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Ichthyosis follicularis with alopecia and photophobia.

We treated two unrelated boys with ichthyosis follicularis, a rare skin disorder characterized by extensive noninflammatory spiny follicular hyperkeratoses, severe photophobia, and generalized noncicatricial alopecia. This disorder must be differentiated from keratosis follicularis spinulosa decalvans; ulerythema ophryogenes; keratosis pilaris rubra atrophicans faciei; atrichia with papular lesions; atrophodermia vermiculata; and keratitis, ichthyosis, and deafness syndrome, all of which share some clinical features. Ichthyosis follicularis with alopecia and photophobia appears to be a familial disorder, but too few cases have been reported to establish the exact mode of inheritance.

Alopecia↗

Leukonychia punctata and pitted nails in alopecia areata.

A 36-year-old man with long-standing alopecia areata was noted to have both leukonychia punctata and pitted nails. Histopathologic evaluation of the leukonychia punctata revealed multiple discrete parakeratotic foci throughout the entire thickness of the nail plate, suggesting both proximal and distal matrix involvement. The finding of shallow pits lined with residual adherent parakeratotic cells suggests that the pathogenesis of nail pits in alopecia areata is similar to that of psoriasis; a result of shedding of parakeratotic cell aggregates from the nail plate.

Adult↗

Lichen sclerosus et atrophicus generalisata, alopecia areata, and polymyalgia rheumatica found in the same patient.

A sixty-eight year old woman with lichen sclerosus et atrophicus since the age of fity-two, alopecia totalis since the age of thirty-six, and polymyalgia rheumatica since the age of fifty-eight is presented. Besides alopecia totalis, the patient has typical lichen sclerosus et atrophicus changes on the trunk, upper arms, and thighs, and at the first visit an ulcer on her left breast. An immunologic link between her three diseases is suspected. As shown in this case Wood's light is of great value in depigmented skin changes.

Aged↗

[Alopecia in thyroid diseases: characteristic trichograms].

Trichograms from the parietal and occipital area were evaluated in 34 patients with thyroid diseases, which complained about hair loss. The followings findings can be regarded as characteristic for alopecia in thyroid diseases, being not related to the functional state of the thyroid gland: increase of dysplastic hairs over 50%, increase of broken hairs over 15% and strenghthening of these findings in the occipital area. Alopecia in thyroid diseases is not caused by changes within the hair cycle but probably by impaired hair quality.

Adult↗

Immunofluorescence studies on complement components in the hair follicles of normal scalp and of scalp affected by alopecia areata.

The deposition of complement components (Clq, C4 C3, C5 and C9) and properdin in scalp hair follicles was examined by the immunofluorescence method in patients with alopecia areata and in normal subjects. C3, C5 and C9 were found deposited there. The complement deposition was most frequent in the case of C3 and less frequent in C9 and C5. There was no difference regarding deposition between normal subjects and patients with alopecia areata. No deposition of Clq, C4 or properdin was observed in the hair follicles of the scalp. These findings suggest a relationship between the hair cycle and the activity of C3 and its late complement components in the scalp.

Adolescent↗

Immunohistochemical analysis of T-cell subsets in the peribulbar and intrabulbar infiltrates of alopecia areata.

In 11 patients with untreated alopecia areata in the progressive stage of the disease, an in situ analysis of the inflammatory infiltrate of the hair bulbs was performed by means of different monoclonal antibodies. Most of the peribulbar cells reacted with the pan T-cell antibodies OKT 3 and Lyt 3. Staining for T-cell subsets revealed that the proportion of OKT 4+ cells was about fourfold higher than that of OKT 8+ cells. Almost all of the T cells were OKIa1+, indicating that they were in an activated state. In four of the 11 cases, both subsets of T lymphocytes were also found to infiltrate the hair matrix itself. These results would appear to be consistent with the assumption that alopecia areata is caused by a T cell mediated autoimmune mechanism.

Alopecia Areata↗

[Anxiety as a state and anxiety as a personality trait in patients with alopecia areata, rosacea and lichen ruber planus].

Our study comprised the examination of 120 persons by psychological methods. Every 30 of them were patients with alopecia areata, rosacea, lichen ruber planus, and healthy skin. The results revealed that patients suffering from skin diseases may be characterized by different anxiety conditions. Patients with rosacea turned out to possess a high level of anxiety trait: accordingly, their reactions towards the disease showed a higher level of anxiety state than could be observed in alopecia areata or lichen ruber planus patients. With regard to rosacea etiopathogenesis, psychogenic factors seem to play a special part if compared to the other diseases. In most of the cases, traumatogenic situations preceded the appearance of dermatoses and aggravated their signs and symptoms.

