Claves diagnósticas en displasias pilosas I / Keys to the diagnosis of hair shaft disorders: Part I

Las displasias pilosas corresponden a alteraciones en la estructura del tallo del cabello y pueden ser congénitas o adquiridas. Se clasifican en dos grandes grupos según la presencia o no de fragilidad capilar. En la mayoría de los casos la valoración del paciente, la anamnesis y la exploración física nos llevarán al diagnóstico. En los últimos años, el uso de la tricoscopia se ha posicionado como una técnica útil y coste efectiva, sobre todo en niños, ya que permite lograr una adecuada exploración sin tener que arrancar los cabellos. En algunas ocasiones las alteraciones en la estructura del cabello serán la clave diagnóstica de enfermedades más complejas, en las que la instauración de un tratamiento precoz puede mejorar el pronóstico. El propósito de esta revisión es aportar las claves que permitan diagnosticar las displasias pilosas más frecuentes y valorar las alternativas terapéuticas disponibles en la actualidad (AU)

Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be either congenital or acquired. Two large categories have been defined according to the presence or not of hair fragility. A diagnosis can usually be made after taking a thorough medical history and performing a physical examination. Trichoscopy has become a useful, cost-effective tool in recent years, particularly for examining the hair of children, because it facilitates inspection without removal of hairs. Structural abnormalities in the hair shaft are sometimes clues to the diagnosis of more complex diseases in which early treatment can improve prognosis. This review describes key features that enable the diagnosis of the most common hair shaft disorders and discusses the various treatments currently available (AU)

[Translated article] Keys to the Diagnosis of Hair Shaft Disorders: Part I / Claves diagnósticas en displasias pilosas I

Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be either congenital or acquired. Two large categories have been defined according to the presence or not of hair fragility. A diagnosis can usually be made after taking a thorough medical history and performing a physical examination. Trichoscopy has become a useful, cost-effective tool in recent years, particularly for examining the hair of children, because it facilitates inspection without removal of hairs. Structural abnormalities in the hair shaft are sometimes clues to the diagnosis of more complex diseases in which early treatment can improve prognosis. This review describes key features that enable the diagnosis of the most common hair shaft disorders and discusses the various treatments currently available (AU)

Las displasias pilosas corresponden a alteraciones en la estructura del tallo del cabello y pueden ser congénitas o adquiridas. Se clasifican en dos grandes grupos según la presencia o no de fragilidad capilar. En la mayoría de los casos la valoración del paciente, la anamnesis y la exploración física nos llevarán al diagnóstico. En los últimos años, el uso de la tricoscopia se ha posicionado como una técnica útil y coste efectiva, sobre todo en niños, ya que permite lograr una adecuada exploración sin tener que arrancar los cabellos. En algunas ocasiones las alteraciones en la estructura del cabello serán la clave diagnóstica de enfermedades más complejas, en las que la instauración de un tratamiento precoz puede mejorar el pronóstico. El propósito de esta revisión es aportar las claves que permitan diagnosticar las displasias pilosas más frecuentes y valorar las alternativas terapéuticas disponibles en la actualidad (AU)

Claves diagnósticas en displasias pilosas II / Keys to the Diagnosis of Hair Shaft Disorders: Part II

Las displasias pilosas constituyen alteraciones en la estructura del tallo que pueden estar provocadas por factores ambientales o por mutaciones genéticas. En esta revisión se presentan las displasias pilosas sin o con escasa fragilidad capilar. Es importante realizar una exhaustiva anamnesis y una exploración del cabello dirigida a detectar cambios en la textura, la densidad, la calidad y la presencia de fragilidad en el cabello. La tricoscopia es una herramienta útil y no invasiva, que permite hacer una aproximación diagnóstica en la mayoría de los casos. Actualmente no existe un tratamiento específico para el manejo de las displasias pilosas, se recomiendan cuidados generales para prevenir el daño del cabello como el cepillado excesivo, los productos químicos, los peinados con tensión y la exposición excesiva al calor. Algunas displasias pilosas mejoran con el inicio de la pubertad, y en otros casos, tratamientos como el minoxidil tópico pueden resultar de utilidad (AU)

Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be caused by genetic mutations or acquired through environmental exposures. The second part of this review presents these disorders classified according to the degree of hair fragility. It is important to take a thorough medical history and examine the hair to detect changes in texture, density, quality, and whether fragility is observed or not. Trichoscopy is a useful, noninvasive tool that can suggest a diagnosis in most cases. Specific treatments for hair shaft disorders are not available at present. We recommend general care practices to prevent hair damage; examples are avoiding excessive brushing, chemical products, hairstyles that introduce tension, and exposure to excessive heat. Some hair shaft disorders improve with puberty. Others may respond to treatments such as topical applications of minoxidil (AU)

