Alopecia universalis in liver transplant patients treated with cyclosporin.
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Adult↗
Biomedical subjects
R Caputo
Publications and source records attributed to R Caputo.
Porokeratosis and immunosuppression.
Immunosuppression may favour the development of disseminated superficial porokeratosis (DSP). We report the clinical features and the outcome of DSP in 24 patients receiving immunosuppressive treatment (group A), and compare the characteristics of the disease with those of 13 immunocompetent patients with DSP (group B). The two groups were similar with regard to age, sex, area of skin involvement and mean follow-up. There was a family history of DSP in only two patients in group A, compared with five patients in group B (P = 0.03). The skin type, based on the tanning response to sunlight, was not significantly different between the two groups. Two of the 24 patients in group A had high sun exposure, compared with five of the 13 patients in group B (P = 0.03). Moreover, 10 patients in group A and 11 in group B (P = 0.01) exhibited worsening of the disease after exposure to sunlight, usually during the summertime. These observations appear to support the hypothesis that sun exposure is not always essential for the development of porokeratosis in immunosuppressed patients. None of our patients developed skin cancer in porokeratotic lesions during the follow-up period.
Adult↗
Acral persistent papular mucinosis in two sisters.
We describe two young sisters with an asymptomatic papular eruption on the forearms, the clinical, histopathological and ultrastructural features of which were consistent with acral persistent papular mucinosis. Familial occurrence of this uncommon disease is exceptional.
Adolescent↗
Lichen striatus--a chameleon: an histopathological and immunohistological study of forty-one cases.
Lichen striatus (LS) is an uncommon dermatosis that generally affects children. The histopathology of LS often shows a polymorphic epidermal reaction process of variable lichenoid and spongiotic changes having no specific histopathological criteria or simulating other diseases. In the present study, we have evaluated the histopathological features of 41 biopsy specimens and the immunohistochemical aspects of 10 cases of LS. In 50% of the cases, we found histopathological and immunopathological features constantly present and allowing a correct diagnosis. In 9 cases, the histopathology was not specific, and in 10 cases, a clear differentiation from other diseases was not possible. Immunohistochemical study demonstrated a CD3+ T-lymphocytic infiltration in which CD8+ cells surrounded necrotic keratinocytes and intraepidermal vesicles filled with Langerhans cells. These findings seem to corroborate the hypothesis that a somatic mutation of a keratinocytic clone could induce an autoimmune response of the host.
Antigens, CD↗
Tuberculosis verrucosa cutis which remained undiagnosed for forty-three years.
An unusual case of tuberculosis verrucosa cutis of the left lower limb in a 65-year-old woman is reported. The disease, arising on the plantar aspect of the foot in 1950, was correctly diagnosed by culture of Mycobacterium tuberculosis complex from a skin biopsy only in 1993. A spectacular improvement of the cutaneous lesions was achieved using combined therapy with isoniazide, rifampicin, ethambutol and pyrazinamide.
Aged↗
The various clinical patterns of xanthoma disseminatum. Considerations on seven cases and review of the literature.
BACKGROUND: Xanthoma disseminatum (XD) is a rare mucocutaneous xanthoma nowadays classified as a benign non-Langerhans cell histiocytosis. OBJECTIVE: We studied 7 cases of XD with a long follow-up and reviewed the literature in order to compare and discuss previous reports. METHODS: We studied clinical manifestations, course, histopathologic, ultrastructural and immunohistochemical findings of 7 cases of XD followed for 1-20 years. RESULTS: We report our findings and compare our cases with those described in the literature. CONCLUSIONS: XD and Langerhans cell histiocytosis were distinguished by clinical differences, as well as ultrastructural and immunohistochemical methods. Three clinical patterns of XD appeared to emerge in our group of patients and in the cases reviewed from the literature.
Adult↗
Eruption resembling erythema gyratum repens in linear IgA dermatosis.
We report a case of linear IgA dermatosis associated with eruptions resembling erythema gyratum repens in a 62-year-old man. The patient revealed no clinical and laboratory evidence of an underlying malignancy. The presence of eruptions similar to erythema gyratum repens during the course of bullous dermatoses has been described in only eight reports.
Basement Membrane↗
Persistent nodular lesions caused by "bee-sting therapy".
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Aged↗
Scanning electron microscopy in the diagnosis of pili multigemini.
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Adult↗
Self-healing juvenile cutaneous mucinosis.
BACKGROUND: Mucinoses represent a puzzling and heterogeneous group of rare diseases, and self-healing juvenile cutaneous mucinosis is an extremely rare disease among them. OBSERVATIONS: A scleroedematous condition of the face, associated with papular lesions and arthropathies, had occurred in a 5-year-old boy 10 days after onset of fever, arthralgia, muscle tenderness, and weakness. RESULTS: Histologic examination revealed an edematous dermis, occupied by mucin. Skin lesions and the joint swellings disappeared spontaneously after 2 months. At the follow-up 5 years later, the patient remains in excellent health. CONCLUSION: Although exceptional, this entity has a well-defined clinical picture, marked by manifestations that are initially worrisome but which, surprisingly, prove to be temporary and benign.
Child, Preschool↗
Olmsted syndrome in twins.
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Child, Preschool↗
Pharmacological profile of dextrorphan.