Adult↗

Telogen effluvium: a clinically useful concept, with traction alopecia as an example.

Telogen effluvium is the excessive loss of normal club hairs. It can be diagnosed simply by microscopic examination of the hairs that are being shed. These hairs have uniform shaft diameter and normally shaped bulbs that contain no pigment. The causes of telogen effluvium are well known and easily distinguishable. They include parturition, febrile illness, surgical operations and anesthesia, anticoagulant drugs, psychi stress, crash diets, and traction. Traction alopecia often produces recognizable patterns of hair loss and can be diagnosed in many cases with reasonable certainty. It is usually not accompanied by inflammatory changes and the prognosis is excellent provided the causative styling practices-braids, rollers, ponytails, etc-are discontinued. Telogen conversion may be caused by interference with papillary blood flow. Since traction alopecia would seem to be reproducible, it may serve as a model for the study of telogen conversion.

Alopecia↗

Congenital temporal triangular alopecia.

Congenital temporal triangular alopecia is a form of nonscarring alopecia that, as its name suggests, is present at birth. Four cases are reported. One patient underwent hair transplantation, which was successful and might be useful in other patients. Cases occurring in a father and his son suggest for the first time a genetic link.

Adult↗

[Positive therapeutic effect of diacarb in the syndrome of progressive muscle spasms, alopecia and diarrhea (Satoyoshi syndrome)].

A 13-year-old girl with Satoyoshi's syndrome is described. The disease manifested itself with generalized cramps, myoclonus, alopecia, diarrhea, growth retardation, muscular hypertrophies, bone malformations, and uterine and gonadal aplasia in the presence of normally developed breasts. Acetazolamide therapy led to a considerable alleviation of cramps and myoclonuses. The child's father displayed gynecomasty, muscular hypertrophy, patent palate, early alopecia and intensified lumbar lordosis; as a child she had had marked cramps in the leg muscles. The examination of the karyotype in the proband and father revealed heteromorphism with regard to the central region of the 22nd chromosome in both cases. Differential staining of the chromosomes failed to show the deletion of any fragment of this chromosome which made it possible to consider this karyotype peculiarity as a variant of the norm.

Acetazolamide↗

Alopecia ichthyotica. A characteristic feature of congenital ichthyosis.

Scarring patchy alopecia is a characteristic feature of various forms of congenital ichthyosis (congenital ichthyosiform erythroderma). 4 patients presenting this scalp disease are described. The hair loss is obviously a consequence of the ichthyosiform erythroderma, and therefore we propose for this type of pseudopelade the term alopecia ichthyotica.

Adolescent↗

[Immunological study of alopecia areata].

Sixteen patients with varying degrees of alopecia areata, without an associated disease were examined before and during a local DNCB therapy for evidence of immunological abnormalities in both cellular and humoral immune parameters. The mean values of IgG, IgA, IgM, IgE, complement factors C3 and C4 and of alpha 1-antitrypsin were found within the norm, of the C1-Inhibitor slight diminished. Antinuclear antibodies in low titer were observed in three, antithyroglobulin antibodies in two young patients. Immuncomplexes were always negative. The mean value for T cells (67 +/- 7%) was normal as well as the lymphocyte transformation to phytohemagglutin, pokweed-mitogen and candidin, the PPD stimulation was slight reduced. No changes in the immunprofile were observed during the DNCB therapy, with exception of a slight, but significant increase of the T cell number from 63 +/- 8% to 77 +/- 5%. Our findings exclude a common systemic immune defect in alopecia areata.

Adolescent↗

Corticosteroids in the treatment of alopecia totalis. Systemic effects.

Fifteen patients with alopecia totalis (AT) or alopecia universalis (AU) were treated with combined topical, intralesional, and oral corticosteroids. All or virtually all scalp hair regrown on seven patients, and they have been able to discontinue oral corticosteroids without recurrence of AT or AU for periods of three months to 7 1/2 years, with an average remission of 32 months. Two additional patients are currently receiving 5 mg of prednisone every two days. Using topical and intralesional corticosteroids in more resistant areas allowed for more rapid lowering of oral doses and thus side effects were minimized. In view of the usually nearly hopeless prognosis for AT and AU and the results reported here, a trial course of topical, intralesional, and oral corticosteroid treatment would seem to be reasonable for highly motivated patients.

Administration, Oral↗

Angiosarcoma (angioendothelioma) of the scalp. An unusual case of scarring alopecia.

