Ruggero Caputo

Ceramide composition of the psoriatic scale

This paper investigates the ceramide composition of the psoriatic scale compared with that of normal human SC. A method was optimalized, based on TLC separation followed by densitometry, allowing the provision of good resolution and quantification of ceramide fractions from both normal and pathological specimens. Seven ceramide fractions were isolated and submitted to compositional analysis. The obtained results suggested a revisitation of previous ceramide designation. Therefore a simple classification is suggested, based on grouping ceramides carrying structural similarities under common codes. According to these rules, ceramides were grouped into five classes designated as: (1) Cer[EOS], which contains ester-linked fatty acids, omega-OH fatty acids and sphingosines; (2) Cer[NS], which contains non-OH fatty acids and sphingosines; (3) Cer[NP], which contains non-OH fatty acids and phytosphingosines; (4) Cer[AS], which contains alpha-OH fatty acids and sphingosines; (5) Cer[AP], which contains alpha-OH fatty acids and phytosphingosines. Analysis of ceramides from the psoriatic scale, compared to those from normal human SC, resulted in an impairment of the Cer[EOS] content as well as of the ceramides containing phytosphingosine, with concurrent increase in ceramides containing sphingosine, being the total amount maintained identical. Since one of the suggested pathways for phytosphingosine biosynthesis involves the water addition to the corresponding sphingosine double bond, we can speculate that the observed alteration is due to a deranged water bioavailability, associated with psoriasis.

Histiocytic syndromes: A review

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.

Roaccutane Treatment Guidelines: Results of an International Survey

BACKGROUND Oral isotretinoin (Roaccutane) revolutionized the treatment of acne when it was introduced in 1982. METHODS Twelve dermatologists from several countries with a special interest in acne treatment met to formally review the survey of their last 100 acne patients treated with oral isotretinoin. The primary purpose of the survey was to identify the types of acne patients who were prescribed oral isotretinoin and how the patients were managed. RESULTS Of the 1,000 patients reviewed, 55% of those who received oral isotretinoin had those indications treated historically, i.e. severe nodular cystic acne or severe inflammatory acne, not responding to conventional treatment. Forty-five percent of patients who were prescribed oral isotretinoin however had either moderate or mild acne. Most patients in this group had moderate acne (85%). However, 7.3% had mild acne on physical examination. The criteria for prescribing oral isotretinoin in this less severe group of patients included acne that improves < 50% after 6 months of conventional oral antibiotic and topical combination therapy, acne that scars, acne that induces psychological distress and acne that significantly relapses during or quickly after conventional therapy. Treatment is usually initiated at daily doses of 0.5 mg/kg (but may be higher) and is increased to 1.0 mg/kg. Most of the physicians aimed to achieve a cumulative dose of > 100-120 mg/kg. Mucocutaneous side-effects occur frequently but are manageable while severe systemic side-effects are rarely problematic (2%). The teratogenicity of oral isotretinoin demands responsible consideration by both female patients and their physicians. Significant cost savings when treating acne patients with oral isotretinoin as compared to other treatment modalities were further proven in this study. CONCLUSIONS Our recommendation is that oral isotretinoin should be prescribed not only to patients with severe disease but also to patients with less severe acne, especially if there is scarring and significant psychological stress associated with their disease. Acne patients should, where appropriate, be prescribed isotretinoin sooner rather than later.

Reticulohistiocytoma of the dorsum.

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.