[Translated article] Keys to the Diagnosis of Hair Shaft Disorders: Part II / Claves diagnósticas en displasias pilosas II

Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be caused by genetic mutations or acquired through environmental exposures. The second part of this review presents these disorders classified according to the degree of hair fragility. It is important to take a thorough medical history and examine the hair to detect changes in texture, density, quality, and whether fragility is observed or not. Trichoscopy is a useful, noninvasive tool that can suggest a diagnosis in most cases. Specific treatments for hair shaft disorders are not available at present. We recommend general care practices to prevent hair damage; examples are avoiding excessive brushing, chemical products, hairstyles that introduce tension, and exposure to excessive heat. Some hair shaft disorders improve with puberty. Others may respond to treatments such as topical applications of minoxidil (AU)

Las displasias pilosas constituyen alteraciones en la estructura del tallo que pueden estar provocadas por factores ambientales o por mutaciones genéticas. En esta revisión se presentan las displasias pilosas sin o con escasa fragilidad capilar. Es importante realizar una exhaustiva anamnesis y una exploración del cabello dirigida a detectar cambios en la textura, la densidad, la calidad y la presencia de fragilidad en el cabello. La tricoscopia es una herramienta útil y no invasiva, que permite hacer una aproximación diagnóstica en la mayoría de los casos. Actualmente no existe un tratamiento específico para el manejo de las displasias pilosas, se recomiendan cuidados generales para prevenir el daño del cabello como el cepillado excesivo, los productos químicos, los peinados con tensión y la exposición excesiva al calor. Algunas displasias pilosas mejoran con el inicio de la pubertad, y en otros casos, tratamientos como el minoxidil tópico pueden resultar de utilidad (AU)

Pilomatrical Tumor of Low Malignant Potential: A Tumor Between Pilomatricoma and Pilomatrical Carcinoma.

ABSTRACT: We report a case of pilomatrical tumor showing intermediate histological features between pilomatricoma and pilomatrical carcinoma. The lesion recurred twice with the same histological features. Similar cases were was probably called aggressive or proliferating pilomatixoma; we think that the term pilomatrical tumor of low malignant potential is more suitable for this lesions. Excision with wide free margins and follow-up are recommended.

Avaliação das propriedades das fibras capilares tratadas com alisante ácido com diferentes valores de pH / Evaluation of hair fibers properties treated with acid straightener at different pH values

Os produtos para alisamento das fibras capilares são amplamente utilizados no Brasil principalmente, pelo público feminino. Porém, estes procedimentos podem causar danos ao cabelo do usuário e, também, para o profissional que está atuando. Os alisantes químicos podem ser divididos em dois grupos: alcalinos e ácidos. Os primeiros (tioglicolato de amônio e os hidróxidos de sódio ou guanidina) apresentam valor de pH elevado (superior a 9,0) e alisam por meio da quebra e reorganização das pontes de dissulfeto (S-S) presentes na queratina, proteína estrutural da fibra capilar. Os alisantes ácidos como o formaldeído, ácido glioxílico e seus associados, possuem pH baixo (≥ 2,0) e causam uma reorganização no interior da fibra e geralmente leva a formação de um filme na superfície do fio. O ácido glioxílico associado à carbocisteína e aminoácidos (nomenclatura INCI -International Nomenclature of Cosmetic Ingredient apresentada entre parênteses) (Glyoxyloyl Carbocysteine (and) Glyoxyloyl Keratin Aminoacids (and) Water) é o único ingrediente permitido ao uso como alisante ácido até o momento. O objetivo deste trabalho foi estudar o efeito deste alisante ácido incorporado em uma emulsão óleo e água (O/A) com valores de pH 1,0 e 2,0 (com reaplicações); e o impacto que podem causar nas propriedades mecânicas e químicas da fibra capilar, como força de ruptura, coloração, elasticidade e teor de triptofano da fibra capilar. E também o efeito da radiação ultravioleta nos fios alisados. Nos testes realizados observou-se que as mechas tratadas com a emulsão a pH 1,0 obtiveram melhor resultado de alinhamento das cutículas e capacidade alisante da fibra, com penteabilidade superior ao cabelo virgem (59,4 %), enquanto as mechas com a formulação pH 2,0 foi apenas 33,0%. Entretanto, a força de tração para a ruptura do fio foi inferior, diminuindo em 16,0% (pH 1,0) e 9,0 % (pH 2,0). Quanto a variação da coloração, a variação do tom de cor foi mais exacerbada para as mechas tratadas com a formulação em pH 1,0. O teor de triptofano foi inferior nas mechas alisadas com a formulação pH 1,0. Para o teste de DSC e Raman, ambas as mechas tratadas apresentaram modificações nas suas estruturas. As fibras alisadas e expostas à radiação UV apresentaram danos maiores nas cutículas, e certa proteção na degradação de proteínas em relação aquelas sem tratamento e irradiadas ao UV. Considerando as reaplicações do produto alisante ácido, quanto maior o número de aplicações, mais alinhadas e seladas tornaram se as fibras, mas mais rígida e suscetível à quebra ficaram. Devemos considerar a importância do valor do pH da formulação no impacto do alisamento e dano da fibra capilar

