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.

Multiple primitive neuroectodermal tumors.

Primitive neuroectodermal tumors are rare, highly aggressive neoplasms that affect both sexes and occur in all age groups. They are a large group of neoplasms with neuroepithelial differentiation, including cutaneous neuroblastomas. Histopathologically they are characterized by a rather uniform population of small, dark cells, with or without Homer Wright rosettes. Immunohistochemically they stain for one or more of the reputed neural markers. Ultrastructurally they show interdigitating processes containing neurosecretory granules, intermediate filaments, and microtubules. We describe a patient with unusual multiple peripheral neuroectodermal tumors localized to the skin, with benign biologic behavior. The patient related the appearance of new lesions for several years without evidence of visceral dissemination or systemic complications. The histopathologic, immunohistochemical, and ultrastructural findings support the diagnosis of a peripheral neuroectodermal tumor.

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.

Rhabdoid tumor of the skin.

A tumor in the skin of a 42-year-old man was analyzed by light and electron microscopic study and immunohistochemistry. The tumor cells were large and contained eosinophilic, periodic acid-Schiff (PAS)-positive inclusions in the cytoplasm. Immunohistochemically, the neoplasm was positive for intermediate filaments, cytokeratin, vimentin, desmin, and alpha-1-antichimotrypsin, and negative for S-100 and leukocyte common antigen (LCA). Ultrastructurally, the cytoplasm and cellular processes of the cells were inundated with intermediate filaments, some of which were tightly bundled. Junctional complexes and secretory granules were absent. These features suggest a rhabdoid tumor: a malignant tumor of uncertain origin.

Intravascular lymphomatosis: report of an unusual case with T cell phenotype occurring in an adolescent male.

Intravascular lymphomatosis is a rare disorder which most often occurs in the elderly. The overwhelming majority of the cases studied immunophenotypically have expressed a B cell phenotype. We report an unusual case of T cell intravascular lymphomatosis occurring in an adolescent male.

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.

[Ductal tumor of the eccrine sweat glands]. / Tumor ductal de glándula sudorípara ecrina.

A dermal ductal tumor developed in sweat gland in 82 years old man, is presented. The lesion was located in the left leg. The clinical diagnosis was verrucous carcinoma and pigmented nevus. Histologically, it was a benign tumor.

[Eruptive and progressive cutaneous leiomyomatosis associated with cerebrovascular abnormalities and osteoma of the petrous bone]. / Leiomiomatosis cutánea eruptiva y progresiva a anomalías vasculares cerebrales y osteoma del peñasco.

He was a patient with 3,065 lesions of different sizes on the skin, placed in arms, legs, sternum area and right thorax, since he was 22 years old. The leiomyomas were painful and with electrical sensation, when you touch them. Faint. He denied similar lesions in relatives. We found abnormal brain vessels and bone tumor in right petrous bone.

[Cancer of the breast in the male. Presentation of 2 cases]. / Cáncer de glándula mamaria masculina. Presentación de dos observaciones.

We present two cases of adenocarcinome of the male mammary gland, without past history of gynecomastia, cirrhosis, malnutrition, hyperoestrogenism, estron therapy for prostatic cancer, testicular tumor, Klinefelter Syndrome, parasitosis, trauma or irradiation. Male mammary adenocarcinoma is rare in all hospital. In our center we found two cases among 204 female mammary adenocarcinomas, that is one porcent, in a revisión between 1971 and 1980. Most male mammary adenocarcinomas are of the ductal/infiltrative type, early metastasis to regional ganglia or by continuity to adjacent skin.

[Actinic reticuloid and pigmented exuberant conjunctivitis. Report of case]. / Actinorreticulosis y conjuntivitis exuberante pigmentada. Reporte de un caso.

The authors report the case of a patient showing simultaneously actinic reticuloid (AR) and pigmented exuberant conjunctivitis (PEC), which are frequently encountered separately in the highlands of Bolivia. This pathological association could be in favour of the actinic origin of PEC, whereas for RA this origin is quite obvious. The main characteristics of PEC are described, insisting on differentiation with spring conjunctivitis. As a consequence of this association and the observation of other similar cases of PEC in the course of other photodermatoses, the authors are led to consider PEC as the only extra-cutaneous alteration up to now, and one more feature in the clinical picture of photodermatoses of altitude.

