Lichen Striatus: An Updated Review.

BACKGROUND: Lichen striatus is a benign dermatosis that affects mainly children. This condition mimics many other dermatoses. OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations of lichen striatus to avoid misdiagnosis, unnecessary investigations, unnecessary referrals, and mismanagement of lichen striatus. METHODS: A search was conducted in June 2023 in PubMed Clinical Queries using the key term "Lichen striatus". The search strategy included all observational studies, clinical trials, and reviews published within the past ten years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of this article. RESULTS: Lichen striatus is a benign self-limited T-cell mediated dermatosis characterized by a linear inflammatory papular eruption seen primarily in children. The onset is usually sudden with minimal or absent symptomatology. The eruption in typical lichen striatus consists of discrete, skin- colored, pink, erythematous, or violaceous, flat-topped, slightly elevated, smooth or scaly papules that coalesce to form a dull red, potentially scaly, interrupted or continuous band over days to weeks. Although any part of the body may be involved, the extremities are the sites of predilection. Typically, the rash is solitary, unilateral, and follows Blaschko lines. In dark-skinned individuals, the skin lesions may be hypopigmented at onset. Nails may be affected alone or, more commonly, along with the skin lesions of lichen striatus. The differential diagnoses of lichen striatus are many and the salient features of other conditions are highlighted in the text. CONCLUSION: Lichen striatus is a self-limited condition that often resolves within one year without residual scarring but may have transient post-inflammatory hypopigmentation or hyperpigmentation. As such, treatment may not be necessary. For patients who desire treatment for cosmesis or for the symptomatic treatment of pruritus, a low- to mid-potency topical corticosteroid or a topical immunomodulator can be used. A fading cream can be used for post-inflammatory hyperpigmentation.

Pinworm (Enterobius Vermicularis) Infestation: An Updated Review.

BACKGROUND: Pinworm infestation is an important public health problem worldwide, especially among children 5 to 10 years of age in developing countries with temperate climates. The problem is often overlooked because of its mild or asymptomatic clinical manifestations. OBJECTIVE: The purpose of this article was to familiarize pediatricians with the diagnosis and management of pinworm infestation. METHODS: A search was conducted in August 2023 in PubMed Clinical Queries using the key terms "Enterobius vermicularis," OR "enterobiasis," OR "pinworm." The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Enterobiasis is a cosmopolitan parasitosis caused by Enterobius vermicularis. It affects approximately 30% of children worldwide and up to 60% of children in some developing countries. Predisposing factors include poor socioeconomic conditions, inadequate sanitation, poor personal hygiene, and overcrowding. Children aged 5 to 14 years have shown the highest prevalence of enterobiasis.. Egg transmission is mainly by the fecal-oral route. Approximately 30 to 40% of infested patients do not show any clinical symptoms of the disease. For symptomatic patients, the most common presenting symptom is nocturnal pruritus ani. The diagnosis of E. vermicularis infection is best established by the cellophane tape test. The sensitivity of one single test is around 50%; however, the sensitivity increases to approximately 90% with tests performed on three different mornings. If a worm is visualized in the perianal area or the stool, a pathological examination of the worm will yield a definitive diagnosis. As pinworms and eggs are not usually passed in the stool, examination of the stool is not recommended. The drugs of choice for the treatment of pinworm infestation are mebendazole (100 mg), pyrantel pamoate (11 mg/kg, maximum 1 g), and albendazole (400 mg), all of the above-mentioned drugs are given in a single dose and repeated in two weeks. Mebendazole and albendazole are both adulticidal and ovicidal, whereas pyrantel pamoate is only adulticidal. Given their safety and effectiveness, mebendazole and albendazole are currently the best available drugs for the treatment of pinworm infestation. For pregnant women, pyrantel is preferred to mebendazole and albendazole. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections because reinfection is common even when effective medication is given. CONCLUSION: In spite of effective treatment of pinworm infestation, recurrences are common. Recurrences are likely due to repeated cycles of reinfection (particularly, autoinfection) because of the short life span of adult pinworms. Good personal hygiene, such as frequent handwashing, especially after bowel movements and before meals, clipping of fingernails, avoidance of finger-sucking, nail-biting, and scratching in the anogenital area, are important preventive measures. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections.

Childhood guttate psoriasis: an updated review.

