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The acute clinical syndrome of toxic epidermal necrolysis (TEN) is currently thought to be a distinct clinical-pathological entity typically resulting from drug hypersensitivity. We describe an adult woman who experienced a fulminate pattern of apoptotic epidermal cell injury following tanning bed exposure while taking naproxen that resulted in a clinical presentation having combined features of drug-induced TEN and an infrequently recognized form of bullous cutaneous lupus erythematosus (LE). This case calls attention to the fact that TEN-like injury can occasionally be seen in settings other than drug hypersensitivity (e.g., LE, acute graft versus host disease) and illustrates the need for a unifying concept in this area. We therefore propose the term 'Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)' to designate a clinical syndrome that is characterized by life-threatening acute and massive cleavage of the epidermis resulting from hyperacute apoptotic injury of the epidermis. We also review vesiculobullous skin disorders that can be encountered in LE patients and suggest a new classification scheme for such lesions.

The short day lengths of late summer are used to program the overwintering adult diapause (dormancy) of the Northern house mosquito, Culex pipiens. Here, we investigated the role of clock genes in initiating this diapause and asked whether the circadian cycling of clock gene expression persists during diapause. We provide evidence that the major circadian clock genes continue to cycle throughout diapause and after diapause has been terminated. RNA interference (RNAi) was used to knock down the core circadian clock genes and to then assess the impact of the various clock genes on the ability of females to enter diapause. RNAi directed against negative circadian regulators (period, timeless and cryptochrome2) caused females that were reared under diapause-inducing, short day conditions to avert diapause. In contrast, knocking down the circadian-associated gene pigment dispersing factor caused females that were reared under diapause-averting, long day conditions to enter a diapause-like state. Our results implicate the circadian clock in the initiation of diapause in C. pipiens.

Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one-third of patients and may precede the development of lung lesions in as many as 10-15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B-lymphocytes in a polymorphous T-cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.

Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. It most commonly occurs in middle-aged Caucasian adults and eventuates in fatality in the majority of patients. A minority of reported patients have a more benign course and a favorable outcome. Degos disease is extremely rare in children. We report Degos disease in a teenager with an extended survival and the absence of internal involvement. We discuss the concept of benign cutaneous Degos and review the 34 patients (30 adults and four children) identified in the world literature with skin biopsy and at least 1 year of follow-up; the average age was 37.6 years; 26 were female and eight were male. As opposed to the malignant form of Degos disease, benign cutaneous Degos is more commonly reported in women (3:1). No laboratory abnormalities have been predictive of a benign versus malignant course. We also discuss the 24 reported instances of Degos disease (malignant and benign) in the pediatric population, of which 14 (58%) were fatal, with death occurring on average 3.6 years after diagnosis.

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The differentiation of primary cutaneous adnexal neoplasms (CANs) from dermal-based metastatic lesions can be difficult. Cytokeratin 5/6 (CK 5/6) has a relatively limited expression profile, being relatively specific for mesothelium and other "pavement" type epithelium such as squamous epithelium. To date, the degree and distribution of CK 5/6 expression in cutaneous neoplasms has not been extensively studied. We speculate that since most of CANs arise from similar epithelium, they should express CK 5/6 and, therefore, CK 5/6 could potentially be helpful in distinguishing these lesions from most of metastatic neoplasms, which usually do not express this marker. Formalin-fixed, paraffin-embedded tissue sections from 228 previously classified CANs and 27 metastatic adenocarcinomas (17 breast, 4 colon, 2 prostate, 2 ovary, 1 lung, and 1 esophagus) were immunostained with anti-CK 5/6. Anti-CK 5/6 labeled 2 of 2 proliferating trichilemmal tumors, 6 of 6 poromas, 4 of 5 hydrocystomas, 10 of 10 cylindromas, 10 of 10 eccrine acrospiromas, 8 of 10 pilomatricoma, 10 of 10 nevus sebaceus, 9 of 9 desmoplastic trichoepitheliomas, 7 of 7 nevus sebaceus with basal cell carcinomas, 10 of 10 pilar cysts, 14 of 14 trichilemmomas, 10 of 10 syringomas, 6 of 7 chondroid syringomas, 10 of 10 hidradenoma papilliferum, 9 of 9 sebaceus adenomas, 3 of 3 microcystic adnexal carcinomas, 10 of 10 eccrine spiradenomas, 4 of 4 syringocystadenoma papilliferum, 3 of 5 ocular sebaceous carcinomas, 28 of 28 basal cell carcinomas, 16 of 16 trichoepitheliomas, and 33 of 33 trichoepitheliomas with basal cell features. By contrast, 9 of 27 metastatic adenocarcinomas stained positively, although only two of these stained strongly. Cumulatively, CK 5/6 was expressed by most (97%) of CANs, while only 33% of metastatic adenocarcinomas showed positive expression. The sensitivity of this marker in the malignant lesions (other than basal cell carcinoma) is 78%, while the specificity is 67%. If all lesions are considered, the sensitivity increases to 97%. Therefore, CK 5/6 may prove to be a useful adjunct marker in distinguishing CANs from metastatic lesions.