An impressive therapeutic result of class III compression stockings in a patient with longstanding, extensive, combined leg ulcers.

BACKGROUND: Only a few publications are available in the literature concerning the treatment of venous leg ulcers by means of medical compression stockings. SUBJECT: A 59-year-old female patient with longstanding, extensive bilateral leg ulcers due to venous insufficiency, arteriolosclerosis and secondary lymphoedema, was treated successfully by flat-knitted medical compression stockings of compression-class III. CONCLUSION: The great advantage of this treatment is the fact that it is comfortable and easy to carry out at home. Furthermore, the costs of compression therapy with medical compression stockings are only a fraction of those of conventional therapy by means of compressive non-elastic bandages. Once or twice weekly visits of the patient to the hospital are not necessary and less specialized dermatological nurses are needed.

Familial primary cryofibrinogenemia.

BACKGROUND: To our best knowledge this is the second case ever described of familial primary cryofibrinogenemia (CFG). PATIENTS: A 29-year-old Moroccan female and two of her three children suffered from painful purpura, slow healing small ulcerations and edema of both feet during the winter season. Laboratory investigations revealed the presence of cryofibrinogen in their blood plasma. All three patients were otherwise healthy and no associated disease could be demonstrated. CONCLUSIONS: The diagnosis of CFG has to be considered in patients with livedo reticularis, edema, painful purpura and slow healing ulcera after cold exposure. Cryofibrinogen-precipitates in the blood plasma have to be determined. Because secondary CFG occurs much more frequently than the primary form, it is important to rule out associated diseases through extensive physical examination and laboratory investigations. This communication also stresses the importance of a through family history of patients with CFG. An autosomal dominant mode of inheritance is supposed.

[D-penicillamine in treatment of scleroderma "en coup de sabre"]. / D-Penizillamin in der Behandlung von Sklerodermie "en coup de sabre".

A ten year old boy with linear erythema above the right eyebrow and a groove in the underlying skull bone is described. Histological examination of a biopsy revealed a spotty, periadnexal, perivascular, lymphocytic infiltrate. The papillary dermis was almost absent and the collagen fibres were slightly thickened. Based on the clinical appearance and the histology a diagnosis of linear scleroderma (en coup de sabre) was made. As the signs and symptoms were progressive, treatment with D-penicillamine was started. Subsequently the erythema disappeared. The shallow groove in the skull bone remained palpable. After thirteen months, treatment was stopped. The patient is currently free of signs and symptoms after a follow-up period of three and a half years. We feel that the strong clinical improvement is due to treatment with D-penicillamine rather than to the natural course of the disease.

[Parasitic pruritus: bird mite zoonosis]. / Prurigo parasitaria: epizoönose door vogelmijten.

In three women with persistent pruritus, aged 49, 28 and 4 years, infestation with the bird or chicken mite (Dermanyssus gallinae) was demonstrated. These mites live in narrow openings and cracks close to the bird housing during the daytime. At night they attack the birds on whose blood they live. When their host disappears, they may attack men, notably when their breeding places are in or near houses. Mite bites result in urticarial and itchy papulovesicular skin eruptions. Treatment of bird mite infestation consists of removing the old nests. Treatment of the patients is symptomatic. Epizoonosis belongs in the differential diagnosis of pruritus; infestation with bird or chicken mites is one of the possibilities.

The value of dynamic hepatic scintigraphy and serum aminoterminal propeptide of type III procollagen for early detection of methotrexate-induced hepatic damage in psoriasis patients.

Oral methotrexate (MTX) is a highly effective drug for the treatment of severe psoriasis. A limitation of this treatment is its potential hepatotoxicity. In the present prospective study the value of dynamic hepatic scintigraphy (DHS) and serum aminoterminal propeptide of type III procollagen (PIIINP) were investigated as screening methods for early detection of MTX-induced hepatic damage. These relatively non-invasive procedures were compared with the liver biopsy classification, until now the gold standard to assess MTX-induced liver damage. Twenty-five MTX patients were studied. The mean cumulative MTX dose was 3.9 g (range 0.2-11.1 g). Twenty-one patients had a normal liver histology (grade I), three patients had steatosis (grade II), and one patient mild fibrosis (grade IIIA). Seven additional patients with non-MTX related hepatic cirrhosis were included as disease controls. DHS showed a clear-cut separation between the portal contribution of the MTX patients with grade I liver histology, and that of all other patients. A portal contribution larger than 52% was associated with a > 95% chance of normal liver histology. If this cut-off value had been used to postpone the liver biopsy, this would have resulted in at least a 55% reduction in the number of biopsies in patients with a normal liver histology. DHS appeared to be very promising as a screening test to differentiate between the presence or absence of MTX-induced hepatic damage, but appeared not suitable to grade the severity of hepatic damage. Although a global relationship was demonstrated between serum PIIINP concentration and hepatic damage, single measurements in individual patients were not reliable. The combination of PIIINP measurements with DHS had only a limited additional value above DHS alone. The present study indicates that DHS has great promise for the detection of early MTX-induced hepatic damage. Pending further studies, regular liver biopsies remain mandatory for the safe prolonged use of MTX in psoriasis patients.