Background: Guttate psoriasis is common and affects 0.5-2% of individuals in the paediatric age group. This review aims to familiarize physicians with the clinical manifestations, evaluation, diagnosis and proper management of guttate psoriasis. Methods: A search was conducted in July 2023 in PubMed Clinical Queries using the key term "guttate psoriasis". The search strategy included all observational studies, clinical trials and reviews published within the past 10 years. The information retrieved from the search was used in the compilation of the present article. Results: Guttate psoriasis typically presents with an abrupt onset of numerous, small, scattered, tear-drop-shaped, scaly, erythematous, pruritic papules and plaques. Sites of predilection include the trunk and proximal extremities. There may be a history of preceding streptococcal infection. Koebner phenomenon is characteristic. Guttate psoriasis may spontaneously remit within 3-4 months with no residual scarring, may intermittently recur and, in 40-50% of cases, may persist and progress to chronic plaque psoriasis. Given the possibility for spontaneous remission within several months, active treatment may not be necessary except for cosmetic purposes or because of pruritus. On the other hand, given the high rates of persistence of guttate psoriasis and progression to chronic plaque psoriasis, some authors suggest active treatment of this condition. Conclusion: Various treatment options are available for guttate psoriasis. Triggering and exacerbating factors should be avoided if possible. Topical corticosteroids alone or in combination with other topical agents (e.g. tazarotene and vitamin D analogues) are the most rapid and efficient treatment for guttate psoriasis and are therefore the first-line treatment for mild cases. Other topical therapies include vitamin D analogues, calcineurin inhibitors, anthralin, coal tar and tazarotene. Ultraviolet phototherapy is the first-line therapy for moderate-to-severe guttate psoriasis, as it is more practical than topical therapy when treating widespread or numerous small lesions. Systemic immunosuppressive and immunomodulatory therapies (e.g. methotrexate, cyclosporine, retinoids, fumaric acid esters and biologics) may be considered for patients with moderate-to-severe guttate psoriasis who fail to respond to phototherapy and topical therapies.

Group A ß-hemolytic streptococcal pharyngitis: An updated review.

BACKGROUND: Group A ß-hemolytic streptococcus (GABHS) is the leading bacterial cause of acute pharyngitis in children and adolescents worldwide. OBJECTIVE: This article aims to familiarize clinicians with the clinical manifestations, evaluation, diagnosis, and management of GABHS pharyngitis. METHODS: A search was conducted in December 2022 in PubMed Clinical Queries using the key term "group A ß-hemolytic streptococcal pharyngitis". This review covers mainly literature published in the previous ten years. RESULTS: Children with GABHS pharyngitis typically present with an abrupt onset of fever, intense pain in the throat, pain on swallowing, an inflamed pharynx, enlarged and erythematous tonsils, a red and swollen uvula, enlarged tender anterior cervical lymph nodes. As clinical manifestations may not be specific, even experienced clinicians may have difficulties diagnosing GABHS pharyngitis solely based on epidemiologic or clinical grounds alone. Patients suspected of having GABHS pharyngitis should be confirmed by microbiologic testing (e.g., culture, rapid antigen detection test, molecular point-of-care test) of a throat swab specimen prior to the initiation of antimicrobial therapy. Microbiologic testing is generally unnecessary in patients with pharyngitis whose clinical and epidemiologic findings do not suggest GABHS. Clinical score systems such as the Centor score and McIssac score have been developed to help clinicians decide which patients should undergo diagnostic testing and reduce the unnecessary use of antimicrobials. Antimicrobial therapy should be initiated without delay once the diagnosis is confirmed. Oral penicillin V and amoxicillin remain the drugs of choice. For patients who have a non-anaphylactic allergy to penicillin, oral cephalosporin is an acceptable alternative. For patients with a history of immediate, anaphylactic-type hypersensitivity to penicillin, oral clindamycin, clarithromycin, and azithromycin are acceptable alternatives. CONCLUSION: Early diagnosis and antimicrobial treatment are recommended to prevent suppurative complications (e.g., cervical lymphadenitis, peritonsillar abscess) and non-suppurative complications (particularly rheumatic fever) as well as to reduce the severity of symptoms, to shorten the duration of the illness and to reduce disease transmission.

Tinea pedis: an updated review.