[Recurrent digital fibromatosis in children]. / Fibromatosis digital infantil recidivante.

We present two cases of recurrent digital fibromatosis, one of them presented lesions since birth, aun the other one since seven months of age. The first case was treated surgically, with relapses and marked deformities. The second one underwent only biopsy. Histopathologic examination showed active fibroblasts, in whorl disposition, and intracytoplasmic inclusions in both cases. By its natural evolution, we believe that recurrent infantile digital fibromatosis has an initial stage, a tumoral or hyperplastic stage, and finally spontaneous evolution, which explains the fact that it is not found in adults.

[Clear cell acanthoma]. / Acantoma de células claras.

We present a case of clear cell acanthoma localized on inferior third of the external portion of the left leg, the evolution was of one and a half year, with previous insect bites on the area. We insist on the histologic appearance, characterized by hyperplasia of epithelial cells, intercellular bridges, and presence of acidophilic hyalins intracytoplasmiques. Nous postulons la possibility of a viral etiology, transmitted by insect bite, of an epidermotropal virus.

[Cutaneous and muucocutaneous leishmaniasis. New findings on the subject]. / Leishmaniasis cutánea y cutáneo-mucosa. Nuevos aportes sobre el tema.

The authors have carried a work in which they studied the Leishmaniasis of the skin and their cutaneo-mucosal manifestations, marking on the fact that both are caused by the same agent. They considered also that the visceral Leishmaniasis would be occasioned by the same parasite but in this latter case, it can occur only in anergic people. Finally, they made a clinical and pathologic classification of the cutaneous Leishmania and cutaneo-mucosal Leishmania in: 1. A primary period with 3 histologic manifestations very well known. 2. A period of latency which is variable in time of duration; some times is permanently with parasites in quiescent state in the cells of the reticulous endothelial system. 3. A period of reactivation with cutaneo-mucosal lesions, which shows 3 anatomic and clinic periods: erythematous edematous, granulomatous-proliferative and granulose-necrotic with very important destructions in oropharynx.

[Lipoidproteinosis]. / Lipoidoproteinosis.

We report the first case of L.P. in our country with typical lesions of skin, pharyngeal and laryngeal mucous membranes. Our observation did not indicate any history of consanguinity nor family incidence, nor visceral engagement. The hepatic biopsy was normal. Connected with the main illness we found a radiopaque area in the right frontal bone which engaged the interior and exterior walls, and a calcification nodule in the base of the right maxillary sinus.

[Eczematide-like purpura (itching purpura--angiodermatitis pruriginosa disseminata)]. / Eczematide-like púrpura (itching púrpura--angiodermitis pruriginosa diseminada).

Between the group of pigmentary and purpury dermatitis, the eczematoid like purpura is better known for its clinical, histological and defined evolutive characteristics. We reviewed all existent biographies and presented two cases of this unusual afection, both with allergic backgrounds, inflammatory capillary lesions and purpura that began in the region of the malleolus and progressed towards the legs, thighs, pelvic girdle, until the chest. These lesions were essentially pruriginous. The histology did not demonstrate signs of allergic vasculitis. What drew our attention was the presence of red blood cells in the Malpighi stratum mucosa. Due to the scarcity of literature and few cases we were not able to clarify satisfactorily this epidermotrophism of the blood cells.

[Melanotic progonoma with androgenic manifestations]. / Progonoma melanótico con manifestaciones androgénicas.

The Melanotic Progonoma is not every common. It is frequent under a year of age however it has been in patients over 23 years old. It has several names. This tumor does not give metastasis, it is identified benign. It is located in several places like the anterior mediastinum, oropharynx, epididymis, particularly in the oral cavity. It is frequent in black people, less in the whites and exceptional in the indians. The kind of tumor has occasionally hormonal evident like acid vanil mandelico and androgens hypersecretion, with constant microscopic morphology. It cames from the neutral crest cells, then we think that the better name will be Neurocrestom Melanotic Infantile.

Hallermann-Streiff-Francois syndrome.

This is a typical case of Hallermann-Streiff-Francois Syndrome in which the glaucomatous complication may be caused by developmental malformations in the anterior segment of the eye. Bilateral trabeculectomy succeeded in controlling the tension after a follow-up of two years. Glaucomatous complication seems to be frequent and as important as the other described cardinal symptoms. The histologic examination of the skin confirmed the atrophic changes that were observed clinically.