[Erythema multiforme vs. Stevens-Johnson syndrome and toxic epidermal necrolysis: an important diagnostic distinction]. / Erythema multiforme versus Stevens-Johnson-syndroom en toxische epidermale necrolyse; belangrijk diagnostisch onderscheid.

Three patients, a girl aged 10 and two women aged 59 and 64 years, had erythema multiforme, Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), respectively. SJS and TEN are rare illnesses with a high morbidity and mortality. The aetiology is mainly iatrogenic: a hypersensitivity reaction to certain pharmaceutical prescriptions. SJS and TEN should be differentiated from the more frequent erythema multiforme, a self-limiting disease without important residual symptoms, which is usually initiated by infection with herpes simplex virus. SJS and TEN are variants in a spectrum of exfoliative dermatoses with epidermal necrosis. SJS and TEN on the one hand and erythema multiforme on the other can be distinguished on the basis of aetiology, clinical symptoms and histopathology. The distinction can, however, be difficult, notably in the early stages. The girl recovered completely. The first woman was treated with corticosteroids and also recovered; she was thought to have developed the syndrome as a reaction to malarial prophylactics. The third patient died, despite extensive treatment, of multiorgan failure and sloughing of 70% of the skin, probably as a reaction to amoxicillin given for pneumococcal pneumonia.

[Diagnostic image (152). A man with a swelling in the groin. Malignant eccrine poroma]. / Diagnose in beeld (152). Een man met een gezwel in de lies. Maligne eccrien poroom.

A malignant eccrine poroma (porocarcinoma) was excised from the right groin of a 78-year-old man.

No additional effect of calcipotriol ointment on low-dose narrow-band UVB phototherapy in psoriasis.

BACKGROUND: Broad-band UVB phototherapy has appeared to be effective in clearing psoriatic lesions. After the advent of calcipotriol ointment, promising results have been obtained by combining these two therapeutic modalities. Also, an additional effect of narrow-band UVB phototherapy on treatment with calcipotriol ointment has been demonstrated. OBJECTIVE: Our purpose was to compare treatment with low-dose narrow-band UVB phototherapy both with and without calcipotriol ointment. METHODS: We included 53 patients suffering from plaque-type psoriasis. All patients underwent low-dose narrow-band UVB phototherapy. Nearly half of the patients were randomized to apply calcipotriol ointment (50 microg/g) twice daily on the affected skin. The Psoriasis Area and Severity Index (PASI) was used to evaluate psoriatic lesions. RESULTS: In this study we showed that low-dose narrow-band UVB phototherapy is effective in the treatment of psoriasis and that calcipotriol ointment does not improve treatment outcome. CONCLUSION: Calcipotriol ointment does not improve treatment with low-dose narrow-band UVB phototherapy.

[A tropical ulcer; cutaneous diphtheria]. / Een ulcus uit de tropen; cutane difterie.

A 43-year-old woman, born in the Netherlands, developed ulcers on her left foot during a holiday in Gambia and Senegal. She had been bitten bij insects. The ulcers were caused by Corynebacterium diphtheriae. The patient was treated with antibiotics and recovered fully. A grey pseudomembrane covering the ulcer is a characteristic feature of cutaneous diphtheria. The treatment is with antibiotics and, after toxin tests have indicated that the bacterium is toxigenic, with antitoxin. In some cases screening of social contacts is advised.

Linear connective tissue naevus of the proteoglycan type ('naevus mucinosus').

We report a case of naevus mucinosus, a recently described condition. Clinically, the lesions presented as unilateral, multiple, firm, 3-5-mm-diameter, coalescent papules in a linear arrangement on the back of a 23-year-old man. Histologically, large amounts of acid mucopolysaccharides (proteoglycans) were demonstrated in the superficial dermis. As far as we are aware, this is the first report of the onset of naevus mucinosus in an adult. Naevus mucinosus appears to be a distinct type of connective tissue naevus which is characterized by an increase in proteoglycans.

Venous hypertension of the hand caused by hemodialysis shunt: immunofluorescence studies of pericapillary cuffs.

BACKGROUND: Venous hypertension of the hand is an unusual complication of an arteriovenous shunt in patients receiving dialysis. OBJECTIVE: We investigated whether in venous hypertension of the hand pericapillary cuffs are present and whether they consist of fibrin and other components. METHODS: Biopsy specimens were taken from three patients with a side-to-side shunt and venous hypertension of the hand, from three patients with an end-to-side shunt without venous hypertension, and from three control subjects. The specimens were stained with antibodies against fibrinogen, fibrin, plasminogen activator inhibitor-I, factor VIII-related antigen, and collagen type IV. RESULTS: Pericapillary cuffs consisting of fibrin only were observed in patients with venous hypertension of the hand. In this fibrin network, factor VIII-related antigen and collagen type IV were also present. The finding of plasminogen activator inhibitor-I in the pericapillary cuff in the patient with the most severe signs of venous hypertension may indicate that breakdown of this fibrin cuff is impaired. CONCLUSION: Pericapillary cuffs may be of pathogenetic significance in venous hypertension of the hand.

Immunofluorescence studies of atrophie blanche with antibodies against fibrinogen, fibrin, plasminogen activator inhibitor, factor VIII-related antigen, and collagen type IV.

In and around ulcers complicating the chronic venous insufficiency syndrome and atrophie blanche a pericapillary cuff of fibrinoid material has been described. The aim of the present study was to find out whether pericapillary cuffs are present in atrophie blanche ulcerations, whether they consist of fibrinogen and/or fibrin in comparison to normal controls, and whether this cuff is composed of other components. Skin biopsies from ten patients adjacent to atrophie blanche ulcers, and from ten controls were taken. In all patients pericapillary cuffs consisting of fibrin were found. However, no fibrinogen was found in these cuffs. In the controls no cuffs were found. In this fibrin network factor VIII-related antigen and collagen type IV were also present. The finding of plasminogen activator inhibitor-I in the pericapillary cuff in several cases may indicate that breakdown of this fibrin cuff is impaired. The possible diffusion barrier caused by the pericapillary cuff together with the pattern of vascularization may be an important event in ulcer formation and impaired ulcer healing.

Lack of exchange among plasma proteins in narrow spaces on glass, demonstrated with metal oxide coatings.

Onto 'activating' surfaces, intact normal plasma deposits an overlapping sequence of proteins, each being desorbed by the next. Ultimately, high molecular weight kininogen (HMK) is deposited unless contact was too short, or space between 2 surfaces too narrow. Thus, injected between a glass slide and a convex lens, intact plasma will leave a disk of HMK with a center of fibrinogen. We describe here how the exchange of proteins on the surface can be demonstrated by staining the adsorbate with a metal oxide suspension. Subsequent flooding of the preparation with more normal plasma causes lift-off of the oxide where underlying fibrinogen is being displaced by the HMK of the newly applied plasma. Kininogen-deficient plasma fails to remove any oxide, while normal plasma can remove nearly all of the oxide and adsorbate, left on glass by HMK-deficient plasma.