Toxic Epidermal Necrolysis in a Patient with Autoimmune Limbic Encephalitis with Anti-Glutamate Receptor Antibodies

We report on a 44-year-old woman who was diagnosed with toxic epidermal necrolysis (TEN) during the recovery phase from autoimmune limbic encephalitis with anti-glutamate receptor antibodies. Both, autoimmune limbic encephalitis and TEN are very rare diseases. The co-existence of the two diseases has not yet been reported. We speculate that the total of 18 drugs needed for the treatment of encephalitis might have increased the risk of TEN. Similar reports would be required to elucidate the pathophysiology of the co-existence

A Case of Carcinoma of the Male Breast Mimicking a Mucinous Carcinoma of the Skin

The authors report a case of mucinous carcinoma of the male breast firstly diagnosed as a mucinous carcinoma of the skin. The immunohistochemical results of this tumor were as follows: cytokeratin7 (-), gross cystic disease fluid protein 15 (-), p63 (-), estrogen receptor (+), and progesterone receptor (+) for the primary nodule; cytokeratin7 (-), thyroid transcription factor-1 (-), gross cystic disease fluid protein 15 (-), p63 (-), cytokeratin8 (+), cytokeratin18 (+), and cytokeratin20 (+) for the recurrent nodule. The tumor cells had cytokeratin7 (-)/ cytokeratin20 (+) phenotype and it was very unusual for mucinous carcinoma of the breast

A case of carcinoma of the male breast mimicking a mucinous carcinoma of the skin

The authors report a case of mucinous carcinoma of the male breast firstly diagnosed as a mucinous carcinoma of the skin. The immunohistochemical results of this tumor were as follows: cytokeratin7 (-), gross cystic disease fluid protein 15 (-), p63 (-), estrogen receptor (+), and progesterone receptor (+) for the primary nodule; cytokeratin7 (-), thyroid transcription factor-1 (-), gross cystic disease fluid protein 15 (-), p63 (-), cytokeratin8 (+), cytokeratin18 (+), and cytokeratin20 (+) for the recurrent nodule. The tumor cells had cytokeratin7 (-)/ cytokeratin20 (+) phenotype and it was very unusual for mucinous carcinoma of the breast

Expression of MK6a dominant-negative and C-terminal mutant transgenes has distinct phenotypic consequences in the epidermis and hair-follicle

Mouse keratin 6a (MK6a) is constitutively expressed in a single cell layer of the outer root sheath (ORS) of hair follicles, but its synthesis can be induced in interfollicular epidermis including the basal cell layer in response to perturbing stimuli. A basally inducible human K6 (HK6) isoform has not been described, and it is not clear which of the known HK6 isoforms is expressed in the ORS. In this study we show that expression of a dominant-negative MK6a construct (Δ2B-P) in the interfollicular epidermis caused severe blistering and neonatal lethality, suggesting that mutations in a yet to be identified basally expressed HK6 isoform might result in a severe blistering phenotype. Surviving Δ2B-P animals showed transgene expression only in isolated epidermal cells and not in all cells of the ORS, but nevertheless developed severe alopecia. Expression of two different C-terminal mutant transgenes also caused alopecia while a third C-terminal mutant had no phenotypic conse- quences. Electron microscopy revealed that Δ2B-P expression resulted in the collapse of keratin filaments, while destruction of hair follicles in the two phenotypic C-terminal mutant lines occurred in the absence of filament abnormalities. The latter finding indicates that the innermost ORS cells are uniquely sensitive to expression of even slightly altered K6 proteins, suggesting that mutations affecting an HK6 isoform expressed in this cell layer could result in alopecia in humans as well