Paquioniquia congénita tipo I o síndrome de Jadassohn- Lewandowsky: un caso de presentación familiar / Congenital pachyonychia type I or Jadassohn-Lewandowsky syndrome: a familial case

La paquioniquia congénita es una rara genodermatosis de herencia autosómica dominante, que produce alteración de la queratinización a nivel de piel, uñas y mucosas. El primer caso fue descrito por Jadassohn y Lewandowsky en 1906, y actualmente afecta a miles de personas en el mundo. El Registro Internacional de Investigación de Paquioniquia Congénita (IPCRR, del inglés, International PC Research Registry) ha identificado a nivel mundial hasta enero de 2020, 977 individuos en 517 familias con paquioniquia congénita, confirmadas genéticamente. Esta condición es considerada en nuestro país como una enfermedad huérfana, y previamente solo hay un reporte en la literatura de un caso en 2009. Se presenta el caso clínico de un paciente masculino de 23 años de edad procedente del área rural del municipio de Timbío, Colombia, con historia clínica de distrofia ungueal hipertrófica en todas las uñas de manos y pies, queratodermia palmoplantar y dolor plantar moderado a severo. Además, presentaba leucoqueratosis oral, hiperqueratosis folicular en muslos e hiperhidrosis palmoplantar. No presentaba quistes foliculares ni antecedente de dientes natales. Sus manifestaciones clínicas se catalogaron como propias de la paquioniquia congénita tipo I. Sumado a lo anterior, se identificaron características clínicas similares en otros miembros de la familia en tres generaciones, incluidos un hermano, la madre y la abuela materna, lo cual evidenció un patrón de herencia propio de esta enfermedad.

Pachyonychia congenita is a rare genodermatosis of autosomal dominant inheritance pattern that affects keratinization at the level of skin, nails and mucous membranes. The first case was described by Jadassohn and Lewandowsky in 1906 and it affects thousands of people around the world. The International Pachyonychia Congenita Research Registry (IPCRR) has identified until January 2020, worldwide, 977 individuals in 517 families with pachyonychia congenita genetically confirmed. This condition is considered in our country as an orphan disease and there has only been one previous case report in 2009. We present a case of a 23-year old male patient from the rural area of Timbio, Colombia, with a history of hypertrophic nail dystrophy in all nails and toenails, palmoplantar keratoderma and moderate plantar pain. In addition, he presented oral leucokeratosis, follicular hyperkeratosis in the thighs, and palmoplantar hyperhidrosis. No follicular cysts were present nor history of natal teeth. Clinical manifestations were classified as typical of congenital pachyonychia type I. Furthermore, similar clinical manifestations were identified in other family members from three generations, including his brother, mother and maternal grandmother, a characteristic inheritance pattern of this illness.

Abnormalities of the hallux skin and nail in the course of very rare arteriovenous malformation / Anormalidades de unha e pele do hálux no decorrer de malformação arteriovenosa muito rara

Abstract Arteriovenous malformations (AVMs) are usually found in the pelvic area and the brain. These vascular anomalies are rarely reported in the toes. AVMs in the toes may be asymptomatic, but can also cause atypical symptoms. Congenital AVMs can expand as patients age and manifest in adulthood. They may be provoked by injury. Acquired AVM might be caused by iatrogenic factors, venous or arterial catheterization, percutaneous invasive vascular procedures, surgery, or degenerative vascular disorders. An AVM can damage surrounding tissues and can cause destruction of skin, nails and bones. The course of the disease is often unpredictable and diagnosis is usually delayed as a result.

Resumo As malformações arteriovenosas (MAVs) são geralmente encontradas na região pélvica e no cérebro.. Essas anomalias vasculares raramente são relatadas nos dedos dos pés. A MAV nesse local pode ser assintomática ou apresentar sintomas atípicos. MAVs congênitas podem evoluir com a idade e se manifestar na idade adulta. O fator provocante pode ser uma lesão traumática. Uma MAV adquirida pode ser causada por fatores iatrogênicos, cateterismo venoso e arterial, procedimentos percutâneos vasculares invasivos, cirurgias e alterações degenerativas vasculares. A MAV pode danificar tecidos adjacentes e pode causar destruição de pele, unhas e ossos. O curso da doença é muitas vezes imprevisível, e como resultado, atrasar o diagnóstico.