Background: Tinea pedis is one of the most common superficial fungal infections of the skin, with various clinical manifestations. This review aims to familiarize physicians with the clinical features, diagnosis and management of tinea pedis. Methods: A search was conducted in April 2023 in PubMed Clinical Queries using the key terms 'tinea pedis' OR 'athlete's foot'. The search strategy included all clinical trials, observational studies and reviews published in English within the past 10 years. Results: Tinea pedis is most often caused by Trichophyton rubrum and Trichophyton interdigitale. It is estimated that approximately 3% of the world population have tinea pedis. The prevalence is higher in adolescents and adults than in children. The peak age incidence is between 16 and 45 years of age. Tinea pedis is more common amongst males than females. Transmission amongst family members is the most common route, and transmission can also occur through indirect contact with contaminated belongings of the affected patient. Three main clinical forms of tinea pedis are recognized: interdigital, hyperkeratotic (moccasin-type) and vesiculobullous (inflammatory). The accuracy of clinical diagnosis of tinea pedis is low. A KOH wet-mount examination of skin scrapings of the active border of the lesion is recommended as a point-of-care testing. The diagnosis can be confirmed, if necessary, by fungal culture or culture-independent molecular tools of skin scrapings. Superficial or localized tinea pedis usually responds to topical antifungal therapy. Oral antifungal therapy should be reserved for severe disease, failed topical antifungal therapy, concomitant presence of onychomycosis or in immunocompromised patients. Conclusion: Topical antifungal therapy (once to twice daily for 1-6 weeks) is the mainstay of treatment for superficial or localized tinea pedis. Examples of topical antifungal agents include allylamines (e.g. terbinafine), azoles (e.g. ketoconazole), benzylamine, ciclopirox, tolnaftate and amorolfine. Oral antifungal agents used for the treatment of tinea pedis include terbinafine, itraconazole and fluconazole. Combined therapy with topical and oral antifungals may increase the cure rate. The prognosis is good with appropriate antifungal treatment. Untreated, the lesions may persist and progress.

Erythema Infectiosum: A Narrative Review.

BACKGROUND: Erythema infectiosum occurs worldwide. School-aged children are most often affected. Since the diagnosis is mainly clinical, physicians should be well-versed in the clinical manifestations of erythema infectiosum to avoid misdiagnosis, unnecessary investigations, and mismanagement of the disease. OBJECTIVE: The purpose of this article is to familiarize physicians with the wide spectrum of clinical manifestations and complications of erythema infectiosum associated with parvovirus B19 infection. METHODS: A search was conducted in July 2022 in PubMed Clinical Queries using the key terms " Erythema infectiosum" OR "Fifth disease" OR "Slapped cheek disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Erythema infectiosum is a common exanthematous illness of childhood caused by parvovirus B19. Parvovirus B19 spreads mainly by respiratory tract secretions and, to a lesser extent, the saliva of infected individuals. Children between 4 and 10 years of age are most often affected. The incubation period is usually 4 to 14 days. Prodromal symptoms are usually mild and consist of low-grade fever, headache, malaise, and myalgia. The rash typically evolves in 3 stages. The initial stage is an erythematous rash on the cheeks, with a characteristic "slapped cheek" appearance. In the second stage, the rash spreads concurrently or quickly to the trunk, extremities, and buttocks as diffuse macular erythema. The rash tends to be more intense on extensor surfaces. The palms and soles are typically spared. Central clearing of the rash results in a characteristic lacy or reticulated appearance. The rash usually resolves spontaneously within three weeks without sequelae. The third stage is characterized by evanescence and recrudescence. In adults, the rash is less pronounced than that in children and is often atypical. Only approximately 20% of affected adults have an erythematous rash on the face. In adults, the rash is more frequently found on the legs, followed by the trunk, and arms. A reticulated or lacy erythema is noted in 80% of cases which helps to distinguish erythema infectiosum from other exanthems. Pruritus is noted in approximately 50% of cases. The diagnosis is mainly clinical. The many manifestations of parvovirus B19 infection can pose a diagnostic challenge even to the best diagnostician. Complications include arthritis, arthralgia, and transient aplastic crisis. In most cases, treatment is symptomatic and supportive. When parvovirus B19 infection occurs in pregnant women, hydrops fetalis becomes a real concern. CONCLUSION: Erythema infectiosum, the most common clinical manifestation of parvovirus B19 infection, is characterized by a "slapped cheek" appearance on the face and lacy exanthem on the trunk and extremities. Parvovirus B19 infection is associated with a wide spectrum of clinical manifestations. Physicians should be aware of potential complications and conditions associated with parvovirus B19 infection, especially in individuals who are immunocompromised, chronically anemic, or pregnant.

