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Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially life-threatening mucocutaneous reactions. Given their rarity, limited cohort studies have been done. The aim of this study is to evaluate and compare the demographics, etiology, management, clinical and laboratory characteristics, complications, and outcome of SJS/TEN patients seen by the inpatient dermatology service at the University of Puerto Rico. Methods A retrospective review of 30 cases with identified diagnosis of SJS, overlap SJS/ TEN, or TEN who were consulted to the Dermatology Department of the University of Puerto Rico from 2006 to 2017.Results A total of 24 adult and six pediatric cases were reviewed. Females were predominant with a female to male ratio of 1.3 : 1. The most frequent offending drugs identified were antibiotics (56.7%), anticonvulsants (23.3%), and nonsteroidal antiinflammatory drugs (NSAIDs) (16.7%) with the most frequent antibiotic identified being trimethoprim/sulfamethoxazole (23.3%). Seventy percent of patients experienced at least one complication, most often of infectious etiology (80.1%). During hospital course, 73% received pharmacologic therapy (23% received IVIG alone, 17% received steroids alone, and 33% both) versus 27% which received only supportive care. Mortality rate in this study was 13.8%. When comparing SCORTEN at day one of admission, deceased cases had a mean SCORTEN at day 1 of 4.0, while survivors had an average of 1.54 (P < 0.001).Conclusion Antibiotics followed by anticonvulsants were the most frequently offending drugs identified within this study.

Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by cutaneous and extracutaneous hamartomas. Dermatologic evaluation is critical for early diagnosis because mucocutaneous manifestations account for 4 of 11 major and 3 of 6 minor diagnostic criteria. Folliculocystic and collagen hamartoma (FCCH) is a recently described entity associated with TSC. We herein describe the case of a 28-year-old woman with a history of TSC who presented with a scalp lesion present since childhood. Physical examination revealed a solitary, well-circumscribed exophytic tumor over the occipital scalp measuring 9 • 8 cm and covered with comedones and cyst-like structures. Biopsy of the lesion demonstrated thickening of the collagen bundles throughout the dermis, concentric perifollicular and perivascular fibrosis, an increased number of dilated vessels, and keratin-filled cysts lined by the infundibular epithelium. Clinicopathologic correlation was diagnostic for FCCH. The patient was referred for surgical excision. In addition, we review 11 other cases of FCCH previously reported in the literature.

Background The anecdotic evidence of the benefits from biologic agents for psoriasis is extensive. However, data on the efficacy of biologic agents for pustular psoriasis are limited.Methods To update the data on the efficacy and safety of biologic agents for the management of pustular psoriasis. A systematic review of published data regarding biologic therapies on PubMED database, used in the management of pustular psoriasis from 2012 was undertaken.Results A total of 209 articles were identified, and 43 articles were selected for inclusion. TNF-a inhibitors were used in 205 patients, and 86 patients received ustekinumab, secukinumab, brodalumab, ixekizumab and IL-1 inhibitors. Overall response was favorable for most modalities. No serious adverse events were reported. Inconsistent measures of treatment response and study variability limited the overall evaluation of data. Conclusions Infliximab and ustekinumab have the most evidence of efficacy and safety for the treatment of pustular psoriasis. Recent evidence supports the use of IL-17 antagonists. Prospective controlled and comparative trials are needed to further explore the efficacy and safety of biologic agents in order to establish objective recommendations for the management of this challenging condition. mia, hypocalcemia, elevated erythrocyte sedimentation rate, C-reactive protein, antistreptolysin O antibody levels, and/or IgG or IgA. 7Although most cases of pustular psoriasis are believed to be idiopathic, there are several factors that can trigger disease such as pregnancy, infections, contact to allergens, drugs, and withdrawal from medications used for psoriasis, including steroids, methotrexate, and cyclosporine. 9-11 Paradoxically, the use of tumor necrosis factor (TNF)-a inhibitors may also precipitate pustular psoriasis. 12,13 Pustular psoriasis is a challenging condition to treat, and evidence regarding management strategies is limited. The National Psoriasis Foundation's (NPF) most recent guidelines of care for pustular psoriasis were published in 2012. 14 Recognizing that the "overall quality of the literature about the treatment of pustular psoriasis is weak," the group recommended acitretin, cyclosporine, methotrexate, and infliximab as first-line treatment options for adult GPP. In 2014, Levin et al. 15 suggested an algorithm for biologic treatments of GPP, recommending infliximab as first-line therapy for patients with acute, severe GPP.These recommendations are not universally accepted and have not been updated to include recently introduced biologic agents such as IL-17 inhibitors. [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33]

Basal cell carcinoma (BCC) is the most common malignancy of the skin. It is an epithelial neoplasm with origin in the precursor cells of the interfollicular epidermis. Even though it has low metastatic potential, delay in management may lead to local destruction and morbidity. In contrast, trichoepithelioma (TE) is a benign tumor originating from the outer root sheath of the hair follicle. Similar to BCC, TE tends to affect the head and neck region. Both neoplasms may exhibit clinical and histopathological similarities, making them prone to misdiagnosis. Multiple immunomarkers have been used to distinguish among these entities, but so far, no single agent or combination of agents appear to be neither sensitive nor specific enough to differentiate between them. This study was divided into 2 parts. First, 17 cases of BCC and 14 cases of TE were stained with androgen receptor and bcl-2. Then, 27 cases of borderline/equivocal of BCC and 13 cases of borderline/equivocal TE were stained with the same protocol. Sensitivity and specificity were calculated for each individual immunomarker and for the combination of them. Androgen receptor positivity was 100% specific for BCC and borderline/equivocal BCC, whereas bcl-2 diffuse staining pattern demonstrated a sensitivity of 82.4% for BCC and 88.9% borderline/equivocal BCC. When both immunomarkers were combined, the sensitivity for BCC decreased (70.6%) but the specificity remained high (100%). Similarly, the sensitivity for borderline/equivocal BCC was 55.6%, whereas the specificity was 100%. Although moderately sensitive, combining both immunomarkers showed an excellent specificity to discriminate between BCC and TE.