Hair straightening products are widely used in Brazil by the female public. However, these procedures can cause damage to the hair shaft and to professional´s and client´s health. Chemical straighteners can be divided into two groups: alkaline and acid. Alkaline straighteners has a very high pH value (> 9.0) and the straightening process is due to the break followed by reorganization of the disulfide bridges (S-S) present in the keratin, a structural protein of the hair shaft. Some examples of alkaline straighteners are ammonium thioglycolate and sodium or guanidine hydroxides. On the other hand, acid straighteners like formaldehyde, glyoxylic acid, and their associated, present a low pH value (≥ 2.0), which causes a rearranging within the fiber, and sometimes creates a film on the surface of the hair. Nowadays, the glyoxylic acid associated with carbocysteine and amino acids (INCI - International Nomenclature of Cosmetic Ingredient nomenclature presented in parentheses) (Glyoxyloyl Carbocysteine (and) Glyoxyloyl Keratin Aminoacids (and) Water) is the only acid straightener permitted. Therefore, the objective of this work is to study the effect of this acid straightener, incorporated into oil in water (o/w) base emulsions at two different pH values 1.0 and 2.0 ( with reapplication) on the mechanical and physicochemical properties of the hair shaft. Also, the impact of UV radiation in straightened tresses. In the experiments conducted, it was possible to observe that tresses treated with the emulsion at pH 1.0 had a better result on the alignment and the straightening capacity, improving the combing test (59.4%/), while the tresses with the formulation at pH 2.0, 33.0%. However, the tensile strength, to pH 1.0 decreased by 16.0% and to pH 2.0 only 9.0%. As for color, the difference when compared with virgin hair it was major at pH 1.0. The tryptophan content also was lower in tresses treated at pH 1, 0. For the DSC and Raman analyzes all the treated tresses presented modifications in their structure. Straightened strands exposed to radiation had major damage in the cuticles, and some protection in proteins degradation compared to untreated tresses exposed to radiation. Considering the reapplications of the straightening product, as higher the number of applications more aligned and sealed it becomes, however the hair fiber becomes stiffer and susceptible to breakage. Thats why we should consider the importance of the pH value in the product, and the impact of straightening and damage of the hai fiber

Epidermal Nevi and Related Syndromes - Part 2: Nevi Derived from Adnexal Structures. / Nevus epidérmicos y síndromes relacionados. Parte 2: Nevus derivados de estructuras anexiales.

Epidermal nevi are hamartomatous lesions derived from the epidermis and/or adnexal structures of the skin; they have traditionally been classified according to their morphology. New variants have been described in recent years and advances in genetics have contributed to better characterization of these lesions and an improved understanding of their relationship with certain extracutaneous manifestations. In the second part of this review article, we will look at nevi derived from the adnexal structures of the skin and associated syndromes.

A proposed classification for follicular involvement by melanoma.

