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J F Stalder

Publications and source records attributed to J F Stalder.

At least 37 records · Page 2Linked to original sources

Clinical validation and guidelines for the SCORAD index: consensus report of the European Task Force on Atopic Dermatitis.

BACKGROUND: We have previously reported how the SCORAD index was designed. This cumulative index combines objective (extent and intensity of lesions) and subjective (daytime pruritus and sleep loss) criteria. AIMS: To study interobserver variability in scoring for objective SCORAD criteria and to optimize the scoring guidelines. MATERIAL AND METHODS: Three scoring sessions were organized in 1993-1994 in Hamburg, Bordeaux and Rotterdam totalizing 19 patients (14 children and 5 adults) and 23 physicians, among whom 12 participated in at least 2 scoring sessions; 169 evaluation sheets have been processed using the SCORAD File Marker Pro software. At each session, total body photographs and close-up views were taken of each patient, and this material was reviewed at the final evaluation. RESULTS: The extent of lesions according to the rule of nines showed interobserver variability mostly for patients with lesions of moderate intensity involving 20-60% of body surface. Intensity items were scored with more consistency overall, but variations subsided especially for oozing/crusts and lichenifications. Low and high scorer profiles and the benefit of training were noted. CONCLUSIONS: This study has allowed to optimize clinical scoring using the SCORAD system. A proposal has been made to grade the severity of atopic dermatitis according to objective criteria in three groups for inclusion in clinical trials. The SCORAD index remains the major criterion for follow-up in trials.

Adolescent↗

[Kasabach-Merritt syndrome of the leg associated with osteolysis or Gorham sign].

INTRODUCTION: Kasabach-Merritt syndrome and Gorham's sign are two uncommon and severe, sometimes life-threatening, complications in infants with vascular lesions. Their association has been described in rare cases. CASE REPORT: An infant with a vast congenital angiomatous lesion including an extensive lymphatic component, developed active regional osteolysis then suddenly suffered disseminated intravascular coagulation of the leg. Medical treatment was unsatisfactory. After unsuccessful use of low molecular weight heparin, pentoxifyllin and alpha interferon, amputation of the leg was required to avoid a fatal outcome. DISCUSSION: Kasabach-Merritt syndrome does not develop on classic immature hemangiomas, despite some contradictory statements in the literature. In our case, a complex tumor developed in association with a lymphatic malformation. The association of Kasabach-Merritt syndrome with osteolysis (Gorham's sign) does not appear to be fortuitous. Therapeutic management of these severe complications is difficult and requires case by case analysis.

Disarticulation↗

[Cutaneous and neurologic vasculitis disclosing EBV-selective immunodeficiency].

INTRODUCTION: Purtilo's syndrome or X-linked lymphoproliferative syndrome (XLP) is a rare genetic disorder affecting boys who have a selective immunodeficit towards Epstein Barr Virus (EBV) and who develop extremely severe forms of EBV infection, of which there are four major types: severe or fatal infectious mononucleosis (60 p. 100), lymphoma (23 p. 100), acquired hypo- or agamaglobulinemia (25 p. 100) and anemia or pancytopenia. We report a case of vasculitis (cutaneous and neurologic) which led to the discovery of a selective immunodeficit towards EBV, similar to Purtilo's syndrome. CASE REPORT: A 17 year-old male with no significant past medical history presented with an eruption initially felt to be consistent with pityriasis lichenoid. Treatment with erythromycin was initiated, this did not prevent the subsequent eruptions of cutaneous vasculitis lesions which were severe, prolonged, debilitating, and associated with fever and general deterioration of the patient condition. All etiologic studies were negative. A course of systemic corticosteroids was begun, but the cutaneous eruptions persisted; and in addition the patient developed signs of polyneuropathy in the lower extremities secondary to neurologic vasculitic lesions. New studies revealed an abnormal EBV serology (absence of anti-EBNA antibodies) as well as hypogammaglobulinemia, suggestive of a selective immunodeficit towards EBV resembling Purtilo's syndrome. DISCUSSION: In our patient, the development of an extensive vasculitis, characterized histologically by an intense lymphocytic infiltrate, positive for EBV, associated with hypogammaglobulinemia, and with abnormal serology suggests an anomaly in the immune response to EBV. Although the age of the patient and absence of family history make the Purtilo's syndrome uncertain, the nature of the immunodeficit is very similar and the patient could well develop a lymphoma. This case is significant in that the disease initially manifested itself as a cutaneous vasculitis, which was not been described previously.

Adolescent↗

Infantile acropustulosis in six immigrant children.

Infantile acropustulosis is a recurrent, pruriginous, vesiculopustular eruption of the palms and soles first described in 1979. We report six cases of infantile acropustulosis in recently emigrated children treated for scabies. Clinical follow-up was obtained by questionnaire addressed to patients' families and general practitioners. Our study suggests infantile acropustulosis is frequent in immigrant infants and could be a non-specific hypersensitivity reaction to Sarcoptes scabiei.