Nail Plate and Bed Reconstruction for Pincer Nail Deformity

Pincer nail deformity is a severe condition in which the nail bed becomes compressed and the nail shows an overcurvature. We retrospectively analyzed 13 pincer nail deformities treated using our nail plate and bed reconstruction technique. Visual analogue scale scores, the width of nail root, width of nail tip, height of nail tip, width index, and height index were assessed before and after surgery. The overcurvature was corrected after detachment of the nail plate. The nail fold was pushed underneath the nail plate and then fixed. The width of nail tip significantly increased after surgery (p < 0.05) and was maintained during follow-up. The height of nail tip decreased after surgery (p < 0.05). This nail plate and bed reconstruction technique is a simple and quick surgical method for correcting deformities and reduces risks of complications such as skin necrosis and infection compared to other existing surgical techniques. We recommend this efficient surgical technique for the treatment of pincer nails.

Pigmented Onychomatricoma Showing a Longitudinal Melanonychia: A Case Report and Brief Review of Literature

No abstract available.

Do you know this syndrome? Nail patela syndrome: a pathognomonic dermatologic finding

Abstract: The nail-patella syndrome involves a clinical tetrad of changes in the nails, knees, elbows and the presence of iliac horns. Nail changes are the most constant feature: absent, hypoplastic, or dystrophic. A pathognomonic finding is the presence of the triangular lunula. The diagnosis of nail-patella syndrome is based on clinical findings. In this paper we will discuss a case report of this syndrome and its relation with a dermatological finding.

Comparative microscopic analysis of nail clippings from patients with cutaneous psoriasis and psoriatic arthritis

Abstract: BACKGROUND: The nail involvement in psoriasis is related to psoriatic arthritis and may represent a predictor of the disease. OBJECTIVES: To analyze, through nail clipping, clinically normal and dystrophic nails of patients with cutaneous psoriasis and psoriatic arthritis. METHODS: This is a cross-sectional multicenter study, conducted between August 2011 and March 2012. Patients were divided into four groups: patients with cutaneous psoriasis and onychodystrophy, patients with cutaneous psoriasis and clinically normal nails, patients with psoriatic arthritis and onychodystrophy and patients with psoriatic arthritis and clinically normal nails. We calculated NAPSI (Nail Psoriasis Severity Index) of the nail with more clinically noticeable change. After collection and preparation of the nail clipping, the following microscopic parameters were evaluated: thickness of the nail plate and subungual region, presence or absence of parakeratosis, serous lakes, blood, and fungi. RESULTS: There were more layers of parakeratosis (p=0.001) and a greater thickness of the subungual region in patients with cutaneous psoriasis and onychodystrophy (p=0.002). Serous lakes were also more present in the same group (p=0.008) and in patients with psoriatic arthritis and normal nails (p=0.047). The other microscopic parameters showed no significant difference between normal and dystrophic nails or between patients with psoriatic arthritis or cutaneous psoriasis. STUDY LIMITATIONS: Small sample size and use of medications. CONCLUSIONS: Nail clipping is a simple and quick method to assess the nails of patients with nail psoriasis although does not demonstrate difference between those with joint changes or exclusively cutaneous psoriasis.

Unusual facies with delayed development and multiple malformations in a 14-month-old boy / 中国当代儿科杂志

Schinzel-Giedion syndrome is a rare autosomal dominant genetic disease and has the clinical features of severe delayed development, unusual facies, and multiple congenital malformations. In this case report, a 14-month-old boy had the clinical manifestations of delayed development, unusual facies (prominent forehead, midface retraction, hypertelorism, low-set ears, upturned nose, and micrognathia), and multiple congenital malformations (including cerebral dysplasia, dislocation of the hip joint, and cryptorchidism). The karyotype analysis and copy number variations showed no abnormalities, and whole exon sequencing showed a de novo heterozygous missense mutation, c.2602G > A (p. D868N), in SETBP1 gene. Therefore, the boy was diagnosed with Schinzel-Giedion syndrome. Myoclonic seizures in this boy were well controlled by sodium valproate treatment, and his language development was also improved after rehabilitation treatment. Clinical physicians should improve their ability to recognize such rare diseases, and Schinzel-Giedion syndrome should be considered for children with unusual facies, delayed development, and multiple malformations. Gene detection may help with the diagnosis of this disease.