Acrodermatitis continua of Hallopeau: response to combined treatment with acitretin and calcipotriol ointment.

Treatment of acrodermatitis continua of Hallopeau (ACH) is difficult and often disappointing. We describe a patient with an extensive ACH of all finger- and toetips, who was treated with acitretin combined with calcipotriol. A within-subject left/right comparison was carried out between calcipotriol ointment (50 micrograms/g) and the ointment base to investigate the additional value of calcipotriol above the ointment base. The side treated with calcipotriol as adjunct therapy showed an impressive improvement, well beyond the degree of improvement at the side treated with the ointment base only.

Prolonged treatment with oral retinoids in adults: no influence on the frequency and severity of spinal abnormalities.

It is generally accepted that the spine is the site of predilection for retinoid-induced skeletal abnormalities. However, the reported prevalence of skeletal problems varies widely. To investigate the frequency and severity of retinoid-induced spinal abnormalities, all records of patients who underwent spinal radiographs at the request of the department of dermatology between 1983 and 1993 were reviewed. This group of 135 patients comprised the total population of retinoid-treated patients and those patients who were investigated for possible future retinoid treatment. The mean treatment period in the total group was 30 months and the mean cumulative dose of retinoid was 31 g. In 50 patients the treatment period was > or = 24 months with 30 patients being treated for more than 48 months. Baseline radiographs were available from 26 patients and these were compared with the most recent X-rays during treatment. The mean treatment period in this 'prospective group' was 25 months and the mean cumulative dose of retinoid was 25 g. The prevalence of diffuse idiopathic skeletal hyperostosis (DISH), degenerative changes and osteoporosis in the total group was respectively 16%, 53% and 29%. There was no statistically significant relation between the duration of treatment or the cumulative dose and the prevalence or severity of DISH, degenerative changes and osteoporosis. Only the age of the patients was significantly related to the frequency and severity of skeletal abnormalities. In the 'prospective group', again, no important changes were observed between the radiographs at baseline and during treatment. In this study no relation whatsoever between spinal abnormalities and prolonged oral retinoid treatment could be established. The performance of annual routine spinal radiographs during retinoid treatment is not necessary in our opinion. Additional controlled and prospective studies on spinal and extraspinal skeletal abnormalities are required to develop definitive screening guidelines for patients submitted to long-term retinoid treatment.

Interruption of long-term methotrexate treatment in psoriasis. Evaluation of clinical course and laboratory parameters after discontinuation and reintroduction of weekly oral methotrexate.

The effects of interval treatment were evaluated in 10 psoriatic patients on long-term treatment with low-dose oral methotrexate (MTX). In all patients, the dosage of MTX had already been tapered off as much as possible. After interruption of MTX treatment, the clinical course and changes in laboratory parameters were evaluated. The mean MTX-free period was 17 weeks, and the mean reduction in cumulative MTX dose was 76 mg (p = 0.05). However, only 3 patients preferred interval treatment to a continuous schedule. During the first 3 weeks of discontinuation, a significant decrease in the serum transaminases was observed, indicating a direct toxic influence of MTX on the liver parenchym. We conclude that interruption of long-term MTX treatment leads to a substantial reduction of the cumulative MTX dose and reduces the hepatotoxic load of MTX. It is necessary to motivate patients on long-term MTX treatment for regular treatment interruptions to establish a further reduction in their cumulative MTX dose.

Extensive extraspinal hyperostoses after long-term oral retinoid treatment in a patient with pityriasis rubra pilaris.

