Prognosis and Management of Congenital Hair Shaft Disorders with Fragility-Part I.

Hair shaft disorders are characterized by congenital or acquired abnormalities of the hair shaft. The objective was to review the literature regarding the prognosis and treatment options of hair shaft disorders. We used keywords in the search engines PubMed and Medline to identify all publications in the English language related to the prognosis and management of hair shaft disorders. Data were extracted from 96 articles that met search criteria. Findings were limited to case reports and small case series, as no studies were found. Disorders that improve in childhood include pili torti, trichorrhexis invaginata, wooly hair, and pili trianguli et canaliculi. Others, such as trichorrhexis nodosa, monilethrix, pili annulati, and pili bifurcati improve with minoxidil. Oral retinoids have improved hair abnormalities in trichorrhexis invaginata and monilethrix. There is no specific treatment for congenital hair shaft abnormalities. Gentle hair care is the mainstay of care for hair shaft disorders associated with fragility. Practices for gentle care include no brushing, backcombing, chemical products, tight braids, heat exposure, or mechanical grooming. Any inherited or congenital disorder requires genetic counseling as part of management.

Congenital combined melanocytic nevus of the scalp with associated alopecia areata.

We report a case of a 20-year-old Chinese man with an alopecic congenital combined compound and blue melanocytic nevus of the scalp, associated with alopecia areata. The diagnosis of a combined melanocytic nevus was confirmed by histopathological examination and immunohistochemical stains, with exclusion of neurocristic hamartoma, which can have a similar clinical and histopathological appearance but different prognosis. In addition, we explore the association of this large melanocytic lesion with alopecia areata.

Usefulness of dermatoscopy for the early diagnosis of sebaceous naevus and differentiation from aplasia cutis congenita.

In early infancy, a congenital area of alopecia of the scalp may be the clinical presentation of various diseases, with the two most common being sebaceous naevus and aplasia cutis congenita. Typical lesions are easily diagnosed, but soon after birth, the differential diagnosis may be difficult if the clinical features of the lesion are not distinctive. In this paper, we report the usefulness of dermatoscopy in such cases.

Congenital alopecia areata.

Alopecia areata, the alleged autoimmune process leading to nonscarring hair loss, is not uncommon. It has been classified as an acquired cause of alopecia; however, recently it has been reported in the neonatal period. We report 4 cases of congenital alopecia areata with follow-up from 3 to 5 years. The diagnosis was made clinically in all cases. All patients had prolonged periods of quiescence of hair loss ranging from 6 to 24 months. Treatments used included minoxidil 2% and a range of topical steroids including hydrocortisone 1%, betamethasone valerate 0.05%, fluocinonide 0.05%, and clobetasol propionate 0.05%. The best regrowth observed resulted from the use of clobetasol propionate 0.05%, giving full regrowth in 50% of those treated. Alopecia areata can occur at all ages and, thus, can be classified as both an acquired and a congenital disorder resulting in hair loss.

Tratamento da alopecia areata com corticóide tópico: estudo prospectivo randomizado duplo cego em crianças / Therapy of alopecia areata with topical corticosteroid: prospective double-blind controlled assay in children

Na alopecia areata há perda súbita e assintomática de pêlos em qualquer área pilosa do corpo. A etiologia é desconhecida, e os tratamentos propostos não costumam alterar o curso natural e imprevisível da doença. Atinge as crianças, menos responsivas às terapêuticas e mais susceptíveis às formas mais extensas. O estado atópico é considerado fator de pior prognóstico; é provável que a relação entre as duas doenças se deva às origens imunológicas, embora sejam desconhecidos seus pontos de intersecção.O objetivo é avaliar a eficácia do dipropionato de betametasona em creme a 0,05 por cento na alopecia areata da criança e a influência da atopia na resposta terapêutica. Estudo prospectivo controlado duplo cego em 35 crianças de até 12 anos portadoras de alopecia areata, atendidas no Ambulatório de Dermatologia do Instituto de Puericultura e Pediatria Martagão Gesteira - UFRJ e do Hospital Municipal Jesus, ambos no Rio de Janeiro, RJ, no período de 1996 a 1998. Das 35 crianças estudadas, 30 (85,7 por cento) evoluíram com resposta positiva ao final de seis meses de acompanhamento. Os percentuais de resposta foram semelhantes nas terapias utilizadas - dipropionato de betametasona e placebo (p= 0,47). Dos 29 atópicos, 26 (89,6 por cento) tiveram resposta positiva aos tratamentos. Não foi estabelecida correlação estatisticamente significativa entre a resposta terapêutica e a condição atópica (p= 0,19).Não houve diferença entre as respostas terapêuticas nos grupos estudados; a atopia não influenciou a resposta ao tratamento efetuado

