Histocitosis benigna cefálica: reporte de 4 casos / Benig cephalic hystiocytosis: report of 4 cases

Se presentan cuatro casos de Histiocitosis Benigna Cefálica, una histiocitosis cutánea de los niños autoinvolutiva, no X, no lipídica. La edad de comienzo fue entre los 5 y los 9 meses de vida, con pápulas y máculas eritematosas localizadas en la cabeza (principalmente en mejillas) con posterior diseminación a tronco y miembros en tres de los casos. Con microscopio óptico observamos infiltrados histiositario en la dermis superficial, S-100 negativo por método de las peroxidasas (método PRP). Uno de los casos mostró partículas vermiformes y uniones de tipo desmosomas en el estudio ultramicroscópico. No se encontraron gránulos de Bierbeck. La Histiositosis Benigna Céfalica es una patología autoinvolutiva que no requiere tratamiento.

A solitary variant of congenital self-healing reticulohistiocytosis: solitary Hashimoto-Pritzker disease.

Four neonates had solitary, congenital, rapidly growing, spontaneously ulcerating tumors of the face, trunk, and extremities. No extracutaneous involvement was found, and all lesions spontaneously involuted. Mononuclear cells of the cutaneous infiltrate were Langerhans' cells. These findings expand the spectrum of congenital self-healing reticulohistiocytosis.

Malignant histiocytosis of Bernese mountain dogs.

A second histiocytic proliferative disorder, which resembled malignant histiocytosis of man, was identified in 13 Bernese mountain dogs. Malignant histiocytosis was clearly distinct from systemic histiocytosis, which was reported earlier in this breed. Eleven cases involved male dogs. Ten dogs occurred in the same family line as the dogs afflicted with systemic histiocytosis. Clinical or radiological evidence of pulmonary involvement was present in nine dogs. Neurological disturbances were present in five dogs. Anemia was observed in five dogs and was associated with prominent erythrophagocytosis in two instances. The clinical course was rapidly progressive. Necropsy examinations revealed that infiltrates were especially frequent in the lungs and hilar lymph nodes. Other lymph nodes, liver, spleen, and central nervous system were also frequently involved. Evidence for primary pulmonary involvement was present in seven dogs. The original diagnosis in seven cases was large cell anaplastic carcinoma of the lung by light microscopy only. The infiltrates were composed of large, pleomorphic, phagocytic mononuclear cells and multinucleated giant cells which also manifested marked cytological atypia and numerous, frequently bizarre, mitotic figures. Ultrastructural studies and the immunohistochemical demonstration of lysozyme and alpha 1-antitrypsin in the tumor cells in the majority of cases were consistent with a macrophage origin.

Benign cephalic histiocytosis. A case report and ultrastructural study.

A 4-year-old Hispanic boy had an asymptomatic eruption of red-brown macules and papules on the face, neck, trunk, and upper extremities. Examination of the skin lesions by light microscopy revealed a diffuse infiltrate of histiocytes in the upper dermis. Examination by electron microscopy showed wormlike inclusion bodies within the histiocytes. A diagnosis of benign cephalic histiocytosis was made on the basis of these distinct clinical, histologic, and ultrastructural features.

Pagetoid reticulosis (Woringer-Kolopp disease): histiocyte marker (lysozyme) study and ultrastructural observations.

A case of pagetoid reticulosis is presented. Histopathology showed infiltration of the epidermis by mononuclear cells. Twenty percent of the mononuclear cells showed the presence of lysozyme indicating a histiocytic origin. Electron microscopy confirmed the presence of lymphocytes and histiocytes but these cells were outnumbered by Sézary cells. The presence of large numbers of Sézary cells indicates that pagetoid reticulosis is a cutaneous T-cell lymphoma closely related to mycosis fungoides.

Congenital self-healing reticulohistiocytoma. A clinical, histological and ultrastructural study.

