epidemiology, clinical characteristics, and clinical outcome of CHs over this 5-year period.

Aplasia cutis congenita: report of 22 cases.

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%).

Clinical and laboratory findings in 8 patients with Bloom's syndrome.

UNLABELLED: Bloom's syndrome is a rare autosomal recessive disorder caused by germline mutation of the BLM gene. The objective of this study was to illustrate the clinical, biological and genetic characteristics of this syndrome through Tunisian series. We report in a retrospective study 8 case of bloom's syndrome observed during 20 years. RESULTS: Our patients were 4 males and 4 females issued from 5 families. For all patients, the parents were consanguineous. The age was 13 to 39 years. The telangiectatic erythema was developed in all the patients between 6 months and 2 years old on the cheeks, on the nose, on the lips and the lower eyebrows. The photosensitivity was constant and was complicated by vesicules and bullae for 5 patients who had extensive lesions, three patients noted accentuation of their telangiectasic erythema. An improvement with the age was noticed for the first four patients. The growth deficiency was observed for all patients. It was marked, between -2 and -4 DS (standard deviation). The number of sister chromatid exchange was increased to twelve fold comparatively to normal subjects. Two patients developed a breast cancer; the evolution was fatal in one. Another patient developed a leukaemia, the evolution was also fatal. CONCLUSION: Bloom's syndrome is a rare genodermatitis. All the patients presented three symptoms: telangiectatic erythema, growth delay and photosensitivity associated with immunodeficiency. There is significant risk of cancer, so that follow up of patients is mandatory.

[Sweet syndrome: retrospective study of 54 cases]. / Syndrome de Sweet: étude rétrospective de 54 cas.

INTRODUCTION: Sweet syndrome is the most common neutrophilic dermatosis. We studied its natural history and epidemiologic, clinical, and therapeutic characteristics from a series of 54 cases. MATERIALS AND METHODS: This retrospective study examines 54 cases collected over a 10-year-period. Diagnosis was based on clinical and histological criteria. RESULTS: Patients' mean age was 47 years. The sex ratio was 8 women for every man. Disease developed most often in autumn and onset was sudden in all patients. Untypical aspects were found in 20 patients. Lesions were located mainly on the arms (43 cases) and legs (33 cases). No mucous membrane involvement was observed. Histological testing found leukocytoclastic vasculitis in 8 cases. Sweet syndrome was idiopathic for 38 patients. Lesions began at the site of previous trauma for 9 patients, occurred during pregnancy for 2, and were associated with cancer for 3. First-line treatment was colchicine for 23 patients and oral steroids for 12. Six patients had recurrences and 2 patients had relapses. COMMENTARY: This large series of patients with Sweet syndrome differs from other reports by the frequency of untypical aspects and post-traumatic forms, as well as the rarity of association with cancer. Histological findings of vasculitis do not rule out a diagnosis of Sweet syndrome.