Nail abnormalities in patients with vitiligo

Abstract: Background: Vitiligo is an acquired pigmentary skin disorder affecting 0.1-4% of the general population. The nails may be affected in patients with an autoimmune disease such as psoriasis, and in those with alopecia areata. It has been suggested that nail abnormalities should be apparent in vitiligo patients. Objective: We sought to document the frequency and clinical presentation of nail abnormalities in vitiligo patients compared to healthy volunteers. We also examined the correlations between nail abnormalities and various clinical parameters. Methods: This study included 100 vitiligo patients and 100 healthy subjects. Full medical histories were collected from the subjects, who underwent thorough general and nail examinations. All nail changes were noted. In the event of clinical suspicion of a fungal infection, additional mycological investigations were performed. Results: Nail abnormalities were more prevalent in the patients (78%) than in the controls (55%) (p=0.001). Longitudinal ridging was the most common finding (42%), followed by (in descending order): leukonychia, an absent lunula, onycholysis, nail bed pallor, onychomycosis, splinter hemorrhage and nail plate thinning. The frequency of longitudinal ridging was significantly higher in patients than in controls (p<0.001). Conclusions: Nail abnormalities were more prevalent in vitiligo patients than in controls. Systematic examination of the nails in such patients is useful because nail abnormalities are frequent. However, the causes of such abnormalities require further study. Longitudinal ridging and leukonychia were the most common abnormalities observed in this study.

Nail Changes in Patients with Alopecia Areata: Clinical Presentation and Associated Factors / 대한피부과학회지

BACKGROUND: Hair and nails are known to be related in their origin and anatomical structures, and they are similar in their structure and growth pattern. Nail changes were first reported to be associated with alopecia areata (AA) in 1898. One of the clinical features of AA is nail abnormalities, and these are found in 7% to 66% of patients with AA. OBJECTIVE: We evaluated the frequency, clinical presentation, and associated factors of nail changes in patients with AA. METHODS: A prospective study of 121 patients with AA was performed. The diagnosis of AA was made on clinical grounds. Patients' medical history was obtained, and thorough examinations were performed. Patients with other diseases associated with nail changes were all excluded from the study. The association between the clinical features of patients with AA and the prevalence of nail changes was evaluated using logistic regression analysis. RESULTS: Of the 121 patients, 40 (33%) presented with nail changes. In multivariable analysis, younger age (compared with ≥19 years old; odds ratio [OR] 3.663, 95% confidence interval [CI] 1.094~12.346, p=0.035), chronicity (disease duration ≥2 years; OR 2.852, 95% CI 1.230~6.613, p=0.015), and disease subtype (alopecia totalis and alopecia universalis); OR 5.846, 95% CI 1.078~31.717, p=0.041) were identified as independent factors associated with nail changes in patients with AA. Among the nail abnormalities identified, nail pitting was the most common, observed in 20 patients (50%). Trachyonychia was present in 7 patients (16%), leukonychia in 7 (16%), and Beau line in 5 (11%). Other changes were also observed, including melanonychia, onycholysis, absence of lunula, and onychoschizia. CONCLUSION: In conclusion, younger age, chronicity, and disease subtype (alopecia totalis and alopecia universalis) were shown to be independent relevant indicators of the nail changes in patients with AA.

Syndrome in question

Abstract The Iso-Kikuchi Syndrome is a rare condition characterized by nail dysplasia involving the index fingers, including micronychia, polyonychia, anonychia, irregular lunula, malalignment and hemionychogryphosis. On the antero-posterior image, radiologic examination reveals a narrowing of the distal phalanx. The lateral image shows a Y-shaped bifurcation of the distal phalanx. We report a case of a patient with typical clinical and radiologic signs of Iso-Kikuchi Syndrome.

Epidermolysis Bullosa Pruriginosa with Nail Dystrophy: A Rare Case Report.

Epidermolysis bullosa (DEB) pruriginosa is a type of dystrophic DEB wherein there is a mutation in gene COL VII A1, which encodes anchoring fibril protein Type VII collagen. Clinically, it is characterized by intensely pruritic linear lichenified or nodular prurigo like lesions over extremities with milia, nail dystrophy, and in some cases albopapuloid lesions over trunk. Here we report a case of an adult onset DEB pruriginosa with typical clinical features which was confirmed by histopathology. In any severely itchy skin lesion over pretibial region, DEB pruriginosa should be kept in mind, and DEB pruriginosa can occur for the first time in adulthood also.