BACKGROUND: Folliculotropism in melanoma is poorly characterized and standard categorization for follicular involvement by melanoma is unavailable. We propose a logical categorization system. METHODS: We conducted a search of our archives over a 24-year period for cases mentioning the terms follicle, follicular, folliculotropic, folliculocentric and melanoma. RESULTS: We identified 90 cases of melanoma with involvement of the hair follicle. Distinct patterns were identified. The invasive patterns were primary follicular, folliculotropic and invasive arising from melanoma in situ (MIS) with extensive follicular involvement. Follicular involvement by MIS was either lentiginous, nested or a combination of both. A total of 29 invasive melanomas were identified. Of these 12 had invasive melanoma around the hair follicle, 2 were primary follicular melanomas, 7 showed folliculotropism and 3 were invasive melanomas arising from MIS around the follicle. Seventeen invasive melanomas had follicles only involved by MIS (9 nested, 6 nested and lentiginous and 2 lentiginous). A total of 61 cases of MIS with follicular involvement were identified; of these 33 were lentiginous, 10 nested and 18 both lentiginous and nested. CONCLUSION: We propose that the three distinct patterns of follicular involvement by invasive melanoma and the three distinct patterns of MIS will be valuable for logically categorizing involvement of the hair follicle by melanoma.

Benign follicular tumors

Abstract: Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.

Benign follicular tumors.

Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.

Panfolliculoma and histopathologic variants: a study of 19 cases.

Panfolliculoma (PF) is an uncommon benign follicular neoplasm exhibiting differentiation toward all components of a hair follicle. Several pathologic manifestations have been described in a limited number of cases. We studied 19 cases of PF to summarize the clinical parameters and to classify this unique neoplasm histopathologically. A study of the largest sample series of PF has been performed here. The lesions affect both genders after age 20. The head is the most common site of PF of all types. On microscopic examination, all cases demonstrated advanced follicular differentiation by showing cell components of infundibulum, isthmus, stem, bulb, and mesenchymal papilla. Based on the findings and various patterns in histopathology, we classified PF into 3 subtypes: nodular, superficial, and cystic. The superficial and cystic variants account for most of the cases in our study. The histopathologic differential diagnosis is also discussed for each subtype.

Telogen effluvium revisited.

Telogen effluvium (TE) is heterogeneous disorder. It can be classified into three main categories: the premature teloptosis, the collective teloptosis and the premature entry into telogen. The last category can be divided in three types: the drug induced TE, TE due to dietary deficiencies and the "autoimmune" TE. Despite this heterogeneity, the large majority of TE that arrive at the dermatologist's observation pertain to the autoimmune type, featuring a standard presentation. The typical patient is a woman claiming to have always had a "full head of hair" and reporting her hair to come out suddenly "by the handful". Usually, she is accurate about the date of onset of her shedding. She is in good health, without signs of anorexia nor nutrient deficiencies. She admits to having been in an anxious state for some months, and felt, occasionally or not, a painful or burning sensation at the scalp (trichodynia). Usually, the course of the disorder is chronic but intermittent, with apparent remissions being irregularly intermitted by relapses. The shed hairs do not exhibit telogen roots, but mostly exogen ones. This distinct entity, shares some analogies with alopecia areata, including the triggering role of emotional stress, trichodynia and the frequent association with Hashimoto's thyroiditis. Methods to assess its severity and to monitor treatment are described. In the absence of a documented etiopathogenesis, no treatment can be endorsed, but a course of topical corticosteroids could be tried.

Clinical effects of non-ablative and ablative fractional lasers on various hair disorders: a case series of 17 patients.

BACKGROUND AND OBJECTIVES: Both ablative and non-ablative fractional lasers have been applied to various uncommon hair disorders. The purpose of this study was to demonstrate the clinical effects of fractional laser therapy on the course of primary follicular and perifollicular pathologies and subsequent hair regrowth. MATERIALS AND METHODS: A retrospective review of 17 patients with uncommon hair disorders - including ophiasis, autosomal recessive woolly hair/hypotrichosis, various secondary cicatricial alopecias, pubic hypotrichosis, frontal fibrosing alopecia, and perifolliculitis abscedens et suffodiens - was conducted. All patients had been treated with non-ablative and/or ablative fractional laser therapies. RESULTS: The mean clinical improvement score in these 17 patients was 2.2, while the mean patient satisfaction score was 2.5. Of the 17 subjects, 12 (70.6%) demonstrated a clinical response to non-ablative and/or ablative fractional laser treatments, including individuals with ophiasis, autosomal recessive woolly hair/hypotrichosis, secondary cicatricial alopecia (scleroderma and pressure-induced alopecia), frontal fibrosing alopecia, and perifolliculitis abscedens et suffodiens. Conversely, patients with long-standing ophiasis, surgical scar-induced secondary cicatricial alopecia, and pubic hypotrichosis did not respond to fractional laser therapy. CONCLUSION: Our findings demonstrate that the use of non-ablative and/or ablative fractional lasers promoted hair growth in certain cases of uncommon hair disorders without any remarkable side effects.

