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Background: Cutaneous leiomyomas are uncommon benign smooth muscle neoplasms of skin of unknown pathogenesis. We report a sporadic case of multiple cutaneous leiomyomas at the site of a chemical burn., Observation: A 47-year-old male presented with multiple grouped red nodules on the right calf painful to cold and to touch. The lesions were located on the site of a chemical wound that had occurred 5 years earlier. Histopathological examination revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma., Discussion: To our knowledge, the literature contains no previous reports of sporadic multiple cutaneous leiomyomas occurring at a chemical burn site. While a chance association cannot be ruled out, there is also the possibility of a physiopathological mechanism similar to that of vascular tumours following exposure to 4-hydrazinobenzoic acid. This compound, a hydrazine derivative of the fungus Agaricus bisporus, can cause smooth-muscle tumours in the aorta and large vessels with morphological characteristics similar to those seen in vascular leiomyomas., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: The purpose of this study is to describe two new trichoscopic signs, "Eastern pancake sign" and "comedo-like structures", not previously reported to our knowledge and seen in 5 patients with alopecic and aseptic nodules of scalp (AANS)., Patients and Methods: We report on 5 patients presenting AANS. Demographic and ethnic characteristics, disease duration, clinical description, treatment and evolution were noted. Trichoscopy was performed for all patients., Results: In all cases of NAAS, trichoscopy revealed 2 signs: "Eastern pancake sign" resulting in heterogeneous dilated follicular orifices without trichoscopic signs of alopecia areata, and "comedo-like structures" resembling comedones., Discussion: To our knowledge, there have been no previous reports in the literature of the 2 new trichoscopic signs of AANS described herein: "Eastern pancake sign" and "comedo-like structures". The trichoscopic feature of comedo-like structures suggests the potential role of follicular occlusion in this disease. We consider that follicular occlusion may be a triggering factor in AANS., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Pilar dysplasias are congenital or acquired disorders, they are characterized by hair abnormalities in color, density, length and structure. Optic microscopy and polarized light microscopy analysis of piliary shafts provides important informations and contribute to the diagnosis of complex syndromes. Many pathological profiles can be visualized, some are specific, other are not. Some authors use a particularly convenient classification including 3 groups: in the first, piliary shaft abnormalities are isolated, in the second the abnormalities correspond to complex syndromes, and in the third they are associated with various malformations. Based on some clinical cases, we will describe various types of pilar dysplasias and we present recent advances, particularly in genetics.

Background: Giant axonal neuropathy is an autosomal recessive condition characterized by progressive degeneration of the central and peripheral nervous system. Sensoromotor neuropathy develops around 3 years of age. Children have particular faces with curly hair. Characteristic pilar anomalies occur early and have diagnostic value. CARE REPORT: An Algerian boy born to consanguineous parents (first cousins) had language retardation and gait disorders developed around 3 years of age. At 9 years, the child was in a cachetic state with valgus feet, amyotrophy and diminished muscle force predominating distally, ataxia, areflexia, sensoromotor neuropathy and nystagmus. Skin tropism was altered with pale, thin and dry skin, cold, cyanotic limbs and thick curly hair. Electrophysiology explorations showed signs of chronic sensoromotor polyneuropathy with axonal predominance. Brain and spinal MRI revealed cerebellar atropy and signs of leukodystrophy. The spinal tap was normal. A neuromuscular biopsy confirmed the diagnosis of giant axonal neuropathy. At examination the hair was thick with reduced refrangibility and a pseudo-pili torti aspect., Discussion: Giant axonal neurpathy is characterized by anomalous organization of the cytoskeleton of intermediary filaments in different types of cells. Hair anomalies occur early, before onset of neurological signs. At gross examination the hair is thick and curly, sometimes crimped and pale. Examination of the pilar shaft shows trichorrhexis nodosa, scalloped fringes and lack of internal structure. On the molecular level, there is a reduction in the number of bisulfur bridges which could be the cause of defective keratin filament alignement. Pathogenicaly, the pilar anomalies are considered as a direct manifestation of defective keratin organization characteristic of the disease.