Dextrorphan (CAS 125-73-5) is the active metabolite of the antitussive agent dextromethorphan (CAS 125-71-3). The activity of dextromethorphan, its specific pharmacology, acute toxicity and general pharmacology in respect to the central nervous system were investigated in comparison to dextromethorphan. The studies showed that dextrorphan exerts an antitussive activity comparable to the one of dextromethorphan, but a better tolerability and a lower toxicity. These results suggest to use dextrorphan instead of its precursor dextromethorphan in therapy.
Administration, Oral↗
An unusual case of non-Langerhans cell histiocytosis.
A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.
Child, Preschool↗
Pityriasis rotunda: report of a familial occurrence and review of the literature.
Pityriasis rotunda is an uncommon dermatosis characterized by multiple, widely distributed, strikingly circular hypopigmented or hyperpigmented patches that are slightly scaly. It has been described in Oriental and black patients, usually in association with certain infective or malignant systemic diseases. Pityriasis rotunda is rare in white patients and does not act as a marker of malignancy. Our ultrastructural and histologic findings demonstrated that pityriasis rotunda is more closely related to congenital ichthyoses than ichthyosis vulgaris, contrary to previous reports. On the basis of our studies and a review of the literature, it seems that two types of pityriasis rotunda exist with significant prognostic differences.
Adult↗
Rapp-Hodgkin syndrome and AEC syndrome: are they the same entity?
Rapp-Hodgkin syndrome and AEC syndrome are two disorders in which ectodermal dysplasia and clefting are associated. Rapp-Hodgkin syndrome is an autosomal dominant condition characterized by cleft lip and palate, peculiar craniofacial features, and ectodermal dysplasia, consisting of abnormalities of teeth, hair, nails and sweating. AEC syndrome manifests the same defects plus ankyloblepharon and a higher frequency of scalp dermatitis. A child affected by ectodermal dysplasia associated with clefting, ankyloblepharon, severe scalp dermatitis, and the characteristic Rapp-Hodgkin facies is reported. The overlap between Rapp-Hodgkin syndrome and AEC syndrome is discussed. Critical review of both disorders suggest that AEC syndrome and Rapp-Hodgkin syndrome represent the same entity.
Cleft Palate↗
Multiple pilomatricomas in Rubinstein-Taybi syndrome: a case report.
Rubinstein-Taybi syndrome is a multisystem developmental disorder due to an autosomal dominant mutation. It is clinically defined by the presence of peculiar facies, mental retardation, and broad thumbs and first toes. Important dermatologic findings include hirsutism, keloids, hemangiomas, and dermatoglyphic abnormalities. We report a 12-year-old girl with the typical phenotype of Rubinstein-Taybi syndrome, associated with numerous pilomatricomas. These are benign epithelial neoplasms with hair cell differentiation that may have a familial transmission. Pilomatricomas have not been reported in patients with Rubinstein-Taybi syndrome, although their association with myotonic dystrophy, another autosomal dominant disorder, is well known. Possibilities to explain the association include contiguous gene syndrome, the action of a pleiotropic gene, predisposition to malformations, and mere coincidence.
Child↗
Asymmetric periflexural exanthem of childhood: report of two new cases.
A newly described exanthem of infancy consists of an eczematous or scarlatiniform eruption that starts initially from one axillary fold and spreads unilaterally and centrifugally on the trunk and the proximal part of the upper limb. Minor lesions are infrequently present on the contralateral side. The rash may be slightly pruritic and sometimes associated with a moderate regional lymphadenopathy. The appearance in small epidemics and the seasonal prevalence imply a viral agent as the probable origin. We report two new cases of this entity, which appears to be not so rare.
Arm↗
Clinical features and course of Kaposi's sarcoma in kidney transplant patients: report of 13 cases.
We retrospectively evaluated the prevalence of Kaposi's sarcoma (KS) in 820 kidney transplant recipients with a follow-up period of at least 6 months. Thirteen patients developed a KS (1.6%): 2 were under conventional therapy and 11 under ciclosporin A. The onset of KS was 38.7 +/- 38.3 (range 6-124) months after transplantation in the whole population and after 33.9 +/- 19.7 months in the patients treated with ciclosporin A only. Nine were men and 4 women (male/female ratio: 2.25:1). The mean age at KS occurrence was 36.8 +/- 11.1 years. The mean follow-up period since KS diagnosis was 35.9 +/- 19.5 months. Clinical manifestation and severity of KS were heterogeneous: 5 patients had a KS with cutaneous involvement only, 8 patients a KS with multiple skin and mucosal and/or visceral lesions. Only 2 patients from the second group died of peritonitis due to intestinal lesions. In these 2 patients, immunosuppressive therapy had either been increased or reintroduced after a partial regression of KS. In all other patients, therapy was promptly reduced or withdrawn. In 1 patient local radiation therapy plus intralesional bleomycin administration were started and 1 patient received intralesional vincristine. Nine patients had a complete and 2 a partial remission of lesions. After therapy reduction, 4 patients lost their kidney (these patients however, had an already ongoing chronic rejection at KS diagnosis), in 2 there was an improvement of graft function, and in the other patients it remained stable. Our experience confirms that in most cases reduction or withdrawal of immunosuppression halts the evolution of both cutaneous and visceral lesions, without compromising graft function.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