Trichothiodystrophy: Photosensitive, TTD-P, TTD, Tay syndrome.

Although the term, "trichothiodystrophy" (TTD) refers to the hair anomalies in this group of patients, this is a heterogeneous, multisystem disease in which any or every organ in the body may be affected. Neuroectodermal derived tissues are particularly likely to be involved. This term was introduced by Price et alin 1980 to designate patients with sulfur-deficient brittle hair, which they recognized as a marker for this complex disease and designated it as a "neuroectodermal symptom complex". Patients with TTD have brittle hair and nails (associated with reduced content ofcysteine-rich matrix proteins), ichthyotic skin and physical and mental growth retardation. Ichthyosis is usually apparent at birth but much less so after the first few weeks of life. Other frequently associated features include ocular cataracts, infections and maternal complications related to pregnancy. Atrophy of subcutaneous fat may also be present. TTD occurs in a pattern of inheritance consistent with an autosomal recessive condition. The disease is extremely heterogeneous in severity and extent, with some patients showing no neurological deficiency. Others show severe, multisystem disease. Many patients die at a young age, most commonly due to infectious disease. TTD is part of a more broadly defined group of diseases identified as IBIDS (ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature). Photosensitive cases are also identified as PIBIDS (photosensitivity with IBIDS). Cases without manifest ichthyosis are also identified as PBIDS. These syndromes defy rigorous definition because of clinical variation between patients. The original two cases were described by Tay in oriental siblings, whose parents were first cousins; thus the disease is also known as Tay syndrome. The hairs in patients with TTD have a distinctive, diagnostically useful appearance on polarized light microscopy consisting of alternating light and dark bands known as the "tiger tail" anomaly. Diagnosis may be confirmed by sulfur content analysis ofhair shafts, which shows decreased sulfur and cysteine content. Approximately half of patients with TTD have photosensitivity, which correlates with a nudeotide excision repair (NER) defect. These patients are designated as having trichothiodystrophy-photosensitive (TTDP). Non-photosensitivepatients are designated as having trichothiodystrophy-nonphotosensitive (TTDN). Skin cancer is very rare in sun-sensitive TTD.

The genetics of hair shaft disorders.

Many of the genes causing hair shaft defects have recently been elucidated. This continuing medical education article discusses the major types of hair shaft defects and associated syndromes and includes a review of histologic features, diagnostic modalities, and findings in the field of genetics, biochemistry, and molecular biology. Although genetic hair shaft abnormalities are uncommon in general dermatology practice, new information about genetic causes has allowed for a better understanding of the underlying pathophysiologies.

Lash ptosis in congenital and acquired blepharoptosis.

OBJECTIVE: To determine the prevalence of lash ptosis (LP) in eyes with congenital and acquired blepharoptosis. METHODS: We retrospectively graded photographs of 228 eyes from 174 patients with congenital or acquired blepharoptosis for LP. We used a 4-point rating scale for LP, in which 0 indicates no LP; 1, minimal; 2, moderate; and 3, severe. A prospective evaluation of LP in 30 eyes from 15 patients without blepharoptosis (control eyes) was also performed. RESULTS: A total of 107 eyes (in 87 patients) demonstrated congenital blepharoptosis and 121 eyes (in 87 patients) had acquired blepharoptosis. A moderate to severe rating of LP (rating, >/= 2) occurred in 60.7% of eyes with congenital blepharoptosis, 28.9% of eyes with acquired blepharoptosis, and 6.7% of control eyes. Lash ptosis (rating, >/= 1) was present in 91.6% of eyes with congenital blepharoptosis, 83.5% of eyes with acquired blepharoptosis, and 33.3% of control eyes. The mean LP rating was 2.1 for eyes with congenital blepharoptosis, 1.3 for eyes with acquired blepharoptosis, and 0.6 for control eyes. CONCLUSIONS: Lash ptosis was common in the patients with blepharoptosis. Moderate to severe LP occurred more commonly in all forms of blepharoptosis compared with normal eyes, with more frequent and more severe LP demonstrated in eyes with congenital blepharoptosis.

Pigmentary disorders in the South East.

In this article, new information is introduced regarding vitiligo and melasma based on clinical studies of Korean patients and specific pigmentary disorders that occur in Asians. These disorders can be psychologically distressing because of their visible nature. They are especially resistant to various kinds of conventional treatments and tend to have a chronic progression that makes patients doubt the results and the prognosis.