Background: Electron microscopy examination of scalp biopsies from a patient with chloroquine achromotrichia gave elements concerning the pathogenesis of chloroquine-induced achromotrichia., Case Report: A 21-year old light brown-haired patient developed achromotrichia after four months of treatment with chloroquine for subacute lupus erythematosus. Hair bleaching completely regressed 5 months after discontinuing chloroquine despite replacement with hydroxychloroquine. During the achromatrichia phase, many ultrastructural anomalies were observed in the hair root melanocytes: the nuclei were small and densified, and there was an accumulation of immature melanosomes in the cytoplasm; these melanosomes, mainly in stage II, were rarely transferred to keratinocytes. After recovery from the achromotrichia, melanocytes displayed a normal aspect., Discussion: Pathophysiological disturbances leading to chloroquine induced achromotrichia are still unclear. The ultrastructural study of hair follicles in our patient show that under chloroquine action melanocytes become unable to perform complete melanin synthesis and to produce normally melanized melanosomes which may be transferred to keratinocytes. Non-melanized or poorly melanized melanosomes accumulate in the melanocytes which finally become inactive cells. These findings suggest that achromotrichia is induced by a toxic effect of chloroquine on the melanocyte.

We present a case of trichothiodystrophy associated with a hypereosinophilic syndrome. This case had been followed for 30 years. Trichothiodystrophy was characterized by lamellar ichthyosis, hair and nail dystrophies, kyphoscoliosis, congenital luxation of the hip. The hypereosinophilic syndrome was characterized by an itching urticaria-like eruption. Although the patient's general condition had remaining stable for 30 years, during the last year it worsened and the patient suddenly died. The authors discuss about the significance of this association.

Oculo-dento-digital syndrome (SODD) as defined by Meyer-Schwickerath in 1957 is a rare entity (84 cases) which belongs to ectodermal dysplasias. It consists of: the characteristic features (long face, pinched nose); syndactyly; ocular, dental and bone abnormalities. This entity is usually transmitted on the autosomal dominant mode. We report two cases (a mother and daughter) with polymalformations which we classed as SODD. Furthermore, agenesis of lacrimal duct and genitourinary abnormalities were noted. The mother had a very particular complex hair shaft dysplasia (incomplete pili torti, "tiger tail" aspect, fractures) with alopecia since she was fifteen years old. The daughter's hair was normal at birth. In SODD, fine and sparse hair is often observed (44 p. 100). Only one patient had hair shaft investigation under polarized light: pili annulati and monilethrix were described but not found in our cases. These two reports incite to the systematic hair shaft study in SODD as other ectodermal dysplasia syndromes.

We observed a case of folliculitis decalvans involving the beard and the scalp with a tufted hair folliculitis aspect in the occipital region. The presence of two clinical presentations in the same patient has not been reported in the literature. This would suggest that the two entities could be morphological aspects of the same pathological process which might cause either atrophy with loss or the annexes or tufted hair folliculitis depending upon the depts and destructive capacity of the inflammatory process.

There are many clinical presentations to fibrofolliculoma, described by Birt, Hogg and Dubé: the solitary and multiple forms, with or without other skin tumors, could also be markers of intestinal polyposis. Little is known of its pathogenesis. A case of multiple fibrofolliculoma of the face and neck is described. A new therapeutic approach by copper vapour laser is proposed.

A typical case of loose anagen hair syndrome in a 4-year old girl is reported here. The key signs of this disease are the absence of hair cut since infancy, an easy and painless uprooting of the hairs and a 100 p. 100 anageneity of the hairs with distorted bulbs on trichograms. There is no known treatment of this anomaly. Cosmetically, the course of the disease is usually favourable, but abnormal trichograms and fragility of the hairs persist in adulthood. A brief review of the literature concerning this probably underestimated condition is presented.

A 42-year-old man presented with multiple hypopigmented macules, 4 to 10 mm wide, irregularly shaped and symmetrically distributed on the neck and face, with a 10 years duration. Histological examinations showed a plate-like epithelial proliferation below the epidermis. The cells had a pale cytoplasm, with few PAS positive granules. In the lower part of the proliferation the peripheral cells were palisading and surrounded by a dense elastic network. Serial sections showed connections with the normal outer root sheath of the neighbour follicles, and with the epidermis. This pattern is very characteristic of Mehregan's tumor of follicular infundibulum. Four distinctive presentations of this tumor can be observed, in spite of its rarity: multiple macules of the upper chest or face (4 cases have previously been described), tumors of follicular infundibulum occurring in Cowden's disease or in the natural course of organoid nevi, and the most common solitary form of the face. With their striking clinical features, the eruptive or multiple forms of this tumor should be recognized as a distinctive clinical entity. Possible transformation into basal cell carcinoma has been described in one of these cases, and long time supervision should be recommended.