Dyskeratosis congenita without oral involvement: a rare hereditary disease

Dyskeratosis congenita [DC] is a rare, progressive, multi-system, inherited disorder of telomere biology, first described in 1906 as the Zinsser-Engman-Cole syndrome. The condition presents with the classic triad of nail dystrophy, reticulate skin pigmentation, and oral leukoplakia. Variable somatic abnormalities may be present; these include pulmonary, gastrointestinal, genitourinary, cerebral, and dental complications. Pancytopenia eventually develops, progressing to bone marrow failure. In our patient, the progression of DC did not follow the [classic triad] typically seen in the condition. Here we report the case of a 27-year-old male who had features consistent with this syndrome but who had a normal oral cavity associated with aplastic anemia for which longstanding nail dystrophy and reticular pigmentation were the dominant clinical features. The patient was advised to avoid smoking and severe exposure to the sun, and to return regularly for follow-up. Because all symptoms of this disorder are not necessarily seen collectively in any one patient, physicians should consider it as a probability in all patients showing signs of reticular hyperpigmentation, especially accompanied by aplastic anemia. Our report emphasizes the fact that not all components of the syndrome need to be present and in all cases of genodermatosis, pleomorphism may be expected with variable clinical manifestations. Even though many variants have been described in literature, sparing of the oral cavity as in our patient is extremely uncommon. Therefore, physicians should be aware of the mucocutaneous manifestation of DC and its diagnosis and refer the patient for better evaluation

Nail alterations in cutaneous B-cell lymphoma with extracutaneous relapse

Introduction: Cutaneous B-Cell lymphoma can be associated with clinically significant nail alterations, the presentation of which can be unusual and misleading. To date, only a few reports have demonstrated direct specific tumor infiltration of the nail bed with lymphoma, while there is much less information about infiltration of nail bed with diffuse large B-cell lymphoma

Case Presentation: We describe the case of a 74 years old man presenting with nail bed ulceration who developed clinically significant nail shape alterations. Light microscopy studies demonstrated the presence of a specific lymphocyte infiltrate within the nail bed. The IHC analysis demonstrated diffuse large B cell lymphoma [DLBCL]

Conclusions: Specific nail involvement should be recognized and considered in all patients with cutaneous B-cell lymphomas specially when there is refractory to conventional treatment

A Case of Localized Darier's Disease / 대한피부과학회지

Localized Darier's disease (DD) is a rare variant of DD. The disease is characterized by multiple hyperkeratotic papules in a unilateral, linear, zosteriform or Blaschkoid distribution with the histological features of classical DD. Unlike DD, which presents as a generalized condition, localized DD lacks family history and other clinical findings suggestive of DD such as distinctive nail abnormalities and keratotic papules on the palms and soles. Herein, we describe a case of localized DD in a 31-year old Korean man on the perianal area that was treated with topical retinoid cream.

45,X/46,XY mosaicism: report on 14 patients from a Brazilian hospital. A retrospective study / Mosaicismo 45,X/46,XY: relato de 14 pacientes de um hospital do Brasil. Um estudo retrospectivo

CONTEXT AND OBJECTIVE: 45,X/46,XY mosaicism, or mixed gonadal dysgenesis, is considered to be a rare disorder of sex development. The aim of our study was to investigate the clinical and cytogenetic characteristics of patients with this mosaicism. DESIGN AND SETTING: A retrospective study in a referral hospital in southern Brazil. METHODS: Our sample consisted of patients diagnosed at the clinical genetics service of a referral hospital in southern Brazil, from 1975 to 2012. Clinical and cytogenetic data were collected from the medical records. RESULTS: Fourteen patients were included in the sample, with ages at the first evaluation ranging from 2 days to 38 years. Nine of them had female sex of rearing and five, male. Regarding the external genitalia, most were ambiguous (n = 10). One patient presented male phenotype and was treated for a history of azoospermia, while three patients presented female phenotype, of whom two had findings of Turner syndrome and one presented secondary amenorrhea alone. Some findings of Turner syndrome were observed even among patients with ambiguous genitalia. None presented gonadal malignancy. One patient underwent surgical correction for genital ambiguity and subsequent exchange of sex of rearing. Regarding cytogenetics, we did not observe any direct correlation between percentages of cell lines and phenotype. CONCLUSIONS: 45,X/46,XY mosaicism can present with a wide variety of phenotypes resulting from the involvement of different aspects of the individual. All these observations have important implications for early recognition of these patients and their appropriate management. .