A 2 300 g male infant presented at birth with multiple, firm, bluish skin nodules, petechiae and thrombocytopenia. Physical examination was otherwise normal and no biochemical signs of organ dysfunction were found. A skin biopsy, done on the second day of life, was consistent with self-healing reticulohistiocytoma by light and electron microscopy. The nodules spontaneously resolved during the following months. At one year follow-up, he was without evidence of disease, the only sequelae being occasional discrete scarring. The major problem with this rare entity lies in its dramatic presentation and the chance of possible hazardous "overtreatment".

Systemic histiocytosis of Bernese mountain dogs.

A histiocytic proliferative disorder was identified in six closely related Bernese mountain dogs. Clinical signs included anorexia, weight loss, stertorous respiration, and conjunctivitis with marked chemosis. Multiple cutaneous nodules were distributed over the entire body but were especially prevalent in the scrotum, nasal apex, nasal planum, and eyelids. Lesions consisted of perivascular infiltrates of large histiocytes as well as minor populations of lymphocytes, neutrophils, and eosinophils. Histiocytes were further characterized by enzyme histochemistry and electron microscopy. Necropsy examinations of four dogs revealed that the histiocytic infiltrates were widespread and involved skin, lung, liver, bone marrow, spleen, lymph nodes, kidneys, testes, orbital tissues, and others. However, skin and peripheral lymph nodes were more consistently involved. The disease course was punctuated by remissions and relapses not clearly influenced by conventional therapeutic measures. Preliminary results of an experimental therapeutic regimen involving administration of bovine thymic extracts in two dogs are present. The relationship of the disorder to other human and canine histiocytic proliferative disorders is discussed.

Mucicarminophilic histiocytosis. A polyvinylpyrrolidone (PVP) storage disease simulating signet-ring cell carcinoma.

In Taiwan, a peculiar type of cell with mucicarmine-positive bubbly or vacuolated cytoplasm was sporadically observed in a variety of surgical specimens removed for neoplastic or non-neoplastic conditions. They closely mimicked signet-ring cell carcinoma. Study of 14 such cases and review of the related literature led to the conclusion that the peculiar cells were storage histiocytes containing polyvinylpyrrolidone (PVP). Because they were shown to be mucicarmine-positive, we designated them " mucicarminophilic histiocytes." Particulate bodies sometimes observed in association with mucicarminophilic histiocytes were believed to be the extracellular counterpart of the cytoplasmic vacuoles. Our survey also revealed that PVP-containing intravenous injection fluids had been used in Taiwan as recently as 5 years ago. For surgical pathologists, PVP-containing mucicarminophilic histiocytes should not be mistaken for signet-ring cell carcinoma or signet-ring cell lymphoma. Mucicarminophilic histiocytes can be identified by the positive mucicarmine, colloidal iron, Gomori methenamine silver, Congo red, Sudan black B, Fontana-Masson argentaffin, and Victoria blue; but negative periodic acid-Schiff, alcian blue, and Giemsa stains.

Benign cephalic histiocytosis.

A 2-year-old boy was seen because of an extensive asymptomatic papular eruption on the face, neck, and shoulders of 18-months' duration. A skin biopsy specimen revealed cellular infiltrates composed predominantly of histiocytes. Electron microscopy of biopsy material disclosed "comma-shaped" inclusion bodies in the cell cytoplasm, consistent with the findings described in benign cephalic histiocytosis. To the best of our knowledge, this is the first report of this clinical entity in the American literature.

Malignant histiocytosis and related tumors. A clinicopathologic study of 42 cases using cytological, histochemical and ultrastructural parameters.

