Response of epidermolysis bullosa fibroblasts to factors derived from macrophages and polymorphonuclear leukocytes in terms of collagenase production.

In order to investigate the role of inflammatory cells in altering the collagenase production by epidermolysis bullosa (EB) fibroblasts, macrophage and polymorphonuclear leukocyte (PMN) factors obtained from mouse peritoneal fluids were added to the fibroblast culture system, and collagenase activity was assayed after a 48-h incubation. Data obtained here revealed that the response of collagenase production by fibroblasts was quite different, depending on the type of EB. Namely, EB dystrophica recessiva (EBDR) (n = 2) fibroblasts produced significant amounts of collagenase in the range of 5.07 (U/ml) to 6.04 in response to macrophage-conditioned medium, macrophage lysate, and PMN lysate, compared with 0.13 in the absence of these. On the other hand, EB dystrophica dominans (EBDD) (n = 1) fibroblasts showed little or no overt increase in enzyme production in the presence of macrophage lysate and PMN lysate, which resulted in a moderate increase to 3.82 in response to macrophage-conditioned medium. Furthermore, EB simplex (EBS) (n = 1) fibroblasts produced collagenase up to 3.84 in response to these three factors. These factors can be inactivated by treating with trypsin, pronase, and phenylglyoxal. Our data clearly indicated that, in the comparisons of EBDD and EBS fibroblasts, EBDR fibroblasts showed quite high response to factors derived from macrophages and PMNs in terms of collagenase production. This fact may raise a clue that accounts for the high levels of tissue collagenase activity, which plays a potentially major role in blister formation in EBDR.

[The treatment of psoriasis. Critical study of the use of ACNU (nimustine hydrochloride)]. / Traitement du psoriasis. Etude critique de l'utilisation de l'ACNU (nimustine hydrochloride).

The present study deals with the results of topical treatment of psoriasis with ACNU (nimustine hydrochloride), a water-soluble derivative of nitrosourea developed in Japan. Solutions at concentrations of 0.1 p. 100, 0.2 p. 100 and 0.4 p. 100 were obtained by dissolving ACNU in water and alcohol. An 0.4 p. 100 ointment was prepared by mixing ACNU with polyethylene glycol. Seventeen patients (12 male and 5 female), ranging in age from 19 to 68 years, were treated. Improvement of the skin lesions was observed in all 10 patients treated with the solution and in 6 of the 7 patients treated with the ointment. Clinical and histological pictures of the 4 main cases are shown with photographs. Compared with other treatments, the ACNU solution and ointment have the same effect as corticosteroid ointments of moderate strength or Goeckerman therapy. Side-effects included papules, redness, pigmentation and irritation. ACNU has been used for the treatment of mycosis fungoides, but we were the first to use this drug in psoriasis. This treatment has some side-effects, but they are less severe than those of other anti-cancer drugs. ACNU is relatively expensive and unsuitable for long term use. Thus, topical applications of ACNU may be of some value in cases where conventional treatments have failed.

Eccrine syringofibroadenomatous hyperplasia in a patient with bullous pemphigoid: a case report and review of the literature.

A 76-year-old man with multiple erythematous lesions on his palms and soles which appeared following bullous pemphigoid (BP) is the subject of this case report. The lesions were not raised above the normal skin level, and there were no nodules on the erythematous lesions. The lesions had the histologic appearance of eccrine syringofibroadenoma. This condition is considered to be not a true tumor but a hyperplasia of eccrine sweat ducts following recurrent subepidermal blister formation in BP.

Genetic counseling for skin diseases. A statistical analysis of 182 cases.

A statistical study was carried out on 182 cases who seeked out genetic counseling about skin diseases. The size of the latent demand for the counseling was assessed to be as much as 3.0% of the outpatients. Most clients were in their twenties. A representative consultand was the patient's child or sibling. The main diseases in question were cleft lip and palate (16%), nevus pigmentosus (12%), Recklinghausen's diseases (8%) and psoriasis (8%). Estimation of the recurrence risk was impossible in 3%, approximate in 54% and accurate in 43%. This defective estimation is due to the insufficiency of the available genetic data, e.g. carrier frequency, penetrance rate, contraction rate by age, etc. The high-risk cases were found to be 36%. In 9 cases (5% of all the counselings) the client decided to avoid reproduction. In 30 cases (16%), the clients asked for counseling after having conceived or given birth to a consultand. They were thought to be too late from the standpoint of prophylaxis of inherited diseases. Our conclusion is that genetic counseling offers some measure of disease prevention and therefore will become an essential branch or dermatology in the future.