CONTEXTO E OBJETIVO: O mosaicismo 45,X/46,XY, ou disgenesia gonadal mista, é considerado uma doença rara do desenvolvimento sexual. O objetivo do nosso estudo foi verificar as características clínicas e citogenéticas de pacientes com este mosaicismo. TIPO DE ESTUDO E LOCAL: Estudo retrospectivo em um hospital de referência no sul do Brasil. MÉTODOS: Nossa amostra foi composta por pacientes diagnosticados em um serviço de genética clínica de um hospital de referência no sul do Brasil, no período de 1975 até 2012. Os dados clínicos e citogenéticos foram coletados a partir dos prontuários médicos. RESULTADOS: Catorze pacientes foram incluídos na amostra, idades na primeira avaliação variando de 2 dias a 38 anos. Nove deles apresentavam sexo feminino de criação e cinco, masculino. A genitália externa, na maioria, era ambígua (n = 10). O paciente com fenótipo masculino foi tratado por história de azoospermia, enquanto que das três pacientes do fenótipo feminino, duas apresentavam achados da síndrome de Turner e a outra, amenorreia secundária isolada. Alguns achados da síndrome de Turner foram observados mesmo entre pacientes com genitália ambígua. Nenhum deles apresentou neoplasia gonadal. Um paciente foi submetido à correção cirúrgica de ambiguidade genital e posterior troca de sexo de criação. Quanto à citogenética, não observamos correlação direta entre a porcentagem de linhas de células e o fenótipo. CONCLUSÕES: O mosaicismo 45,X/46,XY pode apresentar grande variedade de fenótipos resultantes do envolvimento de diferentes aspectos do indivíduo. Todas essas observações têm implicações importantes para o reconhecimento precoce desses pacientes e seu adequado manejo. .

Síndrome de Jackson-Lawler / Jackson-Lawler sydrome: a case report

El síndrome de Jackson-Lawler (paquioniquia congénita tipo 2) es una rara genodermatosis, de carácter autosómico dominante, se debe a mutación de genes de citoqueratinas. Clinicamente se caracteriza por onicodistrofia congénita de las veinte uñas asociada al desarrollo de queratodermia palmoplantar, esteatocistomas múltiples, queratosis folicular, hiperhidrosis palmo-plantar, leucoqueratosis oral. Se presenta caso de mujer de 23 años de edad, sin antecedentes personales y familiares, que acude por alteraciones morfológicas en uñas de manos y pies, asociado a sudoración excesiva en palmas y plantas, aparición de lesiones en axilas, para lo cual se le realiza biopsia infromada como esteatocistomas. Se le diagnóstica sindrome de Jackson-Lawler.

Jackson-Lawler syndrome (pachyoncychia congenital type 2) is a rare genodermatosis of autosomal dominant mutation is due to genes of citokeratins. Clinically it is characterized by congenital onychodystrophy associated with the development of: palmoplantar keratderma, steatocystomas multiplex, follicular leratosis, palmoplantar hyperhidrosis, oral leucoqueratosis. We report a 23 year-old girl, without personal and family history, presented with morphological changes in fingers and feet nails, associated with excessive sweating in palms and soles and axilar lesions informed as steatocystoma after the histopathological study. We diagnose a Jackson-Lawler syndrome.

A Case of Acquired Idiopathic Pterygium Inversum Unguis

Pterygium inversum unguis (PIU) is a rare nail abnormality in which the distal nail bed adheres to the ventral surface of the nail plate, with obliteration of the distal groove. Because of the rarity of this condition, its exact origin is unknown. This disorder can be either congenital or acquired, with or without a family history. The acquired forms may be idiopathic or secondary to systemic connective tissue diseases or other causes such as stroke, neurofibromatosis, leprosy, or the use of nail fortifiers. We present an unusual case of acquired idiopathic PIU of the 10 fingernails in a 22-year-old man.

Onicomatricoma: tumor raro do aparelho ungueal: relato de três casos / Onychomatricoma: a rare tumor of the nail apparatus: report of three cases

Os onicomatricomas são tumores benignos raros que se originam a partir da matriz ungueal e do estroma subjacente. São geralmente assintomáticos e de crescimento lento. Acometem igualmente homens e mulheres de meia idade, comprometendo com maior frequência os dígitos das mãos. As características clínicas fundamentais para o diagnóstico são: faixa longitudinal amarelada de espessura variável, estilhaços hemorrágicos, estrias longitudinais associadas à hipercurvatura transversal e projeções digitiformes emergentes da matriz ungueal. Os autores relatam três casos desse tumor acometendo pododáctilos, enfatizando seus principais aspectos clínicos, achados dermatoscópicos e tratamento cirúrgico.