Bed Bug Infestation: An Updated Review.

In the past decade, there has been a global resurgence of bed bug infestations, especially in developed countries. Proper awareness and identification of bed bug infestations are essential to guide treatment and eradication. The purpose of this article is to familiarize physicians with bed bug bites so that they can effectively diagnose, treat, and address questions about bed bug bites and infestations. Bed bug bites are often painless. Typical reactions include pruritic, erythematous maculopapules occurring in clusters or in a linear or curvilinear distribution in exposed areas of the body. A small red punctum may be visualized at the center of the bite mark. Lesions that appear three in a row and papules on the upper eyelid associated with erythema and edema are highly suggestive of bites from bed bugs. Exaggerated local reactions such as vesicles, urticarial wheals, urticarial perilesional plaques, diffuse urticaria, bullae, and nodules may occur in previously sensitized individuals. Reactions to bed bug bites are self-limited. As such, treatment is mainly symptomatic. Topical pramoxine and oral antihistamines can be used to alleviate pruritus. Topical corticosteroids can be used for significant eruptions to control inflammation and pruritus, and to hasten resolution of the lesions. Integrated pest management, an approach for the eradication of bed bugs, includes monitoring devices (active monitors include the use of heat or carbon dioxide attractants and passive monitors include the use of sticky pads for trapping), and judicious use of nonchemical and chemical treatments known to be effective. Nonchemical interventions include keeping affected areas clean and free of clutter, vacuuming, washing linens with hot water, caulking wall holes and cracks where bugs can hide, proper disposal of highly infested items, and placement of bed bug traps/interceptors at the base of beds and furniture. Chemical interventions involve the use of insecticides such as synthetic pyrethroids, silicates, insect growth disruptors, carbamates, organophosphates, neonicotinoids, diethyl-meta-toluamide, chlorfenapyr, fipronil and plant essential oils. Insecticides should be used with caution to prevent over-exposure and toxicity (in particular, cardiovascular and neurologic toxicity), especially if there are young children around. It is important to note that multiple mechanisms of insecticide resistance exist and as such, chemical treatment should only be undertaken by trained professionals who understand the current literature on resistance. Both nonchemical and chemical technologies should be combined for optimal results. Bed bug infestations may cause diverse dermal reactions, stigmatization, poor self-esteem, emotional stress, anxiety, significant adverse effect on quality of life, and substantial socioeconomic burden to society. As such, their rapid detection and eradication are of paramount importance. Consultation with a professional exterminator is recommended to fully eradicate an infestation.

Infantile vitiligo and alopecia in immunodysregulation polyendocrinopathy enteropathy X-linked syndrome.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is characterized by failure to thrive, severe chronic diarrhea, neonatal type 1 diabetes or thyroiditis, and eczematous dermatitis. We report a patient with infantile onset IPEX syndrome who developed vitiligo, alopecia, and chronic diarrhea. Awaiting stem cell transplant, he had multiple episodes of sepsis and succumbed at the age of 10 months. The constellation of symptoms is important to prompt clinicians to suspect this rare syndrome as early hematopoietic stem cell transplantation is the only cure for IPEX patients.

Poor sleep quality in children with atopic dermatitis and its effects on behavior: A multicenter cross-sectional study from a low-middle-income country.

BACKGROUND: Sleep disturbance in children with atopic dermatitis (AD) frequently goes unnoticed and can be associated with behavioral challenges. The aims of this study were to determine (a) the prevalence and factors associated with sleep disturbance and behavioral problems and (b) the correlation between sleep disturbance and behavioral problems in children with AD. METHODS: This cross-sectional study involved children aged 4-12 years old with moderate to severe AD. Age and sex-matched healthy children were recruited as the comparison group. The Children's Sleep Habits Questionnaire (CSHQ) and the Strengths and Difficulties Questionnaire (SDQ) were used to assess sleep disturbance and behavioral problems, respectively. Higher scores in both questionnaires signify more disturbance. RESULTS: Seventy patients and 141 controls were recruited. Median (interquartile range) age of patients was 5 (4,8) years. Patients had later sleep time (p < 0.001), longer night awakening (p < 0.001), and shorter sleep duration (p < 0.001) compared to controls. CSHQ total scores and all its domains, except for sleep-disordered breathing were significantly higher in patients compared to controls. Patients also had significantly higher SDQ total difficulties scores in all domains, except for peer problems. Severity of AD was significantly associated with high CSHQ and SDQ scores. There was a moderate positive correlation between the total CSHQ score and total SDQ score in patients (r = 0.532). CONCLUSIONS: Children with moderate-to-severe AD had significantly more sleep disturbance and more behavioral problems than their healthy peers. Sleep disturbance had a positive correlation with behavioral problems. Severity of AD was associated with sleep disturbance and behavioral problems.

