[Hair casts in a brother and sister, one of whom is a Darier's disease carrier].
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Biomedical subjects
Publications and source records attributed to R Caputo.
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Response to treatment with daily intramuscularly administered crude calf thymic extract (Suppressin) in 11 patients with Langerhan's histiocytosis (L.H.) is reported. In ten patients, T-lymphocytic subsets were studied before starting immunotherapy: OKT3 positive and OKT4 positive cells were reduced in four patients; OKT8 positive cells were reduced in two patients; three patients were normal. After immunotherapy, one patient entered complete remission, four patients had stationary disease, and six had marked clinical progression. Subsequently eight patients underwent conventional chemotherapy, and only three entered complete remission. This study has demonstrated the heterogeneity of immunological patterns in L.H. and justifies the necessity for investigations on the immunoregulatory mechanism of L.H.
To clarify the nature of reticulohistiocytoma of the dorsum, 19 cases, including three of the seven original cases described by Crosti, were evaluated clinically, histologically, and immunologically. In seven cases gene rearrangement analysis was also performed. Results indicate that reticulohistiocytoma of the dorsum must be considered a primary cutaneous B cell lymphoma of follicular center cell origin. This localized skin disease has a very slowly progressive course, with many patients showing no systemic involvement even after prolonged follow-up.
After an accident in a chemical plant in Seveso, Italy, on July 10, 1976, 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) spread over a populated area. The event was exceptional because children were also affected and because the contamination took place not only through direct exposure but also through inhalation and the ingestion of contaminated foods, especially fruits and vegetables. This paper illustrates the early dermatologic lesions, the late acneic (chloracne) lesions, and their evolution during a 10-year period. Peculiar cutaneous findings, histologic data, and a comparison with previously reported similar accidents are also included.
Reticulohistiocytosis, a rare disorder occurring almost exclusively in adults, was seen in "pure" diffuse cutaneous form in an 8-year-old boy who had tuberous sclerosis since birth. The clinical features consisted of many papulonodular lesions, located mainly on the trunk and to a lesser extent on the head and limbs. Histologic findings were distinctive. Ultrastructural examination revealed the presence of pleomorphic cytoplasmic inclusions in almost all of the giant histiocytic cells of the infiltrate.
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Dermochondrocorneal dystrophy (François' syndrome) is an extremely rare disorder characterized by firm, nodular lesions involving the hands and the face; osteochondrodystrophy of the peripheral extremital bones, resulting in limitation of movement; and corneal dystrophy marked by white or brownish opacities. A nonfamilial case of dermochondrocorneal dystrophy was studied in a 45-year-old woman who had severe involvement of the gingival and palatal mucous membranes.
Generalized eruptive histiocytoma is a papular, non-X, nonlipidic, self-healing histiocytosis affecting mainly adults. This article describes the clinical, histologic, ultrastructural, and immunologic findings in four children, aged 10 months to 4 years, who were suffering from this disease. Generalized eruptive histiocytoma of children differs from generalized eruptive histiocytoma of adults only in that the lesions are not symmetrically distributed, they do not affect the mucous membranes, and they may become xanthomatous.
We describe a unique and puzzling case of a 7-month-old baby with a non-X hypertriglyceridemic histiocytoxanthomatosis. The disease was characterized by a massive nodular eruption that was clinically, histologically, and ultrastructurally consistent with juvenile xanthogranuloma, but it had a rapid, fatal evolution.
Nonspecific gyrate erythemas (NGE) are commonly associated with hypersensitivity reactions to exogenous or endogenous antigens. A case of NGE with clinical resemblance to tinea corporis in a patient with a dental radicular cyst is reported. Cutaneous lesions disappeared after surgical excision of the dental cyst. The clinical and pathological details are described and the relationship between radicular cyst and cutaneous lesions discussed. Earlier reports are reviewed and the possibility of a focal origin is emphasized.
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Jejunal biopsies from patients with either dermatitis herpetiformis or coeliac disease were freeze-fractured and compared with normal jejunal biopsies. The intestinal mucosa of the normal biopsies showed a normal structure, with well-developed and tightly packed microvilli; in dermatitis herpetiformis and coeliac disease degenerative changes of the intestinal mucosa occurred. These changes appeared to be segmental in dermatitis herpetiformis and diffuse in coeliac disease. Emphasis is placed on changes in the tight junctional net at the base of the microvilli, which could represent cellular damage related to increased intestinal permeability to macromolecules in these diseases. An interpretative hypothesis for these observations is presented.
Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head. Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution. All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.
We describe a form of diffuse, rapidly self-healing xanthomatosis with the clinical and ultrastructural features of hyperlipemic xanthoma but occurring in a subject with normal lipid metabolism and with no associated systemic disorders.
This immunopathologic study of both normal and pathologic skin specimens (contact dermatitis [CD], lichen planus [LP], cutaneous T cells lymphoma [CTCL], and histiocytosis X [HX]) allowed as to differentiate four types of dermal OKT6+ cells: (1) cells with the same morphologic features as epidermal Langerhans cells (LCs), rarely found in either normal or pathologic dermis; (2) cells structurally similar to LCs but lacking Birbeck granules (BGs), found mainly in CD and LP; (3) larger cells rich in cytoplasmic organelles, only 5% of which contained BGs. They were especially common CTCL; and (4) cells typical of HX.
Histiocytoses represent a large, puzzling group of rare skin diseases. The purpose of this review is to schematically outline the clinical, histologic, and ultrastructural features of the most important histiocytic syndromes and to provide the pertinent differential diagnoses. For convenience, we have followed the criterion suggested by Winkelmann, distinguishing these conditions into X and non-X. Among the non-X histiocytoses the self-healing forms have been treated first; the progressive forms follow.