Alopecia areata in infants and newborns.

Alopecia areata is a common cause of nonscarring hair loss in children and adults. In newborns and very young infants, however, it is thought to be extremely rare. In this article we describe five cases of alopecia areata in patients less than 6 months of age and briefly discuss the pertinent differential diagnosis of infants and newborns with both patchy and complete hair loss. We propose that alopecia areata may be more common in this age group than the literature suggests.

Congenital alopecia areata.

Alopecia areata has never been documented in a newborn. Thus, it is generally assumed that alopecia areata is acquired only postnatally, and it is believed that the presence of an alopecia at birth virtually excludes its diagnosis. In this report we document a case of alopecia areata in a premature newborn.

Alopecia congénita universal: presentacion de caso

Se presenta un caso clínico de alopesia congénita universal, diagnosticado en un niño de siete años de edad caracterizado por la ausencia completa de pelo en cuero cabelludo, cejas y pestañas; ausencia de vello corporal con distrofia y escaso crecimiento de las uñas de manos y pies. La enfermedad es tipo familiar y hereditario que se transmite generalmente con rasgo dominante, pero a veces en forma recesiva. se puede asociar con trastornos en el crecimiento de la uñas, dedos en membrana, catarata, ataxia de FRIEDERCH, defecto de los dientes y de las glándulas sudoríparas. No tiene tratamiento y desde el punto de vista de su pronóstico, el trastorno es irreversible, no afectando la supervivencia del niño pero sí su estado psíquico por la alteración de su aspecto estético. El diagnóstico diferencial se lo hace con las alteraciones congénitas que se acompañan de Alopecia Universal tales como las displacias ectodérmicas anhidróticas, tipo CLOUSTON, de RAPP y HODHKIN, tipo Robinson, con la progeria de HUTCHINSSON, el síndrome de ROTHMUND-THOMPSON y la acrodermatitis enteropática.

[Alopecia triangularis congenita. Clinical manifestations--surgical correction with autologous hair transplantation]. / Alopecia triangularis congenita. Klinisches Erscheinungsbild--operative Korrektur mit autologer Haartransplantation.

We present the case of a 22-year-old female patient with alopoecia triangularis congenita. Hair transplantation was successfully performed.

Cutaneous and ocular manifestations of Down syndrome.

A patient with Down syndrome and extensive elastosis perforans serpiginosa is presented. There is an increased incidence of elastosis perforans serpiginosa, alopecia areata, syringomas, and Norwegian scabies in patients with this syndrome. Ocular findings include slanting of the palpebral fissures, Brushfield's spots, nystagmus, and epicanthal folds. Serious ocular complications that may impair vision include keratoconus, congenital cataracts, and retinal detachment.

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.

Congenital temporal triangular alopecia.

Congenital temporal triangular alopecia (CTTA) is a condition that occurs very rarely and is often unresponsive to treatment. It is necessary to differentiate it from other conditions such as alopecia areata in order to avoid prolonged treatment which may cause adverse side effects. Two additional cases of CTTA are presented herein. The first case was initially seen eighteen years ago. CTTA was diagnosed after a short period of time, but only after the patient had been mistakenly treated for alopecia areata. In the second case, CTTA was diagnosed during the first examination and no treatment was given. I believe that this condition is not as rare as formerly thought, and I also think that some cases may be misdiagnosed when the physician is not aware that he is presented with a case of CTTA.