Tinea versicolor: an updated review.

Background: Tinea versicolor is a common superficial fungal infection of the skin with various clinical manifestations. This review aims to familiarize physicians with the clinical features, diagnosis and management of tinea versicolor. Methods: A search was conducted in July 2022 in PubMed Clinical Queries using the key terms "tinea versicolor" OR "pityriasis versicolor". The search strategy included all clinical trials, observational studies and reviews published within the past 10 years. Results: Tinea versicolor is caused by Malassezia species, notably M. globosa, M. furfur and M. sympodialis. The condition is characterized by scaly hypopigmented or hyperpigmented macules/patches, primarily located on the upper trunk, neck and upper arms. The diagnosis is usually based on characteristic clinical features. If necessary, a potassium hydroxide preparation test can be performed to reveal numerous short, stubby hyphae intermixed with clusters of spores. Most patients with tinea versicolor respond to topical antifungal therapy, which has a better safety profile (fewer adverse events, fewer drug interactions) and lower cost compared to systemic treatment and is therefore the treatment of choice. Oral antifungal therapy is typically reserved for patients with extensive disease, frequent recurrences or disease that is refractory to topical therapy. Advantages of oral antifungal therapy include increased patient compliance, shorter duration of treatment, increased convenience, less time involved with therapy and reduced recurrence rates. On the other hand, oral antifungal therapy is associated with higher cost, greater adverse events and potential drug-drug interactions and is therefore not the first-line treatment for tinea versicolor. Long-term intermittent prophylactic therapy should be considered for patients with frequent recurrence of the disease. Conclusion: Selection of antifungal agents depends on several factors, including efficacy, safety, local availability, ease of administration, likelihood of compliance and potential drug interactions of the antifungal agent.

Roseola Infantum: An Updated Review.

BACKGROUND: Roseola infantum is a common viral disease that occurs during childhood worldwide. OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations, evaluation, diagnosis, and management of roseola infantum. METHODS: A search was conducted in April, 2022, in PubMed Clinical Queries using the key terms "roseola infantum" OR "exanthem subitum" OR "sixth disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Roseola infantum is a viral illness characterized by high fever that lasts 3 to 4 days, followed by the sudden appearance of rash at defervescence. The disease occurs most frequently in children between 6 months and 2 years of age. Human herpesvirus-6 (HHV-6) is the major cause of roseola infantum, followed by HHV-7. Transmission of the infection most likely results from the asymptomatic shedding of the virus in the saliva of the caregivers or other close contacts. Characteristically, the rash is discrete, rose-pink in color, circular or elliptical, macular or maculopapular, measuring 2 to 3 mm in diameter. The eruption is first seen on the trunk. It then spreads to the neck and proximal extremities. Typically, the rash blanches on pressure and subsides in 2 to 4 days without sequelae. Most children look well otherwise and appear to be happy, active, alert, and playful. The diagnosis is mainly clinical. Febrile seizures occur in 10 to 15 % of children with roseola infantum during the febrile period. In general, serious complications are rare and occur more often in individuals who are immunocompromised. There is no specific treatment. An antipyretic may be used to reduce fever and discomfort. CONCLUSION: Roseola infantum is generally a benign and self-limited disease. Failure to recognize this condition may result in undue parental fear, unnecessary investigations, delay in treatment for conditions that mimic roseola infantum and complications from roseola infantum, unnecessary treatment of roseola infantum per se, and misuse of healthcare expenditure.

Hand, Foot, and Mouth Disease: A Narrative Review.