In 1984, Zaun described a new type of pilary dysplasia characterized by easily pluckable hair. We were able to observe three patients with this anomaly, and this paper is an attempt at reviewing the subject on the basis of these new cases compared with those previously published. Clinical features. The major sign of the anomaly is that hairs can be pulled off in tufts easily and painlessly, and promptly grow again (our cases). In all cases reported so far the hair was blond or dark-blond; in some patients it is described as scintillating (our cases). The hair shaft is usually thin (one of our cases), but it may be of normal caliber (two of our patients). In the occipital region the hairs are entangled, dry and short (our cases). They are implanted wide apart or at normal intervals (our patients); areas of alopecia are sometimes encountered (our cases). Hair growth may be slow (one of our cases) or of normal speed (two of our cases). The eyelashes and eyebrows, as well as other body hairs, are normal in all patients (fig. 1). Microscopy. The trichogram is exclusively composed of anagenic and dystrophic roots (our cases). Pathological examination by biopsy of the scalp is characteristic: transverse and oblique sections of the follicles show that the hairs are oval, triangular or trapezoidal in shape (fig. 7). Alterations of the inner epithelial sheath are also present, including keratinization and early decomposition (fig. 8), lack of complementation (fig. 7) and fissures between the cuticle of the inner epithelial sheath and of the hair, in the keratogenic area (fig. 9). (ABSTRACT TRUNCATED AT 250 WORDS)

A 36-year-old blond woman suddenly developed green tinted hair following exposure to swimming pool water. This was the first green discoloration she noticed, although she had been an active swimmer for several years. Clinical examination showed green tinting toward the distal ends of the most superficial strands of hair. The copper content of plucked green hair measured by atomic absorption was elevated to 3,900 ppm and the copper concentration in water from the swimming pool implicated was 9.94 ppm. Following renewal, the latter value decreased to 107 ppb. Hair examination under polarizing light was normal, and a scanning electronmicroscopic study of hair samples showed a total loss of cuticle with micropits scattered over the hair shaft surface mimicking a "dead tree trunk". Epidemiological investigations showed that the increased copper content of swimming pool water was due to added algaecides without adequate replacement of the water. Hair damage resulting from repeated waving and/or bleaching of the hair turns out to be an important factor in the deposition of copper by inducing an increase in keratin content of cysteic acid and related anionic sulfonate groups which participate in copper adsorption. On the ocassion of this case-report, epidemiological data published by others, together with the chemical and therapeutic aspects of green hair are reviewed.

Clinical investigation and hair plucking performed during trichogram, a traumatic technique for obtaining a hair formula, are the present means of evaluation in patients with alopecia or complaining of loss of hair. These means do not provide quantitative data valuable enough for a possible appreciation of the severity of hair loss, let alone for a reliable evaluation in therapeutical trials. Phototrichogram, a technique proposed by Saitoh (1970), Fiquet and Courtois (1979) and developed by Bouhanna (1982-1983), brings more satisfactory qualitative and quantitative means of studying hair growth and pilar cycles. Its standardisation had yet to be obtained so as to give results comparable from one study to another and reproducible in any given patient. We propose here a standardised optical equipment and particularly recommend a rigid frame allowing a fixed distance from the skin to the lens, and a superimposed glass window with millimetric graduations; the 0.50 square centimeter skin area to be analysed may easily be marked off by an 0.2 mm wide black microtatoo. This personal technical adaptation gives an easily obtainable definition of five quantitative parameters analysing the hair on a given area of the scalp: density of implantation, telogen percentage, growth rate, mean anagen diameter and percentage of fine hair less than 40 mu in diameter. In the normal male adult our measurements gave the following results on the vertex: density 204 +/- 10 hair per square centimeter, telogen percentage 17.8 +/- 2.8 p. 100, growth rate 0.35 +/- 0.03 mm p. day, mean anagen diameter 76 +/- 5 mu, percentage of fine hair 9.2 +/- 1.8 p. 100.(ABSTRACT TRUNCATED AT 250 WORDS)

