Mario Magaña

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.

Gnathostomiasis: clinicopathologic study.

Abstract:Gnathostomiasis is a systemic parasitic disease that is caused by the ingestion of contaminated raw fish, the intermediate host. Involvement of the skin is a common event, and when it does happen, it can produce a superficial or creeping eruption, pseudofurunculosis, and nodular migratory panniculitis. We carried out a retrospective study of 946 cases of gnathostomiasis; 66 of them had skin biopsies. The diagnosis was made based on clinical and epidemio-logic findings as well as the therapeutic response. The most common skin finding was nodular migratory panniculitis affecting the trunk. Most of the patients were males between 20 and 40 years of age. Histopathologically, we were able to see the larva and make a definitive diagnosis in 15 cases, and in 12 cases, the worm was retrieved during the surgical procedure. In remaining cases, despite of our inability to identify the larva, the histopathologic changes were quite characteristic and included: dermal and hypodermal edema with dense mixed infiltrates composed of eosinophils admixed with lymphocytes and neutrophils, eosinophilic vasculitis, flame figures, areas of necrosis, and hemorrhage. Thus, the presence of these histopathologic features in the context of a clinical picture suggestive of gnathostomiasis allows the pathologist to make the correct diagnosis.

Giant Congenital Juvenile Xanthogranuloma

Abstract: Congenital giant juvenile xanthogranuloma is an uncommon lesion that apparently has benign biologic behavior in most reported patients. In one of our three patients it was necessary to perform histochemical reactions with CD 1 (‐), MAC 387, and electron microscopy to rule out Langerhans cell histiocytosis. The other patients had a typical histologlc picture, with foamy histiocytes and Touton cells in association wtth lymphocytes and eosinophils. Comments about treatment are also made.

Skin metastasis from hepatocarcinoma.

Cutaneous metastases may be the first sign of an internal neoplasm; hepatocellular carcinoma is a very rare source of skin involvement and presents with a protean morphology: papules, nodules, and masses that may be by natural sources or by artificial mechanisms. We present a 41-year-old man with hepatocellular carcinoma and hepatic cirrhosis who developed metastatic disease, which in the skin showed as a pyogenic granuloma. We analyze 38 cases from the literature and present a practical review of the subject.

Lucio's Phenomenon Is a Necrotizing Panvasculitis: Mostly a Medium-Sized Granulomatous Arteritis

Lucios phenomenon (LPh) is a vasculitis clinically described in 1852 and microscopically documented in 1948 in patients with diffuse lepromatous leprosy; however, at present, there is no a clear concept about the pathogenesis of the necrosis, or about the type, size, and site of the damaged vessel. The objective of this study was to elucidate the type, size, site, and form of vessel damage in LPh in a retrospective, clinical, and histopathological study. Clinical information was obtained from the charts and records and/or from the histopathology request. Slides stained with hematoxylin and eosin, Ziehl-Neelsen, and Fite-Faraco were retrieved from our files. Direct immunofluorescence had been performed in 6 cases. Twelve cases fulfilled clinical evidence to make unequivocal diagnosis of diffuse lepromatous leprosy with LPh. All of them had necrotic, irregular, purpuric, and/or ulcerative lesions, which under the microscope showed medium-sized arteries, with their walls involved by clusters of macrophages containing large amounts of bacilli, distortion of the structure of the vessel wall, narrowing, and obliteration of their lumen. Smaller vessels showed changes of the leukocytoclastic type. LPh is a distinctive type of granulomatous and necrotizing panvasculitis; the involved vessels are mostly medium-sized arteries, located deeply in the skin, at the base, and within the hypodermis, but any other vessel is likewise involved, their occlusion leads to ischemic necrosis of the whole skin, frequently with detachment of the epidermis. These changes explain clearly and logically the clinical features observed more than 150 years ago.

Cutaneous Amebiasis in Pediatrics

BACKGROUND Cutaneous amebiasis (CA), which is still a health problem in developing countries, is important to diagnose based on its clinical and histopathologic features. OBSERVATIONS Retrospective medical record review of 26 patients with CA (22 adults and 4 children) treated from 1955 to 2005 was performed. In addition to the age and sex of the patients, the case presentation, associated illness or factors, and method of establishing the diagnosis, clinical pictures and microscopic slides were also analyzed. CONCLUSIONS Cutaneous amebiasis always presents with painful ulcers. The ulcers are laden with amebae, which are relatively easy to see microscopically with routine stains. Erythrophagocytosis is an unequivocal sign of CA. Amebae reach the skin via 2 mechanisms: direct and indirect. Amebae are able to reach the skin if there is a laceration (port of entry) and if conditions in the patient are favorable. Amebae are able to destroy tissues by means of their physical activity, phagocytosis, enzymes, secretagogues, and other molecules.

Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients.

Abstract:Hydroa vacciniforme–like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein–Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4–27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein–Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.

Congenital melanocytic nevus: two clinicopathological forms.

Abstract:Congenital melanocytic nevus (CMN) is a hamartomatous disease for which many attempts at classification have been proposed. This disease is relevant not only because of its functional and esthetic implications but also because it is a well-documented precursor to malignant melanoma. We performed a clinical and pathological prospective study of 200 cases of CMN and were able to identify 2 different forms of CMN, each one with biological, clinical, and histopathological features and criteria that are consistent and repeatable. We propose to name them types I and II. Type I CMN is the most common, usually, if not always, a single lesion, it consists of a plaque that involves only 1 anatomic region and does not go beyond it; type I CNM grows in proportion to the growth of the child, melanoma rarely develops from it, and when it does it usually arises at the dermoepidermal junction. Its histopathology shows cords, strands, nests, and single units of melanocytes spreading between collagen bundles only in the dermis and frequently the epidermis too, but without trespassing to the hypodermis, that is, it is superficial. Type II CMN is always made up of many lesions, one of them being very large and surrounded by many lesions; histopathologically, it involves not only the skin but also deeper structures, sometimes bone and central nervous system; therefore, it is deep; when melanoma develops, it does in the dermal component and usually from the largest plaque. This type of CMN is the one that develops neurocutaneous melanocytosis. This system is not only easy and logical but it also has biologic advantages and the clinical–pathological correlation and criteria are repeatable by clinicians and pathologists.