Animals↗

[Two-stage excision of extensive cutaneous carcinoma of the face. A retrospective study of 80 patients].

INTRODUCTION: Delayed reconstruction (two-step surgery) comprised of tumor resection and repair three or four days later, after paraffin tissue fixation and histopathological examination, is one of the techniques used for complete excision of spreading cutaneous carcinomas. PATIENTS AND METHODS: In order to evaluate the efficacy and long term prognosis of delayed reconstruction surgery, we carried out a retrospective study of 80 patients treated by this method and (Kaplan- Meier) for correlation with the local recurrence rate: sex, age, anatomic site, histologic type and existence of previous therapy. Long term results have been estimated. Among the 80 patients studied, 76 patients have been followed. The sex-ratio was 0.48. Fifty-two percent of the population was more than seventy years old. Fifty-five percent of the tumors were situated on the nose. In 32,5 p. 100 of the cases, a prior therapy (surgery, electrodessication or radiation therapy) has been carried out. Histological analysis showed 87 p. 100 basal cell carcinomas (of which 26 p. 100 of morpheaform basal cell carcinomas) and 13 p. 100 squamous cell carcinomas. RESULTS: After the first operative step, there was total excision in 69 p. 100 of thecases. In the remaining cases, a second excision was performed with a margin of normal tissue. Recurrences were observed (some precocious recurrences and some late recurrences) in 18.4 p. 100 of the cases. No risk factor correlated significantly with the recurrence rate. However, analysis of the survival graph suggests that the squamous cell carcinomas recurred more often than the basal cell carcinomas. CONCLUSIONS: Although extemporaneous examinations (Mohs' technique) remains the gold standard technique in the management of spreading cutaneous carcinomas of the face, the delayed reconstruction (two-step surgery) is a credible alternative when compared to the recurrence rates reported in the literature with the other techniques (electrodessication, radiation therapy or cryosurgery).

Actuarial Analysis↗

Autosomal dominant Muckle-Wells syndrome associated with cystinuria, ichthyosis, and aphthosis in a four-generation family.

Muckle-Wells syndrome is a rare autosomal dominant disorder characterized by chronic recurrent urticaria, periodic arthritis, sensorineural deafness, general signs of inflammation, and secondary amyloidosis (AA type). We report on a 4-generation family with 7 persons sharing various signs of this syndrome associated with bipolar aphthosis in 5 cases and cystinuria in one. Two other relatives in the family had ichthyosis.

Adolescent↗

Local steroid therapy and bacterial skin flora in atopic dermatitis.

A double-blind, randomized trial was conducted to determine the influence of topical steroid therapy on atopic skin flora. The bacteriological and clinical effects of desonide (Locapred), compared with those of its excipient, were studied in 40 children. Clinical scoring and bacteriological sampling were performed before the start of the trial and after 7 days of once-daily topical treatment. Before treatment, no differences in clinical score or Staphylococcus aureus colonization were noted between the two groups. After treatment, the clinical score improved (P < 0.001) in the desonide group, and S. aureus density decreased dramatically (P < 0.001). In the excipient group, no significant differences in clinical score or S. aureus density were noted. A comparison of the two groups demonstrated statistically significant differences with regard to clinical score (P < 0.001) and S. aureus density (P < 0.05). These results show the efficacy of topical corticosteroid treatment alone on S. aureus colonization in atopic skin, and confirm the critical role of inflammation in bacterial colonization.

Adolescent↗

[Lichenoid eruption induced by gold salts. An autonomous eruption].

INTRODUCTION: We report a severe lichenoid drug eruption due to gold salts which relapsed 8 months after the cessation of chrysotherapy. CASE REPORT: A 56 year old man, 3 months after the beginning of a gold sodium propanol sulfonate therapy, developed a polymorphous eruption with violin papules on the trunk, eczematous lesions on the limbs and erosive stomatitis. Gold salts were definitively withdrawn. We saw the patient four months after gold therapy cessation: the eruption remained and diagnosis of severe drug cutaneo-mucous lichenoid eruption was done. We saw thereafter the patient again, 8 months after gold therapy cessation: the eruption had relapsed, more intense. DISCUSSION: We suggest that lichenoid eruption, first caused by gold salts, has become autonomous.

Antirheumatic Agents↗

[Blue rubber bleb nevus].

Blue Rubber Bleb Naevus (BRBN) is characterized by the presence of bluish haemangiomas on the skin and in the digestive tract. We report a new familial case of this rare entity. Analysis of this female patient's pedigree confirmed that BRBN is transmitted as an autosomal dominant trait. In this particular case gastrointestinal haemangiomas appeared over a 4-year period. Although all treatments of this disease have proved disappointing on the skin as well as in the digestive tract, we insist on the need for a regular and prolonged surveillance.

Adult↗