Light and electron microscopical, immunohistochemical and clinical characteristics in 42 cases of malignant neoplasms, arising from true histiocytes, are described. These were separated in a lymphoma-like subtype, called true histiocytic lymphoma (29 patients) and a disseminated variant, called malignant histiocytosis (9 patients). In addition 4 related histiocytic tumors are discussed, including 2 tumors arising from interdigitating cells. Sinus pattern and cytologic features, especially 'window' nuclei, are emphasized as diagnostic criteria. Erythrophagocytosis was not a constant finding. Electron microscopic features, presence of acid phosphatase, acid alpha-naphthylacetate esterase, lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, Ia-antigen and absence of B- and T-cell markers, were important in establishing the histiocytic nature or excluding a non-histiocytic tumor. A distinct male predominance existed (male:female = 2.5:1) with a higher relapse free period in females (p = 0.032). A high number of mitotic figures appeared to be a favourable sign, p = 0.020 and 0.019, for remission rate and relapse free period respectively. The degree of cell differentiation and the immunohistochemical pattern did not show a correlation with remission and relapse free period. Extranodal involvement and the presence of short profiles of endoplasmic reticulum were prognostically unfavourable signs. True histiocytic lymphomas showed a higher remission rate (p = 0.041) and relapse-free period (p = 0.017) than malignant histiocytosis.

A case of malignant histiocytosis.

Malignant histiocytosis, presenting initially with swelling and subcutaneous tumor in the lid, was reported in a 14-year-old boy. The diagnosis of malignant histiocytosis was made on the basis of light- and electron-microscopic findings.

The synovium and synovial fluid in multicentric reticulohistiocytosis--a light microscopic, electron microscopic and cytochemical analysis of one case.

The synovium and synovial fluid have been studied in a patient with multicentric reticulohistiocytosis. Previously unreported histochemical, immunocytochemical and ultrahistochemical findings are presented and their relevance to the aetiology and pathogenesis are discussed. The synovial fluid analysis in this disease is characteristic and may be helpful in its early diagnosis.

Woringer-Kolopp disease (pagetoid reticulosis).

We report the clinical, histopathological, and ultrastructural features of a case of Woringer-Kolopp disease, review the literature, and discuss the relationship of the condition to mycosis fungoides. At the present time it is best to regard Woringer-Kolopp disease as a form of cutaneous T-cell lymphoma that may remain localized for many years or may eventually be associated with widespread mycosis fungoides.

[Malignant histiocytosis in children (pathologicoanatomic study of 16 cases)]. / Zlokachestvennyi gistiotsitoz u detei (patologoanatomicheskoe issledovanie 16 sluchaev).

Malignant histiocytosis is a rare neoplastic disease from the group of acute leukemias. Morphological characteristics of the disease are presented on the basis of histological, histochemical, and electron microscopic examinations of section materials from 16 cases of malignant histiocytosis in children of 6 to 14 years. Systemic focal-diffuse proliferation of tumor cells of histiocytic type of various degrees of differentiation was observed in organs and tissues. Most frequently, typical macro- and/or microscopic lesions were detected in lymph nodes, bone marrow of vertebrae, flat and tubular bones, liver, and spleen. The histiocytic nature of tumor cells was judged primarily from such signs as their capacity for phagocytosis detectable by light and electron microscopy, high diffuse cytoplasmic activity on nonspecific esterase and acid phosphatase, and features of the ultrastructure. In the establishment of the diagnosis, malignant histiocytosis had to be differentiated from Letters-Siwe disease, sinus histiocytosis with massive lymphadenopathy, lymphogranulomatosis, immunoblastic lymphosarcoma, melanoma, undifferentiated cancer.

[Generalized eruptive histiocytoma. Apropos of a case. Review of the literature]. / Histiocytome éruptif généralisé. A propos d'un cas. Revue de la littérature.

About one case of generalized eruptive histiocytoma (G.E.H.) (the second French case) and about thirteen others cases mentioned in literature, the authors remind the clinical, histological, immuno-histochemical and evolutive aspects of this misunderstanding disease, described by Winkelmann in 1963. After having classed it, in the group of nodular, non X, non lipidique, benign histiocytosis, they expose the imprecision of this nosological limits of the G.E.H., in comparison to the bordering diseases. At last, they insist upon the fact that the few number of cases published up to this time, the lack of stand back and the casual transformation into a multicentric reticulo-histiocytosis must induce to watchfulness by requiring for each patient affected by G. E. H. and for a long time, a regular, clinical, radiological and hematological supervision.