CD8+ Pagetoid Reticulosis Presenting as a Solitary Foot Plaque in a Young Woman.

Background: Pagetoid reticulosis is a rare variant of mycosis fungoides. This rare condition typically presents as a solitary plaque located on the extremities with an indolent clinical course (Woringer-Kolopp disease) or as a more generalized presentation with diffuse cutaneous involvement and a more aggressive clinical course (Ketron-Goodman disease). Purpose: To review the cutaneous manifestations, pathology, and treatment of localized pagetoid reticulosis. Methods: The authors describe a 24-year-old woman with a slowly enlarging, localized plaque of seven months duration, representing the localized form of pagetoid reticulosis with CD8+ immunophenotype. Results: The histological, immunohistochemical, and clinical features of the patient's skin lesion were characteristic for a diagnosis of Woringer-Kolopp disease. Systemic work-up for lymphoma was negative. Conclusion: Woringer-Kolopp disease is most commonly seen in middle-aged men as a solitary lesion of the extremities, and it should always be considered in the differential diagnosis when a patient presents with such a lesion. A histological analysis demonstrated atypical lymphocytes preferentially localized to the epidermis with a CD4+, CD8+, or CD4-/CD8- phenotype. The treatment of choice for a solitary lesion may be localized radiation therapy, but newer therapies, such as bexarotene, may warrant further investigation.

Prognosis of amyotrophic lateral sclerosis

Two cohorts of amyotrophic lateral sclerosis (ALS) patients were identified. One incidence-based cohort from Harris County, Texas with 97 cases, and the other a clinic referral series from an ALS clinic in Houston, Texas with 439 cases were followed-up to evaluate the prognosis of ALS. The overall Kaplan-Meier 3-year survival after diagnosis was similar, 0.287 for the incidence cohort and 0.313 for the referral cohort. However, the 5-year survival was much lower for the incidence cohort than the referral cohort (0.037 vs. 0.206). The large difference in 5-year survival was thought to be the results of a stronger unfavorable effect of the prognostic factors in the incidence cohort than in the referral cohort.^ Cohort-specific Weibull regression models were derived to evaluate the cohort-specific prognostic factors and survival probability with adjustment of certain prognostic factors.^ The major prognostic factors were: age at diagnosis, bulbar onset, black ethnicity, and positive family history of ALS in both cohorts. Female gender, simultaneous upper and lower extremities onset were specifically unfavorable factors in the incidence cohort. In the incidence cohort the prognosis was relatively favorable for cases with duration from onset to diagnosis longer than 4 months, however in the referral cohort the relatively favorable prognosis only occurred in cases with duration from onset to diagnosis 1 year or longer and was strongest in cases with duration 5 years and longer. Age at diagnosis modified the effect of bulbar onset in the incidence cohort but not in the referral cohort. The estimated survival with presence of an unfavorable prognostic factor identified in the incidence cohort was higher for the referral cohort than for the incidence cohort. Future studies are indicated to investigate the disease heterogeneity issue of ALS based on survival distribution of ALS. ^

AMERICAN TRYPANOSOMIASIS (CHAGAS') DISEASE - A STUDY OF FETO-MATERNAL MORBIDITY AND MORTALITY

The possibility of a relationship between American Trypanosomiasis (Chagas') disease and pregnancy outcome was analyzed measuring feto-maternal morbidity and mortality in a sample of 604 pregnant women and their offspring seen at the Hospital Universitario de Maternidad y Neonatologia in Cordoba, Argentina during 1979.^ A cross-sectional, "case-comparison" investigation was employed to determine the degree of risk between having a reactive chagasic serologic test and a negative pregnancy outcome as determined by abortion, stillbirth, and infant death prior to one week of age. Patients were selected using a dichotomous, 0-1 scale with either the presence or the absence of a reactive Machado-Guerreiro complement fixation serologic blood test result.^ The data obtained were analyzed using appropriate statistical techniques for measuring the comparisons between the case and control groups under various demographic and socioeconomic variables such as, age, marital status, educational attainment, and residence. Similarly, additional biological variables of birth order, maternal and fetal complications, and prematurity were examined.^ From the analysis of the data obtained in this investigation, no definite conclusions can be reached regarding the risk of having an unsuccessful pregnancy outcome in the presence of a reactive serologic finding because the study design was a cross-sectional one and the number of events were too few for an adequate analysis. Notwithstanding these limitations, the results obtained, after statistical adjustments were employed, demonstrated that women with a reactive test result were older, were of a higher parity, and were less educated. Marital status and residence were not significant variables. The risk of pregnancy wastage, however, was almost twice as frequent in the reactive group as in the non-reactive group of women. Statistically significant differences in maternal morbidity involved two complications, polyhydramnios and varicosities of the lower extremities and vulva; while in the newborn, infection was higher in infants whose mothers exhibited a reactive serologic test result.^ In summary, what this research study has shown is the need for engaging in a larger, longitudinal study for an in-depth exploration of feto-maternal morbidity and mortality--an investigation that would corraborate or refute the findings of this study.^