An adenomatoid tumour was found in the anterior maxillary region of a 15 year old female patient. Two impacted teeth were found in the tumour. The lateral incisor found in the tumour was dilacerated, and the roots of the first premolar were resorbed. A review of the English literature indicated that 294 similar cases have been reported.
Recessive dystrophic epidermolysis bullosa (RDEB) is an uncommon and severely disabling genetic disorder characterized by trauma-induced blisters, intractable skin ulcers, scarring, milia, and nail dystrophy. Patients with RDEB have an increased tendency for fast-growing and early metastasizing squamous cell carcinoma (SCC). We report here a 13-year-old girl with RDEB who developed a large SCC on the left knee. At 6 months of evolution it was resected and covered with an autologous skin graft. To our knowledge, this is the youngest patient with RDEB complicated by SCC to be reported, and therefore may serve to emphasize the importance of vigilance in surveying RDEB patients for SCC.
We describe a 16-year old female with lupus erythematosus panniculitis with unusual manifestations. She had noted to have developed erythematous nodules and plaques in the right axilla and inguinal region at the age of one year. These lesions resolved gradually with scar formation. However, new lesions were noted at the same locations in the following years. Some of her lesions at the scalp and the left axillary regions developing within the last two years slowly enlarged showing an annular configuration and subsequently resulted in hair loss. The erythematous border of her lesion in the left axilla consisted of two parallel red lines. Histopathological and direct immunofluorescent findings were consistent with lupus erythematosus panniculitis. Similar clinical findings in the same locations were also observed in the mother.
This study was performed to examine inducible nitric oxide synthase (NOS-2) expression, nitrotyrosine formation and apoptosis in rats with elevated intraocular pressure (IOP) and/or ocular inflammation. Ocular inflammation was induced via injection of intra-vitreal lipopolysaccharide (LPS) while IOP was elevated by episcleral vessel cauterization. Animals were randomized to one of the following conditions: elevated IOP, LPS, elevated IOP+LPS, and control. Immunohistochemical staining and western blot analysis of retinal lysates revealed NOS-2 and nitrotyrosine immunoreactivity in all disease groups. NOS-2 expression and protein nitration was significantly greater in rats with elevated IOP+LPS compared to elevated IOP, LPS, and control groups. Nitrite levels in the retina affirmed significantly increased levels of nitric oxide generation in LPS-treated rats with elevated IOP (346+/-23.8 microM) vs LPS-treated, elevated IOP and control groups (195.6+/-12.6, 130+/-2.5 and 76.6+/-15.6 microM, respectively). Retinal TUNEL staining showed apoptosis in all diseased groups. Percent of apoptotic cells was significantly greater in the elevated IOP+LPS group compared to LPS-treated or elevated IOP groups. Presented data illustrates that both elevated IOP and ocular inflammation augment NOS-2 expression, retinal protein nitration and apoptosis in rats.
Papuloerythroderma of Ofuji (PEO) is a disease of elderly men, characterized by intensely pruritic and widespread, red, flat-topped papules with sparing of the body folds and creases (the so-called 'deck-chair' sign). The etiopathogenesis of the disease remains unknown. Psoralen plus UVA (PUVA), topical and systemic corticosteroids, etretinate, cyclosporin and interferon are the main approaches in the treatment of this rare disease. A case of PEO in a 60-year-old man who responded to retinoid plus PUVA (Re-PUVA) treatment is reported here and a review of the therapy with other relevant cases is presented.
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