Onychomatricomas are rare benign tumors originating from the nail matrix and underlying stroma. They are usually asymptomatic and slow growing, affecting both middle-aged men and women, and more frequently involve the digits of the hands. Key clinical features for diagnosis are: yellowish longitudinal band of variable width, splinter hemorrhages, longitudinal grooves associated with the transverse overcurvature and fingerlike projections emerging from the nail matrix. The authors report three cases of this tumor affecting toes, emphasizing main clinical aspects, dermoscopic findings, and surgical treatment.

Mimetização Ungueal / A surgical technique for nail mimicry

Nails play important roles in helping individuals pick up small objects and protecting the finger tips. However, since nails are located at the distal portion of the fingers, they are susceptible to injuries that can require their amputation. Here we present our technique for nail mimicry that aimed to provide a better aesthetic result after amputation. Methods: A total of 14 surgical procedures were performed in 10 patients over 5 years. Results: The goal of the surgery was achieved in all cases and no complications were observed. The surgery was repeated in three cases due to unsatisfactory results. Conclusions: Our technique is easy to perform. Patients were highly satisfied with the results since they no longer had the stigma of an amputated finger with a stump, which improved their quality of life...

As unhas cumprem importante papel de auxílio na pinça e proteção das extremidades digitais. Por seu posicionamento distal nos dedos, estão sujeitas a traumas que podem levar a sua amputação. Apresentamos nossa técnica de mimetização ungueal com o objetivo de melhora estética do dedo tratado. Método: 14 procedimentos cirúrgicos foram realizados em 5 anos, beneficiando 10 pacientes. Resultado: em todos os casos o objetivo da cirurgia foi atingido, sem nenhuma complicação observada. Em 3 casos a cirurgia foi repetida, devido ao resultado inicial ser insatisfatório. Conclusão: é uma técnica de fácil execução e que traz ao paciente um grau de satisfação elevado por retirar o estigma de um coto de amputação com melhor aparência para um convívio social...

Clinical patterns of alopecia areata in children in South Jordan

To describe the clinical patterns of alopecia areata in a group of children in Al-Karak City in the South of Jordan. This study was conducted at Prince Ali Ben Al-Hussein Hospital in Al-Karak City during the period June 2011 to May 2012. Patients who presented with alopecia areata and were aged less than 14 years old were included in the study. The diagnosis of alopecia areata was based on clinical grounds and was made by two expert dermatologists. All patients underwent a thorough history and physical examination. Age, gender, age of onset, type of alopecia, extent and duration of the disease, presence of specific signs, associated medical or dermatological conditions and nail involvement were recorded for all patients. Simple statistical analysis [frequencies, means and percentages] was used to describe the study variables. A total number of 58 pediatric patients were included in the study. There were 31 male and 27 female with a ratio of 1.1:1. The age of patients ranged from two to 14 years. The age of onset ranged from one to 13 years [mean = 7 years].The most common age of presentation was in the age group four to eight [34.5%] and eight to 12 [32.8%]. Most patients [77.6%] presented with limited alopecia areata, and 22.4% of patients presented with widespread alopecia areata. The majority of patients [77.6%] presented with primary alopecia areata and 22.4% of patients presented with recurrent alopecia areata. The mean duration of the disease was three months. Patients with primary alopecia areata had a median duration of two months while those with recurrent alopecia areata had a median duration of four months. Atopic dermatitis was found in 5 patients, vitiligo in one patient and thyroid disease in one patient. Severe alopecia areata was noted mainly in male patients [25.9%], younger age groups [66.7% of patients in the age group 0 to 4], patients with recurrent alopecia areata [60%], patients who had ophiasis [87.5%] and in all patients who had nail abnormality. Alopecia areata occurred in 5.9%, 0.6% and 0.2% of first, second and third degree relatives of patients respectively. There was a slight preponderance for male gender in pediatric alopecia areata in South Jordan. Most patients had limited alopecia areata. Severe alopecia areata is associated with male gender, younger age of onset, recurrent alopecia areata, presence of ophiasis and presence of nail involvement. Patients with recurrent disease tend to have a longer duration of their disease. Relatives of patients have a higher frequency of alopecia areata which indicates the important role of genetic factors