Rola Al Dhaybi

Treatment of Periocular Infantile Hemangiomas with Propranolol: Case Series of 18 Children

PURPOSE To study the efficacy of propranolol in the treatment of periocular infantile hemangiomas (IHs). DESIGN Retrospective interventional case series. PARTICIPANTS Eighteen children presenting periocular IH with occlusion of the pupil, anisometropic astigmatism, proliferating eyelid IH, or cosmetically disfiguring periocular IH. METHODS All patients received treatment with propranolol started at 0.5 mg/kg/day with an incremental increase by 0.5 mg/kg/day every 4 days, up to a maximum of 2 to 3 mg/kg/day. Complete eye examinations and serial photographs were obtained before, during, and after treatment. Doppler ultrasound and magnetic resonance imaging performed pre- and post-treatment were compared when available. MAIN OUTCOME MEASURES Evolution of the treated IH was evaluated with respect to astigmatism, amblyopia, and size of the lesion. RESULTS The IH size decreased in 17 of 18 patients. We noted a greater reduction when treatment was administered during the proliferative phase of growth of IHs. At the conclusion of treatment, none of our patients had amblyopia. The mean value of amblyogenic astigmatism (n = 7) decreased from 2.71 diopters (D) pretreatment to 1.03 D post-treatment. On radiology, 8 patients had significant regression of the lesion size of their IH and 1 patient had a limited progression. Propranolol had to be temporarily discontinued in only 1 patient because of symptomatic hypotension. CONCLUSIONS Propranolol seems to be an effective modality of treatment for periocular IH. It seems to be most efficacious when initiated in the proliferative phase of IH but may be beneficial even in the later stage. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Expression of CD133+ cancer stem cells in childhood malignant melanoma and its correlation with metastasis.

Cancer stem cells expressing CD133 exist in a wide array of tumors and their identification in malignant melanoma may help refine classification, diagnosis and treatment. To study the correlation between CD133 expression in childhood melanoma and lymph node and/or visceral metastasis, we evaluated 12 cases of malignant melanoma and 12 control cases of Spitz nevus occurring in children. Double immunostaining with CD133 and Ki-67 was performed in the cases showing CD133 positivity. Three melanoma patients had lymph node metastasis and only one had multivisceral metastases; CD133 was positive only in these four patients. The Ki-67 index was lower in the CD133+ cells in comparison with the CD133− melanoma cells in three cases. We found no positivity for CD133 in all the Spitz nevi. CD133+ cancer stem cell expression in childhood malignant melanoma might correlate with lymph node and/or visceral metastasis and may have a low proliferative Ki-67 index that might explain their chemoresistance.

Targetoid hemosiderotic hemangiomas (hobnail hemangiomas) are vascular lymphatic malformations: A study of 12 pediatric cases

BACKGROUND Targetoid hemosiderotic hemangioma (THH), also called hobnail hemangioma, is a benign vascular lesion and thought to be of lymphatic origin. OBJECTIVE We sought to perform a clinicopathologic analysis of cases diagnosed as THH in a tertiary care childrens hospital. METHODS Clinical and histopathologic data were obtained from a chart review of 12 confirmed pediatric cases of THH. To determine the presence or absence of lymphatic vessels in lesional biopsy specimens, we evaluated the expression of the lymphatic endothelial cell marker podoplanin using the D2-40 antibody. Wilms tumor-1 gene immunostaining and Ki-67 proliferation index were also performed to evaluate the proliferative nature of these lesions. RESULTS Three children had a lesion since birth and 4 had a history of trauma before appearance of the THH. D2-40 immunostaining was positive in every case. Wilms tumor-1 gene immunostaining was negative in 9 cases, focally positive in two cases, and not performed in one case. The Ki-67 proliferation index was very low in all cases studied. LIMITATIONS The small number of cases and restriction to a pediatric population were limitations. CONCLUSION Our findings suggest that THH should be classified as a lymphatic vascular malformation.

High prevalence of angiotropism in congenital melanocytic nevi: an analysis of 53 cases.

