Stevens-Johnson syndrome and toxic epidermal necrolysis: a retrospective descriptive study.

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 anti-inflammatory 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.

Mohs micrographic surgery: 10 year experience in Puerto Rico.

OBJECTIVE: The incidence of nonmelanoma skin cancer (NMSC) is increasing rapidly worldwide. As NMSC incidence increases, the modalities to treat this condition have become diverse. However, Mohs surgery remains the standard treatment for skin cancer in several particular locations such as the face. The objective of this study is to compare the changes, occurring over a 10-year period, in the characteristics of those cancers that were treated with Mohs micrographic surgery (MMS) at the dermatology clinics of the University of Puerto Rico as well as the modifications in the repair patterns used to close the surgical defects. METHODS: A retrospective chart review of patients treated with MMS at the dermatology of the University of Puerto Rico in the years 2000 and 2010. Variables analyzed include patient demographics, the anatomic site of each patient's lesion, pathology, the preoperative tumor size, the postoperative defect size, and the repair method. RESULTS: Thirty-eight (38) patients in the year 2000 and 55 patients in the year 2010 were treated with MMS, signifying a 44% increase in this kind of treatment over a 10-year period. The 2000 cohort was found to be slightly older (P = 0.22), with no gender predominance (P = 0.44). In both years, the majority of tumors were located on the head and neck region, being the nose the most frequent site of involvement (P = 0.06). Basal cell carcinoma (BCC) was the most common neoplasm (P = 0.65). No statistical difference was found in preoperative tumor sizes (P = 0.27). More stages were required to remove a given tumor completely in the year 2000 (P = 0.025). Postoperative defects were smaller in 2000 (P = 0.027) than they were in 2010. Flap repair was used more often in 2010 (P = 0.001) than in 2000. CONCLUSION: This study shows a trend toward larger defects in a slightly younger population of patients in the 2010 cohort compared to the 2000 cohort. It also demonstrates a reduction in the number of stages required to excise the tumors, and a tendency to reconstruct the surgical defects with flaps. However, the tumor types, preoperative tumor sizes, and anatomic sites of the lesions were all similar in the 2 cohorts.

Clinical and dermoscopic patterns of melanocytic nevi in Hispanic adolescents: a descriptive study.

BACKGROUND: Melanocytic nevi are well-known, important precursors of melanoma among children and adults. The adolescence period is an important period for nevi formation and evolution. This study provides data of a longitudinal study of nevi in a Hispanic adolescent population. MATERIALS AND METHODS: A cross-sectional survey and 1-year prospective follow-up study was performed on Hispanic students from grades 6 and 7 at a school in Caguas, Puerto Rico (n = 90). The survey was completed by the students and one of their parents. The backs of the children were clinically examined for melanocytic nevi using digital photography and dermoscopy. Follow-up was conducted one year later. RESULTS: The study cohort consisted of 53 (59%) boys and 37 (41%) girls, with an average age of 11.9 years (range 11-13 years). At the beginning of the study, 85% (n = 71/90) of the students presented with melanocytic nevi on their backs. After one year, new nevi were identified in 62% (n = 44/71), and there was a mean increase in nevus count of 1.8 (P < 0.001). A trend toward increased nevus count in lighter skin types was observed (P < 0.001). The predominant dermoscopic pattern was reticular (44%). The globular pattern was found most commonly in children with skin-type II (100%), while the reticular pattern was the most common among skin-types III (32%), IV (56%), and V (45%). CONCLUSIONS: This study supports the utility of digital photography and dermoscopy for the evaluation of melanocytic nevi, providing evidence of the interrelationship between nevus count, dermoscopic pattern, and skin phenotype.

Skin cancer in Puerto Rico: a multiannual incidence comparative study.

BACKGROUND: The incidence of skin cancer continues to increase worldwide. The purpose of this study was to determine the incidence of skin cancer in Puerto Rico in a selected year (2005) and to compare these findings with those previously reported for Puerto Rico in 1974 and 1981 and with other countries. METHODS: The data was collected from the pathology reports corresponding to the period of January to December 2005 of 21 participating Pathology Laboratories throughout Puerto Rico. The rate and distribution of the main types of skin cancer was calculated based on sex, age, anatomic location and laterality. RESULTS: The incidence of skin cancer in Puerto Rico for 2005 was 6,568 cases, which represent a rate of 167.9 per 100,000 inhabitants. The most common type of skin cancer was basal-cell carcinoma. Skin cancer was more common in males except for melanoma, which was more common in females. The incidence increases with age on all types of skin cancer. The head and neck area was the most frequent location, except for melanoma in women, which was more common on the legs. The incidence rate was 41.5/100,000 in 1974, 52.5/100,000 in 1981 and 167.9/100,000 in 2005, a 305% increase. CONCLUSIONS: We found an increasing incidence of skin cancer in Puerto Rico when compared with previous reported data. This analysis provides a comprehensive evaluation of the epidemiology of skin cancer in Puerto Rico.

MSH-2 and MLH-1 protein expression in Muir Torre syndrome-related and sporadic sebaceous neoplasms.