Angiosarcoma with initial involvement of the scalp is a rare, malignant neoplasm. We present the case of a 77-year-old woman in whom extensive scarring alopecia developed that was proved histologically to be due to angiosarcoma. She had a good initial response to treatment with electron-beam therapy, but after seven months, extensive nodules and indurated areas on the face beyond the borders of the radiation field developed. Subsequently, massive cervical adenopathy and metastases to the lungs, liver, and spleen developed. Angiosarcoma must be differentiated from other malignant blood vessel tumors and should be considered in the differential diagnosis of scarring alopecia of the scalp in elderly patients.

Aged↗

Topical treatment of alopecia areata.

It has been shown previously that alopecia areata can be treated with dinitrochlorobenzene (DNCB) and other contact allergens. Whether these agents work by inducing immunologic stimulation or simply a nonspecific inflammatory reaction has not been definitively demonstrated. To test the relative importance of these two mechanisms, we have randomly studied 22 patients with alopecia areata to whom either DNCB or croton oil was applied topically. Sixty-three percent of patients without spontaneous regrowth of hair regrew hair after DNCB application. None of those treated with croton oil regrew hair when treated later with DNCB. Therefore, a proved contact allergen was shown to be required for therapeutic success. Patient acceptance of the induced contact dermatitis was excellent. In light of recent data on the mutagenicity of DNCB to bacteria, other contact allergens for topical immunotherapy are being sought.

Adult↗

[Alopecia areata--an autoimmune disorder?].

The authors indicated examinations of actual immune states in 42 patients with alopecia areata in whom no cause of the disease was stated. The examination revealed a deficit in the cellular component of immunity and an increased level of immunoglobulin M and C3 component of complement. The examination of these patients included also the direct immunofluorescence examination of a skin excision from the alopetic focus. Out of 43 examined patients 30 had positive immunocomplex findings. Immunocomplexes were most frequently constituted of M immunoglobulin, C3 component of complement, as well as of A and G immunoglobulins. Immunocomplexes were localized in the lower and sometimes in the medium part of the folicle or perifolicullary, the intensity of fluorescence depended on the severeness of the disease. The deficit in the cellular component of immunity, dysimmunoglobulinemia in the peripheral blood and a positive immunocomplex findings in hair folicles at the examination of skin excisions from the alopetic focus by means of direct immunofluorescent method enables to include the disease of alopecia areata among autoimmune disease. The conclusion deals with the possibilities of utilization of local and general immunomodulation therapy. (Tab. 2, Fig. 1, Ref. 20.)

Adolescent↗

[Immunostimulation therapy in patients with alopecia areata].

The authors recorded decreased values of active and total E rosettes and increased values of IgM and C3 components of complement in 13 patients with alopecia areata. Direct immunofluorescence examination of skin excisions from the alopetic foci revealed immunocomplexes in hair follicles. On the basis of these examinations, excluding other causes of the disease of alopecia areata, the authors indicated the general immunostimulation therapy. (Decabris tbl., Transfer faktor inj.). This therapy resulted in an increase in active and total E rosettes and a decrease in IgM, and thus in the recovery of cellular and indirectly in the humoral component of immunity in the peripheral blood under the circumstances of a favourable clinical therapeutic effect.

Adolescent↗

[Binswanger's type encephalopathy without alopecia and lumbago in young hypotensive patients].

Binswanger's type encephalopathy is characterized by progressive dementia and diffuse subcortical ischemic lesions associated with arteriosclerosis. Hypertension is believed to be a major pathogenic factor in causing this encephalopathy but there are some cases of the encephalopathy not suffering from hypertension. In 1985, Yamamura et al. and Fukutake et al. reported familial cases of normotensive juvenile Binswanger's type encephalopathy with alopecia and lumbago, and considered it to be possibly a new clinical syndrome. We reported three cases of relatively young-onset (under the age of 40) Binswanger's type encephalopathy with persistent hypotension. All three patients suffered from neither alopecia nor lumbago. Patient (male aged 40) had repeated episodes of ischemic stroke and had progressive dementia. Patients 2 (male aged 41) and 3 (male aged 34) were not in a state of dementia, but had a history of transient ischemic attacks, and at present are completely symptom-free. Though there were no risk factors for cerebrovascular disease in these cases, the repeated episodes of ischemic stroke and the existence of small multiple lacunes in the basal ganglia on CT and MRI suggest that the white matter damage was principally due to a vascular disorder. In these cases, persistent hypotension was characteristic and might be a factor for the induction and exacerbation of this encephalopathy. These three cases are different from the classic form of Binswanger's type encephalopathy based on hypertension. Normotensive cases have been described before, but our cases do not seem to fall into this category because the blood pressure constantly remained hypotensive.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