Abstract:The mechanisms responsible for the development of congenital melanocytic nevi (CMN) have yet to be elucidated. A potential clue to their origin is the observation of angiotropism of nevus cells in CMN. Interestingly, neural crest stem cells (NCSCs), the precursors of melanocytes, demonstrate angiotropism in the embryo. There is accumulating evidence that NCSCs migrate along the external surfaces of vessels during a portion of their journey to the skin. Comparable angiotropism and migration of melanoma cells have been described as extravascular migratory metastasis in melanoma. In this report, we systematically examined for the first time, the frequency of angiotropism in 53 CMN. The lesions originated from 27 females and 26 males with an average age of 9.81 years (range 0.42–28 years). The mean nevus size was 7.43 cm (range 0.3–40 cm). Twenty-seven (50.9%) of the 53 lesions were less than 1.5 cm in diameter. Sixteen nevi (30.2%) were medium sized (1.5–19.9 cm), and 10 CMN (18.9%) were large/giant (>20 cm in diameter). The trunk was the most common location (23/53) followed by the head and neck (17/53). Thirty-eight (71.7%) of the 53 lesions were compound melanocytic nevi, and 15 (28.3%) of the 53 lesions were dermal nevi. In summary, angiotropism was observed in 50 (94.3%) of 53 cases. Consequently, such angiotropism may potentially explain the origin of the precursor cells giving rise to CMN. Further explanations concerning dysregulated growth are clearly needed for the actual appearance of CMN and their physical characteristics.

Unusual association between autoimmune hepatitis and severe pyoderma gangrenosum.

JPGN Volume 50, N nosum (PG), classifi ulcerative cutaneous P yoderma gangre ed as a neutrophilic dermatosis, is an condition of unknown origin (1). It predominantly occurs between the second and fifth decades of life. Pyoderma gangrenosum affects both sexes, with a female predominance. The incidence is around 3 to 10 per million per year (2). The pathogenesis of the disease is still unclear, but a neutrophilic dysfunction was suggested. Some defects in chemotaxis or hyperreactivity in neutrophil trafficking and some aberrant integrin oscillations on neutrophils seem to be implicated (1,3). Moreover, a multifactorial origin is likely with a genetic predisposition, environmental, paraneoplastic, or paraimmune factors, and possible infectious triggers (1). The diagnosis of PG is proposed in the presence of a necrolytic cutaneous ulcer with an irregular undermined border, painful and progressing rapidly, and by excluding other causes of cutaneous ulcerations (1). Pyoderma gangrenosum is associated with an underlying disease in 50% to 70% of cases (1,3). All patients with PG should undergo extensive evaluation to rule out associated disorders before beginning therapy that may include corticosteroids, antimicrobial agents, steroid-sparing immunosuppressive agents, and immunomodulating drugs (1,3). In adults, PG is associated with inflammatory bowel diseases (IBDs) in 10% to 15% of cases (4), seronegative rheumatoid arthritis (5), and lymphoproliferative disorders (2,6,7). Exceptionally, cases of PG associated with chronic viral (8) and autoimmune hepatitis (AIH) (9,10) have been reported in the adult population. We describe the case of a young girl with a history of type 2 AIH who developed PG at the age of 14 years. Her PG was refractory to classical immunosupressive therapy, necessitating the introduction of anti-tumor necrosis factor (TNF)-a drugs.

Childhood linear IgA bullous disease induced by trimethoprim-sulfamethoxazole.

BACKGROUND Linear IgA bullous disease (LABD) is a rare mucocutaneous autoimmune subepidermal blistering disease that can affect children mostly of pre-school age. As many as two-thirds of LABD are related to drug ingestion, particularly certain antibiotics, non-steroidal anti-inflammatory drugs and diuretics. MAIN OBSERVATION We describe a 3-year-old boy who presented a CMV infection followed by LABD induced by trimtheporim-sulfametoxazole. To our knowledge, this is the first reported case of trimethoprim-sulfamethoxazole that was confirmed by a rechallenge. CONCLUSIONS Most cases of drug-induced LABD are patients being treated with multiple systemic drugs that could induce the LABD. In the lack of suitable alternative treatment, the identification of the causative drug can be achieved by a rechallenge under close medical surveillance.