Pastor Sangüeza

Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): A distinctive type of cutaneous T-cell lymphoma

BACKGROUND Angiocentric cutaneous T-cell lymphomas of childhood (ACTCLC) are an unusual type of T-cell lymphomas that present with a vesiculopapular eruption mimicking hydroa vacciniforme. Most patients have been children from Asia and Latin America. OBJECTIVE The purpose of this study was to describe four cases of ACTCLC; to discuss its clinical, histopathologic, and immunohistochemical features; to consider its possible relationship to the Epstein-Barr virus (EBV); and to clarify its classification within the spectrum of angiocentric lymphomas. METHODS The clinical, histopathologic, and immunohistochemical features of four cases of ACTCLC were identified and analyzed. In addition in situ hybridization for EBV was performed in all cases. RESULTS The clinical features were similar to previous cases reported under different names, such as hydroa-like lymphomas, edematous, scarring vasculitic panniculitis. Histologically, all showed angiocentric infiltrates composed mainly of T cells. In all cases there were variable numbers of CD30+ cells. The EBV was present in three of the cases. CONCLUSION ACTCLC is a distinct type of T-cell lymphoma. It affects mainly children, and the EBV appears to play a role in the pathogenesis of this disease.

Mucocutaneous leishmaniasis: A clinicopathologic classification

BACKGROUND Leishmaniasis, a chronic parasitic disease transmitted by Phlebotomus sandflies, affects millions of patients. Mucocutaneous leishmaniasis caused by Leishmania brasiliensis is endemic in much of South America. OBJECTIVE Our purpose was to develop a system for staging mucocutaneous leishmaniasis on the basis of clinical, histopathologic, epidemiologic, and immunologic criteria. METHODS Seventy-five Bolivian patients with mucocutaneous leishmaniasis were evaluated by history and physical examination; cutaneous, mucosal, and bone marrow biopsy specimens; and immunologic assessment. RESULTS Mucocutaneous leishmaniasis may progress through three clinical stages: (1) the primary or cutaneous, (2) the cicatricial, and (3) the secondary or mucocutaneous stages. The primary stage can be further subdivided into three phases: lymphoplasmocytic, tuberculoid, and diffuse cutaneous phases. The secondary stage can also be subdivided into three phases: edematous, granulomatous proliferative, and granulomatous necrotizing stages. CONCLUSION A classification or staging system enhances the ability to evaluate patients with mucocutaneous leishmaniasis and determine more accurately their prognosis and treatment.

Rhabdomyoma of the tongue

Rhabdomyomas, which are rare striated muscle neoplasms, may occur on oral or genital mucosal surfaces as well as on the skin. Little discussion of rhabdomyomas occurs in any dermatology or dermatopathology literature. We report an 84-year-old patient who had a rhabdomyoma of her lateral tongue and discuss the clinical and histologic characteristics of this unusual type of benign neoplasm.