Clinical Features and Course of Kaposi’s Sarcoma in Kidney Transplant Patients: Report of 13 Cases

We retrospectively evaluated the prevalence of Kaposis 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)

Gianotti-Crosti syndrome: A retrospective analysis of 308 cases†

BACKGROUND There is no agreement as to whether papular acrodermatitis of childhood caused by hepatitis B virus can be differentiated from other papulovesicular acrolocated syndromes. OBJECTIVE We attempted to establish whether such differentiation is possible comparing histories, signs, and symptoms of all patients who have been previously diagnosed as having papular acrodermatitis of childhood or papulovesicular acrolocated syndromes. METHODS Files of 308 patients hospitalized in the past three decades were studied. Photographs were examined by a panel of experts to determine whether it was possible to distinguish between papular acrodermatitis of childhood and papulovesicular acrolocated syndromes solely on the basis of cutaneous signs. RESULTS The retrospective analysis confirmed a significant overlapping of the two types of the disease. The blind survey of photographs of the patients revealed that a distinction between the forms was not clinically possible. CONCLUSION Acrodermatitis is a self-limiting cutaneous response to different viruses; clinical differences are probably due to individual characteristics of each patient rather than the causative virus.

The junctions of normal human epidermis: A freeze-fracture study

Freeze-fracture has confirmed the presence of desmosomes, gap junctions, and tight junctions in fixed human epidermis. On the basal cell plasma membrane directed toward the dermis structures are detected that are reminescent of the hemidesmosome. On the lateral and apical plasma membrane of the basal cell and in the spinous and granular layers the desmosome, when cross-fractured, possesses a simple structure. Fine filaments are observed where the attachment plaque is seen in thin section. When the fracture reveals the apposed membranes “en face,” the desmosome appears as an aggregate of particles on both the P and E membrane faces. The area occupied by the junctions is at a different level than that of the adjacent nonjunctional membrane. In the horny layer the desmosome appears as an elevated plaque on the E face and a cluster of particles on the P face. Gap junctions are rather small and are found in a limited number from the basal to the granular layers. Tight junctions as well are rarely observed in the intermediate layers. Both types of junctions are never observed in the horny layer. Freeze-fracture demonstrates the presence of an extensive network of grooves on the P fracture face and ridges on the E fracture face of the plasma membrane of the first cells of the horny layer. The ridges and the grooves are in close relation with the desmosomes.

Pityriasis lichenoides in children: A long-term follow-up of eighty-nine cases

Pityriasis lichenoides is usually classified into an acute and a chronic form. From a review of 89 cases of the disease seen since 1974 it seems that a more realistic classification into three main groups, according to the distribution of pityriasis lichenoides lesions, could be made, namely, a diffuse, a central, and a peripheral form, each characterized by a different clinical course. Conversely, no correlations were detected in our series between the severity of skin lesions and their distribution or the overall course of the disease. None of our cases suggests the possible evolution of pityriasis lichenoides into lymphomatoid papulosis. Although no infectious causative agent has been identified, a viral origin seems likely in some cases. Most patients responded favorably to UVB irradiation. Our conclusions are (1) that pityriasis lichenoides is probably a clinical disorder with a diverse etiology and (2) that its classification by distribution seems more useful than its subdivision into an acute and a chronic form.

Cutaneous manifestations of tetrachlorodibenzo-p-dioxin in children and adolescents: Follow-up 10 years after the Seveso, Italy, accident

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.

Multiple pilomatricomas in Rubinstein-Taybi syndrome. A case report

Abstract: Rubinstein‐Taybl syndrome is a multisystem developmental disordar due to an autosomal dominant mutation. It Is clinically defined by the presence of peculiar facles, mental retardation, and bread thumbs and first toes. Important dermatologic findings include hirsutism, keiolds, hemanglomas, and dermatoglyphic abnormalities. We report a 12‐year‐old girl with the typical phenotype of Rubinsteln‐Taybi syndrome, associated wtth numerous ptlomatrloomas. These are benign epithelial neoplasms with hair eel differentiation that may have a familial transmission. Pilomatricomas have not been reported in patterns with Rubinstem‐Taybl syndrome, although their association with myotonlc dystrophy, another autosomal dominant disorder, is well known. Possibilities to explain the association Include contiguous gens syndrome, the action of a piefotropic gene, predisposition to malformations, and mere coincidence.