BACKGROUND: Muir-Torre Syndrome (MTS) is a rare autosomal-dominant disorder characterized by the predisposition to both sebaceous neoplasm and internal malignancies. MTS-associated sebaceous neoplasms reveal mutations in DNA mismatch repair (MMR) genes and microsatellite instability. A significant part of MTS patients represents a phenotypic variant, the hereditary nonpolyposis colorectal cancer (HNPCC). A strong correlation between microsatellite instability and immunostaining has been demonstrated. The early recognition of sebaceous neoplasm as part of MTS, and their differentiation from sporadic sebaceous neoplasm may have an important application in a clinical setting. The absence of MLH-1 or MSH-2 expression by immunostaining identifies tumors with mismatch repair deficiency. OBJECTIVES: Our aim is to determine whether an immunohistochemical approach, targeting DNA repair proteins MSH-2 and MLH-1 in MTS-related sebaceous neoplasm and their sporadic counterparts, can be used for their identification. METHODS: We examined 15 sebaceous neoplasms (including 6 internal malignancy- associated sebaceous neoplasms and 8 sporadic sebaceous neoplasms) from 11 patients for the expression of MSH-2 and MLH-1 by immunohistochemistry. RESULTS: Four of 5 internal malignancy-associated sebaceous neoplasms showed loss of expression of MSH-2 or MLH-1. Correlation of the immunostaining pattern of the sebaceous neoplasms and the patients' positive history of colon carcinoma was 80%. Seven of 8 sporadic sebaceous neoplasms showed a positive expression of MSH-2 and MLH-1. The prevalence for loss of expression of MMR proteins in sebaceous neoplasms was 38.5%. MMR immunostaining had 87.5% specificity and 80% sensitivity. LIMITATIONS: This study is limited by a small sample size, and by bias selection due to the use of non nationwide data-base as the resource of cases. CONCLUSIONS: Our findings demonstrate that immunohistochemical testing for internal malignancy-associated sebaceous neoplasms is a practical approach to confirm a suspected inherited MMR gene defect, and an accurate method to distinguish between sporadic and MTS-associated sebaceous lesions.

Prevalence of contact allergens in a Hispanic population.

BACKGROUND: Patch testing has been used as a screening tool to identify those allergens responsible for the development of allergic contact dermatitis. The objectives of this study were to identify the most common allergens found at the Patch Testing Clinic (PTC) of the Department of Dermatology, Medical Sciences Campus (MSC) University of Puerto Rico (UPR), to correlate patch testing results with demographics and clinical data, and to compare the results with similar studies in other institutions. METHODS: A retrospective analysis of the records of patients who underwent patch testing for suspected acute allergic contact dermatitis at the Department of Dermatology of the Medical Sciences Campus of the UPR, from March 31, 2001 to May 31, 2005. Information regarding demographics, medical history, occupation, and test results was retrieved. RESULTS: The most common allergens identified were: carba mix, nickel sulphate, thiuram mix, paratertiary butylphenol formaldehyde resin, paraphenylenediamine, and neomycin sulphate. The most commonly affected area was the hands. A correlation was observed between paratertiary butylphenol and involvement of the feet, paraphenylenediamine and generalized dermatitis, and potassium dichromate and occupational exposure. CONCLUSIONS: Patch testing remains a worthwhile and quick diagnostic tool for the evaluation of patients with suspected allergic contact dermatitis.

Epidemiology of melanoma in Puerto Rico, 1987-2002.

BACKGROUND: The incidence of cutaneous malignant melanoma continues an increasing trend worldwide and has had the highest rise in incidence of all malignancies during the last decades. Mortality rates for melanoma have remained relatively stable in most countries despite the increase in incidence rates. The purpose of this study is to describe the epidemiological trends of malignant melanoma in Puerto Rico from 1987 to 2002 and to compare these findings with those previously reported for Puerto Rico as well as with those reported for other countries. METHODS: All cases of malignant melanoma reported to the Puerto Rico Cancer Registry from 1987 to 2002 were included. Age-adjusted incidence and mortality rates were calculated. Trends were compared by age, gender, anatomical location of the tumor and tumor thickness. RESULTS: There were 1,568 new melanoma cases reported between 1987 and 2002 in Puerto Rico, comprising a 50% overall increase in incidence during this period. Male to female incidence ratio was 1:0.86. The most common anatomical location in women was the lower extremity followed by the head and neck region. In men, the most common anatomical location was the trunk, followed by the head and neck region. Most of the superficial tumors (< 1mm) were located on the trunk followed by the lower limb. The most common specific histologic diagnosis reported was melanoma in situ. In 40.8% of the cases the tumor thickness value was not provided. The number of thinner melanomas (< 1mm) reported increased during the study period. Mortality rates were slightly higher for men than for women, still mortality rates remained stable for both genders and all age groups analyzed. CONCLUSIONS: Melanoma incidence rates continue to increase in Puerto Rico. This, in combination with an increase in the diagnosis of thin melanomas, suggests that the stable mortality rates may be due, in part, to earlier diagnosis and improved overall prognosis.