BACKGROUND: Hand, foot, and mouth disease is a common viral disease in childhood. Because the disease has the potential to reach epidemic levels and mortality is high in some countries, early recognition of this disease is of paramount importance. OBJECTIVE: This purpose of this article is to familiarize pediatricians with the clinical manifestations and management of hand, foot, and mouth disease. METHODS: A search was conducted in February 2022 in PubMed Clinical Queries using the key term "hand, foot, and mouth disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in English were included in this review. RESULTS: Hand, foot, and mouth disease is characterized by a painful oral enanthem and asymptomatic exanthem on the palms and soles. Children younger than 5 years are most commonly affected. Hand, foot, and mouth disease caused by enterovirus A71 is more severe and has a higher rate of complications than that attributed to other viruses such as coxsackievirus A16. Circulatory failure secondary to myocardial impairment and neurogenic pulmonary edema secondary to brainstem damage are the main causes of death. Fortunately, the disease is usually benign and resolves in 7 to10 days without sequelae. Given the self-limited nature of most cases, treatment is mainly symptomatic and supportive. Intravenous immunoglobulin should be considered for the treatment of severe/complicated hand, foot, and mouth disease and has been recommended by several national and international guideline committees. Currently, there are no specific antiviral agents approved for the treatment of the disease. Drugs such as ribavirin, suramin, mulberroside C, aminothiazole analogs, and sertraline have emerged as potential candidates for the treatment of hand, foot, and mouth disease. Vaccination of susceptible individuals in high-risk areas and good personal hygiene are important preventative measures to combat the disease. CONCLUSION: Familiarity of the disease including its atypical manifestations is crucial so that a correct diagnosis can be made, and appropriate treatment initiated. A timely diagnosis can help avoid contact with the affected individual and decrease the risk of an outbreak.

Uncovering incontinentia pigmenti: From DNA sequence to pathophysiology.

Incontinentia pigmenti (IP) is an X-linked dominant genodermatosis. The disease is known to be caused by recurrent deletion of exons 4-10 of the Inhibitor Of Nuclear Factor Kappa B Kinase Regulatory Subunit Gamma (IKBKG) gene located at the Xq28 chromosomal region, which encodes for NEMO/IKKgamma, a regulatory protein involved in the nuclear factor kappa B (NF-κB) signaling pathway. NF-κB plays a prominent role in the modulation of cellular proliferation, apoptosis, and inflammation. IKBKG mutation that results in a loss-of-function or dysregulated NF-κB pathway contributes to the pathophysiology of IP. Aside from typical skin characteristics such as blistering rash and wart-like skin growth presented in IP patients, other clinical manifestations like central nervous system (CNS) and ocular anomalies have also been detected. To date, the clinical genotype-phenotype correlation remains unclear due to its highly variable phenotypic expressivity. Thus, genetic findings remain an essential tool in diagnosing IP, and understanding its genetic profile allows a greater possibility for personalized treatment. IP is slowly and gradually gaining attention in research, but there is much that remains to be understood. This review highlights the progress that has been made in IP including the different types of mutations detected in various populations, current diagnostic strategies, IKBKG pathophysiology, genotype-phenotype correlation, and treatment strategies, which provide insights into understanding this rare mendelian disorder.

Efficacy and Tolerability of Methotrexate in the Treatment of Severe Paediatric Alopecia Areata.

Introduction: Alopecia areata (AA) is a chronic, autoimmune condition affecting hair follicles, and its occurrence in the paediatric population is associated with poorer prognosis and limited treatment options compared to adults. Treatment with oral methotrexate (MTX) has been documented in adults, but there is a paucity of data for its use in the paediatric population. We aimed to study the efficacy and tolerability of MTX in severe paediatric AA. Methods: We performed a retrospective review on paediatric patients with severe AA who were treated with MTX in our centre from January 2019 to December 2020. Results: Thirteen patients were included (6 boys and 7 girls) aged between 4 and 16 years at the initiation of MTX (mean age of 8.8 years). The interval from diagnosis of AA to commencement of MTX was between 8 months and 9 years (mean duration of 3.3 years). Oral MTX was administered once weekly with a mean maximal dose of 0.4 mg/kg/dose. Out of 12 assessable patients, 5 were considered treatment success as they had more than 50% regrowth, while the other 7 were treatment failures. No serious side effects were reported. Conclusion: MTX was shown to have variable efficacy for the treatment of paediatric AA with overall good tolerability. MTX can be considered in the treatment of severe refractory AA for children.