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Biomedical subjects

R Happle

Publications and source records attributed to R Happle.

At least 325 records · Page 18Linked to original sources

Antigenic competition as a therapeutic concept for alopecia areata.

Alopecia areata can be treated effectively by topical application of potent contact allergens. To explain the response, the following hypothesis is presented. Alopecia areata is considered an autoimmune disease. The characteristic peribulbar round cell infiltrates probably reflect a cell-mediated immune reaction to some hair-associated antigen. With the elicitation of contact allergy, a second antigen is introduced at the same site. The infiltrates of the allergic contact dermatitis contain suppressor T cells and suppressor macrophages which, in terms of local immunoregulation, exert a nonspecific inhibitory effect on the immune response against hair follicles. Regrowth of hair would be due to a change in the local balance between helper and suppressor cells. In conclusion, the phenomenon of antigenic competition is proposed as a therapeutic concept.

Alopecia Areata↗

The CHILD syndrome. Congenital hemidysplasia with ichthyosiform erythroderma and limb defects.

The term CHILD syndrome is proposed as an acronym for congenital hemidysplasia with ichthyosiform erythroderma and limb defects. The syndrome is characterized by unilateral erythema and scaling, with a distinct demarcation in the middle of the trunk. The dermatosis is either present at birth or develops during the first weeks of life. Ipsilateral limb defects may vary from hypoplasia of some fingers to complete absence of an extremity. In addition, ipsilateral hypoplasia of other parts of the skeleton, as well as defects of the brain and the viscera are found. In some cases, ipsilateral punctate epiphyseal calcifications have been observed. Two further cases of this syndrome are reported, and a review of 18 previous observations is presented. The ratio of females to males is 19 : 1. Apparently, the CHILD syndrome is genetically determined. Arguments are presented in favor of the hypothesis that the conditions is due to an X-linked dominant gene lethal in hemizygous males.

Abnormalities, Multiple↗

Contact allergy as a therapeutic tool for alopecia areata: application of squaric acid dibutylester.

Squaric acid dibutylester (SADBE), a potent contact allergen, was tested for mutagenicity in the bacterial plate incorporation assay (Ames test), in the presence and absence of mammalian microsomes. In contrast to dinitrochlorobenzene which is mutagenic in this test, SADBE was found not to be mutagenic. In 53 patients with extensive or total alopecia areata, SADBE dissolved in acetone was applied weekly to one side of the head, the other side serving as control. In 46 patients (87%), hair regrew either exclusively on the treated side, or considerably faster and denser on this side. In some patients, continuous treatment failed to maintain the response. Persistent response was observed in 37 patients (70%). These results are essentially the same as those obtained with DNCB. Therefore, contact allergy is proposed as a therapeutic concept for alopecia areata.

Adolescent↗

[X-linked dominant chondrodysplasia punctata (author's transl)].

Chondrodysplasia punctata displays genetic heterogeneity. The differentiation between the rhizomelic type and the Conradi-Hünermann type is well known. In 1977, an X-linked dominant form was described as a third type. The syndrome of X-linked dominant chondrodysplasia punctata includes skeletal, ocular and cutaneous anomalies with asymmetric involvement of the body. The cutaneous signs and symptoms are characteristic: congenital ichthyosiform erythroderma with linear and patchy hyperkeratoses; ichthyosis in the older child; linear and blotchy atrophoderma mainly involving the hair follicles; circumscribed alopecia; coarse, lusterless and irregularly twisted hair; sparse eyebrows and lashes that grow in various directions; flattened nail plates and onychoschizia. A further case of X-linked dominant chondrodysplasia punctata is reported. The ratio of females to males is so far 40:0. Apparently, the underlying gene defect is lethal in male embryos. The linear and patchy pattern of skin lesions reflects functional X-chromosome mosaicism.

Abnormalities, Multiple↗

[Pit-shaped enamel defects in tuberous sclerosis].

In 6 patients affected with tuberous sclerosis, pit-shaped enamel defects were noted. Apparently, these defects are pathognomonic of this syndrome and may help to establish the diagnosis in oligosymptomatic cases.

Dental Enamel↗

[DNCB therapy of alopecia areata].

Long-term treatment of alopecia areata with dinitrochlorobenzene is effective. During the last two and a half years, 227 patients who suffered, in the majority of cases, from total or subtotal hair loss, were treated by this method. Unilateral application of DNCB induced unilateral regrowth of hair in 88% of these patients. Under continuous treatment of both sides of the head, this initial response was followed by complete regrowth of hair in 78%. The same result could be obtained by application of squaric acid dibutylester, another potent contact allergen. This indicates that the essential mechanism is contact allergy. Possibly, the regrowth of hair is due to the induction of local nonspecific immunosuppression.