We describe a patient with severe pityriasis rubra pilaris in whom extensive extraspinal hyperostoses developed after 13 years of oral retinoid treatment. The most prominent abnormality was a bridging exostosis between the left acetabulum and collum. X-ray examinations of the spine during retinoid therapy showed no abnormalities. During oral retinoid treatment, it is important to ask the patient on a regular basis about any skeletal pains or mobility restriction. Normal spinal x-ray results are no guarantee that a patient is free of hyperostoses. Discontinuation of acitretin therapy resulted in a severe exacerbation of the patient's pityriasis rubra pilaris after 2 weeks. The clinical response to administration of azathioprine was clearly inferior to that of acitretin. However, low-dose oral methotrexate therapy appeared to be a good alternative in this patient, with a clinical result comparable to acitretin and no side effects after 6 months of therapy.

Methotrexate revisited: effects of long-term treatment in psoriasis.

In the past 22 years, 113 patients with severe psoriasis have been treated with low-dose methotrexate (MTX) in our department. The maximum weekly dosage was 15 mg (Weinstein schedule), the estimated mean cumulative dose was 4803 mg, and the estimated mean duration of therapy was 8 years and 11 months. In 81% of the patients, prolonged clearance or near clearance was achieved, indicating the potent and sustained potential of MTX in the treatment of both the pustular and erythematosquamous variants of psoriasis. Eighty-three patients (73%) had side-effects, most frequently abnormal liver function tests, nausea and gastric complaints. Apart from hair loss in seven patients, there were no mucocutaneous side-effects, probably because of the low-dose treatment schedule. In 71 patients MTX therapy was discontinued; in 33 patients because of side-effects. In 55 patients one or more liver biopsies were performed. Fibrosis was seen in seven of these patients (13%) and cirrhosis in two (4%). There was no relation between liver biopsy classification and cumulative dosage or duration of MTX therapy, nor was there any relation between liver histology and abnormal liver function tests. During this 22-year period, there were no deaths or life-threatening side-effects attributable to MTX treatment. We conclude that low-dose MTX (< or = 15 mg/week) is a relatively safe therapy for severe psoriasis, if patients are carefully selected beforehand, and regular monitoring for side-effects and drug interactions is performed during therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Acitretin in the treatment of lamellar ichthyosis.

Etretinate and its metabolite acitretin have been shown to be highly effective in the treatment of various disorders of keratinization, including lamellar ichthyosis. The aim of the present study was to provide further information on acitretin dosage regimens in the management of lamellar ichthyosis. Seven patients with classical manifestations of lamellar ichthyosis participated in the study. Five patients improved markedly, and the remaining patients showed mild to moderate improvement. Two patients improved following gradual incremental increase in dosage (> or = 35 mg/day). Four patients, including a patient with the erythrodermic variant, required low-dose acitretin treatment (< or = 25 mg/day), as higher doses resulted in a marked deterioration in their skin condition. The dichotomy with respect to the response to acitretin suggests that lamellar ichthyosis is a spectrum of at least two conditions.

Ichthyosis bullosa of Siemens responds well to low-dosage oral retinoids.

Two patients with ichthyosis bullosa of Siemens (IBS) and one patient with bullous ichthyosiform erythroderma of Brocq (BIE) were treated with etretinate. Two additional patients with IBS received acitretin. All the patients had a marked improvement when on retinoids and the maintenance dose required was for IBS 10-25 mg per day. The patient with BIE was on a maintenance dose of 40-60 mg per day.

[Acitretin therapy in keratinization disorders]. / Acitretinetherapie bij verhoorningsstoornissen.

The introduction of the oral retinoid etretinate in the past decade has proved to be a major advance in the treatment of disorders of keratinization. During the past few years a new retinoid acitretin has been investigated in clinical trials. Acitretin has a half-life time of 2 days, versus 100 days for etretinate. Acitretin has therefore a great advantage with respect to the teratogenic potential of retinoids. The period of contraception after cessation of acitretin therapy is only two months, compared with two years for etretinate. From December 1989, etretinate has been replaced by acitretin in The Netherlands. In the Department of Dermatology of the University Hospital of Nijmegen, 36 patients with various disorders of keratinization were treated with acitretin. In this communication therapeutic results of acitretin therapy are reported. Clinical efficacy and side effects of acitretin are similar to those observed with etretinate.

Severe hepatotoxic reaction with progression to cirrhosis after use of a novel retinoid (acitretin).

We report the case of a 50-year-old female who suffered from severe palmar and plantar pustulosis. During treatment with acitretin, a novel oral retinoid, which is the main derivative of etretinate, the patient developed a severe hepatotoxic reaction. Subsequent histological studies strongly suggested the development of liver cirrhosis. Reversible elevation of serum aminotransferase values during treatment with acitretin has been reported. However, the present observation indicates that severe hepatotoxic injury may also follow treatment with this agent.