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Disseminated superficial actinic porokeratosis. Analysis of an affected family.

A family is analysed in which disseminated superficial actinic porokeratosis (DSAP) occurred in five members. All three children of one generation are affected. Clinical, histopathological and genetic aspects of DSAP are discussed. Disseminated superficial actinic porokeratosis appears to be a clinical variant of porokeratosis of Mibelli. Since DSAP is a genodermatosis inherited as an autosomal dominant it can occur in any geographical location; however excess sunlight can definitely exacerbate the condition.

Adult↗

DNA synthesis and mitosis in uninvolved epidermis of persistent palmoplantar pustulosis.

Mitotic and DNA synthesizing cell counts have been performed in uninvolved epidermis of twenty-one patients with persistent palmoplantar pustulosis (PPP). There was no difference in mitotic counts and DNA synthesis in PPP compared with normal epidermis, but both were significantly lower than those found in the clinically uninvolved epidermis of patients with psoriasis.

Aged↗

Oral methoxsalen photochemotherapy of recalcitrant dermatoses of the palms and soles.

PUVA therapy successfully cleared various dermatoses mainly confined to the palms and soles in 18 of 20 patients treated. The conditions treated were: plaque-type psoriasis, pustular psoriasis, endogenous eczema and persistent palmoplantar pustulosis. Seventeen patients were treated in a controlled study of PUVA therapy versus no treatment at all and in 16 of these patients the disease was cleared in the PUVA-treated areas while the untreated areas remained unchanged or deteriorated. Twelve of the 18 patients were maintained in a clear state by continued maintenance PUVA treatment over 6--31 months while 3 patients had a spontaneous remission and are free of disease off all treatment.

Adolescent↗

A controlled trial of photochemotherapy for persistent palmoplantar pustulosis.

Twenty-two patients with bilaterally symmetrical persistent palmoplantar pustulosis were treated on one randomly selected side with oral psoralen and long-wave ultraviolet light. The treated side cleared completely in twelve patients, almost cleared in five patients and improved in four. One patient improved on both sides. Fifteen of the twenty-two patients were then treated with topical psoralen and long-wave ultraviolet light on the side that had previously been used as a control. Similar results were obtained. Seven patients cleared completely, six patients were much improved and two were improved. These results are significantly different from those liable to occur by chance (P < 0.001).

Administration, Oral↗

Assessment of anti-stratum corneum antibody titres in pustulosis palmaris et plantaris.

Titres of anti-stratum corneum (SC) antibodies, determined by an indirect immunofluorescence (IF) technique, were significantly higher on average in pustulosis palmaris et plantaris (PPP) and pompholyx than in normal controls. By a complement IF technique, the antibodies fixed complement (C3) in high titer in PPP, but to a much lesser degree in pompholyx and normal controls. Direct IF microscopy studies showed the deposition of IgG and C3 in the SC in some of the biopsy specimens of the pustular lesions. These findings suggest that in PPP anti-SC antibodies may be involved at least in part in activation of the complement system and the subsequent accumulation of polymorphonuclear leukocytes.

Antibodies↗

Clinical features of superficial fungal infections caused by Hendersonula toruloidea and Scytalidium hyalinum.

The clinical appearances of infections in 128 patients caused by the mould fungi Hendersonula toruloidea (102) and Scytalidium hyalinum (37) are described. All the patients originated from the tropics or subtropics but those infected with S. hyalinum came from either the Caribbean or West Africa. Forty-one per cent of those studied had a concurrent dermatophyte infection. While the clinical features of Hendersonula and Scytalidium infections resembled those seen in 'dry type' Trichophyton rubrum infections, there were a number of distinctive features. These included the absence of dorsal infection on the feet, lateral and distal onychomycosis with extensive onycholysis and the development of paronychia on the fingers. The majority of affected patients showed no serious underlying abnormality or predisposing skin disease which could facilitate invasion. The routine incorporation of cycloheximide, which inhibits the growth of these organisms, in mycological media may explain why they are infrequently diagnosed.

Adult↗

A comparison of PUVA-etretinate and PUVA-placebo for palmoplantar pustular psoriasis.

Seventeen patients with palmoplantar pustular psoriasis and three with hyperkeratotic psoriasis of palms and soles were treated with either PUVA-etretinate (1 mg/kg) or PUVA-placebo. Patients were randomly allocated to each group and the trial was conducted according to a double-blind protocol, so far as the side-effects of etretinate made this possible. PUVA was given three times a week for a maximum of 18 weeks, after 2 weeks on daily placebo or etretinate alone. All ten patients in the PUVA-etretinate group cleared, but there were four failures in the PUVA-placebo group (P = 0.03). The PUVA-etretinate treated patients required significantly fewer PUVA treatments (13.1 +/- 2.9; mean +/- s.e.) and cleared in a significantly shorter time (30.3 +/- 7.1 days) than the PUVA-placebo group (23.2 +/- 4.2 treatments; 59.2 +/- 11.5 days, P less than 0.05). The cumulative UV-A dose to clear was less in the PUVA-etretinate group (53.9 +/- 18.5 J/cm2) than the PUVA-placebo group (113.1 +/- 33.4 J/cm2). This difference was not significant due to the exceptionally large dose of UV-A used on one patient but the results were significant when it was excluded. The therapeutic advantage of adding etretinate to PUVA is offset by the side-effects of cheilitis, hair loss and peeling skin which occurred in eight of the ten PUVA-etretinate patients, and an increase in fasting triglyceride concentrations and serum alkaline phosphatase activity.