Alopecia Areata↗

[Advances in topical therapy of skin diseases (author's transl)].

The anti-inflammatory effect of the new topical corticosteroid fluocortin butyl ester is approximately equal to that of hydrocortisone acetate but it has the advantage that systemic side-effects are lacking. Vitamin A acid and benzoyl peroxide have brought significant advances in the topical treatment of acne. For the treatment of chloasma and other hyperpigmentations the combination of vitamin A acid and hydroquinone with a corticoid is considerably more effective than any of the single components alone. Povidone-iodine with its extraordinarily low sensitization rate has proved useful for external antimicrobial treatment. Extensive or multiple precancerous lesions are effectively treated with 5-fluorouracil. New hair growth can be induced in alopecia areata by the local application of DNCB.

Acne Vulgaris↗

X-linked dominant chondrodysplasia punctata. Review of literature and report of a case.

X-linked dominant chondrodysplasia punctata is a syndrome consisting of skeletal, ocular, and cutaneous anomalies with asymmetric involvement of the body. The skin lesions, the hallmark of this condition, are distributed in a linear or blotchy pattern and include congenital ichthyosiform erythroderma, systematized atrophoderma mainly involving the hair follicles, and circumscribed alopecia. The remaining scalp hair is in part normal and in part irregularly twisted and coarse. The eyebrows and lashes are sparse. The nails may be flattened and split into layers. Thirty-five cases of this new syndrome are reviewed, and an additional observation is reported. The ratio of females to males is 36:0. The concept of X-linked dominant chondrodysplasia punctata has been suggested, and it has been postulated that there is a connection between the mosaic phenotype and the limitation to the female sex. Both facts would be explained by an X-linked gene giving rise to a pattern of lyonization in females, and lethal in hemizygous males. The classification of chondrodysplasia punctata thus includes three forms: the rhizomelic type, the Conradi-Hünermann type, and the X-linked dominant type. Two of these, the rhizomelic type and the X-linked dominant type, are well-defined entities. Whether the Conradi-Hünermann type, after separation of the X-linked form, is still heterogeneous, remains to be determined.

Adolescent↗

Laboratory investigations in patients with generalized psoriasis under oral retinoid treatment. A multicenter study of computerized data.

Numerous laboratory parameters were examined 235 patients with generalized psoriasis treated orally with retinoid and in 35 patients treated topically with anthralin as control. Computer evaluation of the obtained data revealed statistical trends to elevation of the total serum bilirubin level and increasing number of blood monocytes after long-term oral treatment. No other statistically significant changes of the laboratory data were found. Particularly, the liver function tests (transaminases, prothrombin and alkaline phosphatase) showed no significant alterations. Only in a few cases did the retinoid compound have an influence on the GPT and GOT levels. The reasons for this individual sensitivity to the drug remain unknown. No significant alterations were found in the control group treated topically with anthralin.

Administration, Oral↗

[White nevus of the oral mucosa].

A white sponge nevus of the oral mucosa is described in a 12-year-old girl and her 36-year-old mother. This anomaly, which is inherited as an autosomal dominant trait, deserves no treatment. Because of the bilateral involvement, which is found in the majority of cases, the white sponge nevus is often misdiagnosed as therapy-resistant thrush.

Child↗

[Pregnancy and delivery combined with hereditory angioneurotic edema (author's transl)].

Two deliveries in a patient combined with hereditory angioneurotic edema are reported. Although even the smallest trauma can lead to life threatening edema the tendency to edema is reduced during pregnancy. Neither in the vagina nor the vulva the deliveries caused edema. The episiotomy did not cause a concomitant edema of the vulva, despite the predilection of the external genital organs for edema. It is possible that a correlation between the gonadotrophin level and the tendency to edema exists in hereditory angioneurotic edema.

Adult↗

[X-linked dominant chondrodysplasia punctata: an osteocutaneous syndrome].

A further case of X-linked dominant chondridysplasia punctata is described. This syndrome is characterized by the following cutaneous anomalies: congenital ichthyosiform erythroderma with thick, adherent hyperkeratoses; widespread atrophic skin lesions discernible after the first weeks of life; patchy alopecia; coarse and lusterless hair; onychoschisis. The hyperkeratoses of the newborn as well as the ensuing atrophoderma predominantly involve the hair follicles and are distributed in a bizarre linear or blotchy pattern. In some instances, a linear pattern of pigmentary disturbance has also been observed. These cutaneous signs and symptoms are so typical that the diagnosis of X-linked dominant chondrodysplasia punctata can be established even without X-ray examination. The syndrome has so far been observed exclusively in females. Apparently, the underlying X-linked gene defect is lethal in hemizygous males.

Child↗