Leprosy in Puerto Rico: insight into the new millennia.

BACKGROUND: Leprosy, or Hansen's disease, is a chronic infectious disease caused by the bacillus Mycobacterium leprae. In 2000, the World Health Organization (WHO) defined the elimination of the disease as a global prevalence of less than one case per 10,000 population. However, disease transmission is an ongoing worldwide public health concern, as evidenced by the more than 220,000 new cases diagnosed each year. METHODS: This study is an update of the incidence and prevalence of leprosy in Puerto Rico for the period of 2000-2014. A retrospective analysis of data was obtained from the Tropical Disease Clinic (TDC) of the University of Puerto Rico School of Medicine. RESULTS: Sixty-three new cases of leprosy are detailed in this study. Disease incidence and prevalence were 1.65 and 5.26 per 100,000 inhabitants (of the island of Puerto Rico), respectively, and an average of 4.2 new cases per year. Most of the male patients in the study suffered from lepromatous leprosy (P = 0.026). In all, 47 (74.6%) patients had been born in Puerto Rico, and 29 (46%) had an affected family member or were in close contact with someone with leprosy. CONCLUSIONS: Compared to those of previous studies, these results demonstrate a decrease in both the incidence and prevalence of leprosy in Puerto Rico over the past 15 years. The relatively high prevalence of leprosy in Puerto Rico means that it remains endemic on the island. Concerted efforts must be undertaken to achieve the goal of the elimination of this old and stigmatized disease.

Extraocular Sebaceous Carcinoma-A Clinicopathologic Reassessment.

Sebaceous carcinoma is an aggressive adnexal neoplasm with sebaceous differentiation. Few reports have described the histopathologic characteristics of the sebaceous carcinoma occurring extraocularly. Seventy-two cases of extraocular sebaceous carcinoma were identified from the database of a Dermatopathology Laboratory from January 1, 2007 to May 31, 2013. More cases occurred in men (60%), with a mean age at diagnosis of 65.8 years (range 39-99 years). Neoplasms were histopathologically classified as well-differentiated (22%), moderately differentiated (67%), and poorly differentiated (11%). Sixty-seven percent (67%) of cases demonstrated a squamoid growth pattern and thirty-three percent (33%) demonstrated a basaloid growth pattern. A majority of the neoplasms histopathologically classified as well-differentiated (94%) and moderately differentiated (65%) demonstrated a squamoid growth pattern. Ten percent (10%) of cases exhibited cystic histopathologic changes. The histopathological features reported in this study aid in the understanding of extraocular sebaceous carcinoma and its eventual diagnosis and classification.

Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis.

Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients.

Warfarin-induced skin necrosis following heparin-induced thrombocytopenia.

Anticoagulants, such as heparin and warfarin, are commonly used in the treatment and prevention of thromboembolic events. The risk of developing warfarin-induced skin necrosis (WISN) with warfarin is reported to be <1%. However, the risk of WISN may be increased with the initiation of warfarin in the setting of heparin-induced thrombocytopenia and thrombosis syndrome (HITT). WISN can lead to catastrophic tissue necrosis requiring amputations and mass debridement. This report describes a case of WISN following HITT and discusses the appropriate medical management of patients with HITT to avoid secondary WISN.

Ocular manifestations of acute graft versus host disease after allogeneic bone marrow transplant.

A 17-year-old-male with Sickle Cell Disease underwent allogenic bone marrow transplant. Two years after the transplant the patient developed violaceous lichenoid papules coalescing into plaques over the face and upper extremities complaining of decrease visual acuity, foreign body sensation, and eye pain. A slit lamp examination showed injected conjunctiva, superficial I punctate keratopathy and decreased baseline Schirmmer test. Dermatologic evaluation and biopsy demonstrated chronic graft versus host disease along with the diagnosis of secondary keratoconjunctivitis sicca.