Etretinate↗

Acute palmoplantar pustulosis.

This paper describes five cases of an uncommon acral rash, acute palmoplantar pustulosis, which is precipitated by infection, and may represent a true pustular bacteria. Acute and chronic palmoplantar pustulosis are probably different forms of the same disease, but the prognosis in the acute form is good and aggressive therapy is not indicated. The relationship to psoriasis is discussed.

Acute Disease↗

Alterations of surface receptors on intralesional neutrophils in pustular psoriasis and palmo-plantar pustulosis.

Alterations of binding capacity of surface IgG-Fc and complement receptors were demonstrated in polymorphonuclear neutrophils (PMNs) obtained from the pustular lesions of psoriasis. A marked decrease of C3 receptors, but not of IgG-Fc, was found in PMNs from the lesions of palmo-plantar pustulosis (PPP) and bacterial pustules. PMNs from pustular lesions of psoriasis exhibited only a slight decrease in the number of C3 receptors. No significant decrease in membrane receptors was noted in circulating PMNs from psoriatic patients. We suggest that mechanisms of formation of aseptic subcorneal pustules, mediated by PMN membrane receptors for C3 fragments, are different in pustular psoriasis and PPP.

Bacterial Infections↗

The effect of etretinate compared with different regimens of PUVA in the treatment of persistent palmoplantar pustulosis.

Eighty-four patients with persistent palmoplantar pustulosis (PPP) of long duration were treated with either etretinate or one of three PUVA regimens. PUVA was given either with oral methoxsalen (thirteen cases), with a 1% methoxsalen cream (thirty-three cases) or with trioxsalen baths (eighteen cases). Twenty patients were treated with etretinate. Patients were assessed every fourth week. A mean score for each group was calculated at each visit based on erythema, desquamation, induration and pustulation. In addition, the number of pustules was calculated at each visit. After 12 weeks four of twenty-eight patients treated with local methoxsalen and fourteen of seventeen patients treated with etretinate had completely cleared. At this stage no patient treated with local trioxsalen or oral methoxsalen showed complete clearance.

Chronic Disease↗

The prevalence of accentuated palmoplantar markings and keratosis pilaris in atopic dermatitis, autosomal dominant ichthyosis and control dermatological patients.

The prevalence of keratosis pilaris and accentuated palmoplantar marking was evaluated in 61 patients with atopic dermatitis, 35 patients with dominant ichthyosis vulgaris and 247 other dermatological cases taken as controls. Our data showed that (1) these features are of no diagnostic significance for atopic dermatitis and (2) they are significantly more frequent in patients with ichthyosis vulgaris without associated eczema than in those with atopic dermatitis. Consequently, they should be considered as part of the phenotype of ichthyosis vulgaris rather than attributed to a concomitant atopic dermatitis as suggested by some. These findings should be taken into account when evaluating atopic dermatitis or ichthyosis. To assess the frequency of scaling under winter weather conditions, 155 control subjects were also examined for evidence of visible desquamation and 25.8% showed slight but definite scaling.

Adolescent↗

Neutrophil and monocyte chemotaxis in pustulosis palmo-plantaris and pustular psoriasis.

Polymorphonuclear leukocyte (PMN) and monocyte (MN) chemotaxis in nine patients with pustulosis palmo-plantaris (PPP) and ten patients with pustular psoriasis (PP) was determined by an objective in vitro assay employing a 51Cr-labelling technique. PMN chemotaxis was significantly enhanced in both groups of patients compared with controls. MN chemotaxis was normal. There was no difference in the chemotactic responsiveness of leukocytes from patients with PPP and PP. The random migration of PMN and MN from the patients was normal. Homogenized tissue specimens from lesional skin with and without pustules, and from perilesional, normal-looking skin of PPP and PP were analysed for the presence of chemoattractant(s) for PMN. Lesional skin had considerable chemoattractant properties, but perilesional skin did not induce directed migration of PMNs.

Adult↗

Etretinate in pustular psoriasis of palms and soles.

In a double-blind controlled study of patients with pustular psoriasis of palms and soles who were allocated at random to etretinate or placebo, we found that etretinate improved the condition as assessed by pustule count and overall clinical response. Side-effects occurred but were accepted by the patients in the short-term. The clinical usefulness of etretinate in this condition will depend on time to relapse, and whether this can be prevented or postponed by continuous treatment. Toxicity in the long-term will also be important.

Adult↗

Demonstration of anaphylatoxins C3a, C4a and C5a in the scales of psoriasis and inflammatory pustular dermatoses.

Complement components C3a, C4a and C5a were assayed in corneal scale extracts from psoriasis and other dermatoses characterized by sterile subcorneal pustules, using radioimmunoassay. Larger amounts were detected in psoriasis and related pustular dermatoses than in extracts of non-inflammatory stratum corneum. It is concluded that complement is activated via the classical pathway and releases the neutrophil chemotactic fragment C5a.

Anaphylatoxins↗

A new device for the treatment of hyperhidrosis by iontophoresis.

A new device for the treatment of hyperhidrosis by iontophoresis is described. Twenty-five patients have so far been treated, six with hyperhidrosis of the palms, 13 with hyperhidrosis of the soles and six with axillary hyperhidrosis. In 21 cases there was an excellent result. The effect of the treatment usually lasted for several weeks. Maintenance treatment every 4-6 weeks was found to be required.

Adult↗