A double-blind study of prazosin in the treatment of Raynaud's phenomenon in scleroderma.

Nineteen patients with Raynaud's phenomenon in conjunction with progressive systemic sclerosis were given either prazosin hydrochloride (1 mg orally three times a day) or a placebo for eight weeks, after which the treatment procedure was reversed for four weeks. Prazosin was shown to be effective in reducing both the frequency and the severity of vasospasm reported by the patients.

Intra-arterial reserpine for Raynaud's syndrome. Systemic reactions without therapeutic benefit.

Twenty-four patients classified as having Raynaud's disease or Raynaud's phenomenon were given bilateral brachial artery injections of reserpine or saline in a double-blind fashion. In the six weeks following injection, there was no indication that reserpine produced clinical improvement or changed vasomotor reactivity in the treated patients. However, intra-arterial reserpine did produce systemic cardiovascular effects lasting up to six weeks. It is concluded that intra-arterial reserpine as used in this study is an ineffective treatment for Raynaud's disease or Raynaud's phenomenon and may have significant adverse effects.

Serum triglyceride elevation following high-dose vitamin A treatment for pityriasis rubra pilaris.

We describe two patients with pityriasis rubra pilaris in whom hypertriglyceridemia developed following the oral administration of high-dose vitamin A (retinol). The effects of transient hypertriglyceridemia are unknown, as both patients tolerated high-dose vitamin A therapy with minor, if any, other side effects. Both patients had minimal clinical improvement with this therapy. Patients being treated with 1,000,000 IU of vitamin A each day for ten to 14 days should have frequent determinations of serum lipid levels and liver function during the period of drug administration.

Neuroendocrine response to cold in Raynaud's syndrome.

Eleven patients with Raynaud's syndrome accompanied by monospecific IgG ANA, nine patients with Raynaud's syndrome in the absence of ANA, and nine normal volunteers were exposed to an ambient cold challenge during which time venous blood was continuously sampled. ANA negative patients were shown to have significantly higher levels of cortisol during a cold challenge than either ANA positive patients or normal controls, and exhibited significantly lower levels of plasma norepinephrine compared with normal controls. ANA positive patients did not differ significantly from normals in their neuroendocrine response to cold. It is suggested that the high plasma cortisol found in Raynaud's syndrome in the absence of ANA may be responsible for the vasospasticity in this group of patients.

Correlation of epidermal plasminogen activator activity with disease activity in psoriasis.

The specific activity of plasminogen activator was increased in clinically involved psoriatic epidermis compared with the uninvolved skin of the same eight patients. Alterations in plasminogen activator activity correlated with disease activity as measured by extent of body surface involved with psoriasis. Levels of plasminogen activator increased in uninvolved epidermis of psoriatic patients during retinoid therapy.

Delayed onset of bullous pemphigoid after PUVA and sunlight treatment of psoriasis.

The coexistence of bullous pemphigoid and psoriasis has previously been noted. In most patients, the bullous lesions start during PUVA or UVB therapy of psoriasis. We report on a psoriatic patient who developed bullous pemphigoid approximately three to four weeks after discontinuing PUVA treatment, and then three to four weeks after exposure to natural sunlight. Delayed induction of bullous pemphigoid has not been previously reported, and it raises the question of ultraviolet therapy inducing subclinical or latent bullous pemphigoid in psoriatic patients.

Leiomyosarcoma of the skin and subcutaneous tissue.

Leiomyosarcomas arising in the skin and subcutaneous tissue are rare tumors, and diagnosis usually is made by microscopic examination of the lesion. These lesions typically appear as a single, smooth nodule in the skin but may also occur as multiple nodules with varying surface changes. After local excision, these lesions recur in a large proportion of patients. However, metastatic spread that leads to death seems to occur only in patients with the subcutaneous variety of the tumor. Three patients had varied clinical appearances of leiomyosarcomas. In one case, which was available for long-term evaluation, no recurrence was noted six years after surgical excision of a single lesion.

The effect of 13-cis retinoic acid on epidermal lysosomal hydrolase activity in Darier's disease and pityriasis rubra pilaris.

Five patients with Darier's disease and 6 patients with pityriasis rubra pilaris were treated with 13-cis-retinoic acid. Extracts of separated epidermis were assayed for extractable protein, lactic dehydrogenase, Cathepsin D, beta glucuronidase and neutral proteinase before beginning therapy and 2, 4 and 8 weeks after therapy had begun. The epidermal extracts from patients with pityriasis rubra pilaris before beginning therapy were similar to extracts from normal control patients. During the course of therapy with 13-cis-retinoic acid, protein extractability, lactic dehydrogenase and neutral proteinase did not change; there was a highly significant decrease in the specific activity of the lysosomal hydrolases Cathepsin D and beta glucuronidase. A similar but less dramatic fall was noted in the Darier's patients taking 13-cis-retinoic acid. Darier's patients also had a decrease in neutral proteinase activity before beginning therapy; the specific activity of this enzyme increased during the course of therapy. 13-cis-retinoic acid does not induce clinical remission by increasing the intracellular concentration of lysosomal enzymes in epidermis in vivo.

Evaluation of 13-cis retinoic acid in lamellar ichthyosis, pityriasis rubra pilaris and Darier's disease.

A new synthetic oral retinoid, 13-cis retinoic acid, is fairly well tolerated in patients and appears to be effective in those with Darier's disease and lamellar ichthyosis. It is less effective in those with pityriasis rubra pilaris. The mechanism of action of 13-cis retinoic acid in disorders of keratinization is unknown at the present time; however, it does not appear to cause lysosomal proliferation in therapeutic doses.

Pilomatricomas associated with myotonic dystrophy.

A report of the ninth and tenth cases of myotonic dystrophy associated with pilomatricomas and a review of the previously reported eight cases showed that seven of the ten patients had multiple pilomatricomas. Six of ten patients had another relative with both myotonic dystrophy and pilomatricomas. Patients with both myotonic dystrophy and pilomatricomas are more likely to have multiple tumors and to have a familial occurrence. The prevalence of pilomatricomas in patients with myotonic dystrophy is higher than in the general population. Pilomatricomas may be a cutaneous manifestation of the myotonic syndrome.