A progressive papular eruption on the face, limbs and vulva had been present for seven years in a 38-year old female patient. Some papules were small and confluent while others were sparse, as in nodular prurigo. Histology showed massive proliferation of the follicular sheath epithelium, connected to the lower surface of the epidermis. The lesion formed a plate-like area very similar to that observed in superficial basal cell carcinoma and corresponding to a tumour of the follicular infundibulum. Remarkable features of this case were the large number of lesions, their photosensitivity (they became pruriginous and increased in size after exposure to the sun), the presence of eccrine sweat glands beneath a skin lesion of the thigh, the basal cell degeneration of two lesions and the slight improvement observed after etretinate therapy. To our knowledge, such findings have not yet been reported in tumours of the follicular infundibulum.

The mixture at equal volumes of a 0.1 p. 100 solution of rhodamine B in distilled water and of a 0.25 p. 100 solution of toluidine blue in Walpole's pH 4.4 buffer dyes each pilar sheath differently. At the level of the medulla, the granules fix rhodamine B, so do the cortical cells at the level of the keratogenic zone. Once they are keratinized, however, cortical cells remain colorless. Concerning the cells of the inner root sheath, on the other hand, their trichohyaline granules are dyed by rhodamine B, whereas the keratinized cells turn dark blue under the effect of toluidine blue. The trichilemmal keratin both of the isthmus of the anagen hair and of the follicular sac becomes light red. This technique, which can be easily applied to the trichogram, allows us to identify more or less mature pilar anlages in in adnexal tumors, to differentiate keratinizing cysts and to trace various pathological keratins. Thanks to a chemical study, it was shown that the mixture rhodamine B-toluidine blue is only a mechanical mixture which works through its acide-bases properties.

Trichothiodystrophy is a pilar dysplasia which is characterized by the existence of brittle hair with trichoschisis, a typical pattern of transmission of polarized light and decreased levels of sulfur containing amino acids. In this report we show various aspects of the hair dysplasia and of the hair bulbs by light and scanning electron microscopy. Normal levels of cystin in the peripheral blood were associated with decreased levels of this amino acid in the hair shafts. Incorporation of radio-labelled cystine in hair follicles seemed however normal. Our results do not support the generally accepted hypothesis of a defective transport mechanism in the hair follicle. As a similar defect in other tissues (e. g. nervous system, spermatozoids...) is also thought to be responsible for the associated symptoms (e. g. nervous impairment, sterility in males...) we think it is important to lead further research in this field in order to elucidate the metabolic pathways underlying these rare clinical syndromes.

Piedra (stone in Spanish) is the name given to a trichomycosis characterized by the formation of nodules resembling small stones. There are two varieties of the disease, depending on the colour of the nodules: white piedra and black piedra. Black piedra sharply differs from white piedra on three main scores: a) the causative agent is a black filamentous and sexed dematicious fungus, Piedraia hortai; b) the disease exclusively affects the scalp, and c) the geographical distribution of human black piedra is limited to tropical and subtropical areas (South America, South-East Asia). White piedra has a different aetiology, being caused by an asexual fungus, Trichosporon beigeli. The genus Trichosporon (Behrend, 1890) and the species T. beigeli (Vuillemin, 1902) were created from a case of piedra of the moustache. White piedra may involve hairy regions other than the scalp, such as the beard and moustache, less frequently the armpits, eyebrows, eyelashes and pubic hair. The disease has been observed in all continents, except Africa, and under all climates, although it is exceptionally found in cold areas (two indigenous cases in Finland). The observatio princeps of white piedra (on a false chignon) was published in 1865 by Beigel, in London. In France, only three cases, all concerning the moustache, were reported at the very beginning of this century No other case has been published in that country in the east 80 years. T. beigeli is a common saprophyte in nature. It has been found in soil, water, fruit, rotten vegetables, sawdust, as well as in man (skin, skin appendages, mucosae) and in animals (mammals, insects, mussels).(ABSTRACT TRUNCATED AT 250 WORDS)