Precautions and suggestions for pemphigus patients.

The onset and course of pemphigus are often the result of an interaction between predisposing genetic factors and environmental triggering agents. The latter are heterogeneous, numerous and increasing, ranging from drug intake (the commonest cause of pemphigus induction) to the exposure to physical agents (heat, UV and ionizing rays, surgical and cosmetic procedures), viral infections (especially by herpesvirus), contact dermatitis, certain diet ingredients and even emotional stress. Alerting physicians and pemphigus patients to the effects that unsuspected precipitating factors may have on the progression of the disease is an important task. In fact, avoiding or limiting deleterious habits (e.g. overindulging in unnecessary drugs) and suggesting alternative ways (e.g. substituting potentially pemphigus-inducing drugs with others considered harmless in this respect) may be a useful precaution in the management of pemphigus patients, since it can improve the efficacy of conventional treatments, reduce the risks of relapses and sometimes result in a cure.

Type III Ehlers-Danlos syndrome: correlations among clinical signs, ultrasound, and histologic findings in a study of 35 cases.

BACKGROUND: Patients with type III Ehlers-Danlos syndrome show hypermobile joints, luxation, and minimal atrophic scars. The disease has an incidence of 9-16% and the molecular defect that underlies this subtype is unknown. In order to widen diagnostic investigations in type III Ehlers-Danlos syndrome, skin and articular areas were studied by ultrasound (US) test. METHODS: Thirty-five patients, 20 women and 15 men, aged from 18 to 25 years, with type III Ehlers-Danlos syndrome were enrolled in this study. Patients showed hypermobile joints (35 cases), minimal atrophic wrinkled scars (35 cases), and a few ice-pick acne scars. Hypermobility of phalangeal joints was studied by means of the Beighton scoring system and by a US test (performed by Sonora Logic 400 MD unit with 10 MHz probe). The following US parameters were considered: the distance of the intra-articular space and the thicknesses of the extensor tendon and of the overhanging dermis and subcutis. RESULT: US reports showed an increase in distance of the intra-articular space and a reduction in the thickness of the extensor tendon and of the dermis and subcutis covering it. The dermis showed high and homogeneous echogenicity with irregular hyperechogenic lines. CONCLUSION: The Beighton scoring system, the US findings, and the aspect and diameter of the scars suggested that the disease can be divided into three different stages which allow the correlation, over progressive intervals of values, of the articular hypermobility and atrophic wrinkled scars. Our results showed that US test is able to confirm the clinical diagnosis and to evaluate the seriousness of hypermobile joints.

In vivo enalapril-induced acantholysis.

Enalapril is a widely used antihypertensive drug with a very powerful in vitro acantholytic effect. It has been known to potentially induce pemphigus in genetically predisposed subjects. The action mechanism is complex and still only partially understood. We describe the case of a 66-year-old man, affected with intermediary basal cell carcinoma, in whom the histological examination showed suprabasal acantholytic clefts in the perilesional epidermis. Surprisingly a second biopsy taken from the apparently healthy skin of his back confirmed the presence of acantholytic changes. Clinical signs of pemphigus were absent. The patient's history did not reveal any relevant data but a mild arterial hypertension that had been treated for 1 year with 10 mg enalapril. Taking into account the patient's history (enalapril long-term administration), the absence of any bullous or erosive lesions and the histological findings, a diagnosis was made of in vivo enalapril-induced acantholysis.