Juvenile Dermatomyositis: Advances in Pathogenesis, Assessment, and Management.

BACKGROUND: Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes. OBJECTIVE: The purpose of this article is to provide readers with an update on the evaluation, diagnosis, and the treatment of juvenile dermatomyositis. METHODS: A PubMed search was performed in Clinical Queries using the key term "juvenile dermatomyositis" in the search engine. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); the presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is a high-dose corticosteroid (oral or intravenous) in combination with methotrexate. CONCLUSION: For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited, and their use in the treatment of juvenile dermatomyositis is considered investigational.

Infantile Hemangioma: An Updated Review.

BACKGROUND: Infantile hemangiomas are the most common vascular tumors of infancy, affecting up to 12% of infants by the first year of life. OBJECTIVE: To familiarize physicians with the natural history, clinical manifestations, diagnosis, and management of infantile hemangiomas. METHODS: A Pubmed search was conducted in November 2019 in Clinical Queries using the key term "infantile hemangioma". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 20 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: The majority of infantile hemangiomas are not present at birth. They often appear in the first few weeks of life as areas of pallor, followed by telangiectatic or faint red patches. Then, they grow rapidly in the first 3 to 6 months of life. Superficial lesions are bright red, protuberant, bosselated, or with a smooth surface, and sharply demarcated. Deep lesions are bluish and dome-shaped. Infantile hemangiomas continue to grow until 9 to 12 months of age, at which time the growth rate slows down to parallel the growth of the child. Involution typically begins by the time the child is a year old. Approximately 50% of infantile hemangiomas will show complete involution by the time a child reaches age 5; 70% will have disappeared by age 7; and 95% will have regressed by 10 to 12 years of age. The majority of infantile hemangiomas require no treatment. Treatment options include oral propranolol, topical timolol, and oral corticosteroids. Indications for active intervention include hemorrhage unresponsive to treatment, impending ulceration in areas where serious complications might ensue, interference with vital structures, life- or function-threatening complications, and significant disfigurement. CONCLUSION: Treatment should be individualized, depending upon the size, rate of growth, morphology, number, and location of the lesion (s), existing or potential complications, benefits and adverse events associated with the treatment, age of the patient, level of parental concern, and the physician's comfort level with the various treatment options. Currently, oral propranolol is the treatment of choice for high-risk and complicated infantile hemangiomas. Topical timolol may be considered for superficial infantile hemangiomas that need to be treated and for complicated infantile hemangiomas in patients at risk for severe adverse events from oral administration of propranolol.

Acute Hemorrhagic Edema of Infancy: A Diagnostic Challenge for the General Pediatrician.

BACKGROUND: Acute hemorrhagic edema of infancy (AHEI), a benign and self-limited disease, can be easily mistaken to be a number of diseases with similar dermatological manifestations but with potentially adverse outcomes. OBJECTIVE: This review aimed to familiarize pediatricians with the natural history, clinical manifestations, diagnosis, and management of AHEI. METHODS: A PubMed search was conducted in February 2020 in Clinical Queries using the key terms "acute hemorrhagic edema of infancy" OR "Finkelstein disease" OR "Seidlmayer disease". The search strategy included meta-analyses, randomized controlled trials, 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: AHEI, a rare cutaneous leukocytoclastic small-vessel vasculitis, typically presents with palpable purpura, peripheral acral edema, and frequently with fever, most often in children between 4 and 24 months of age. A significant number of children experience prodromal symptoms of an upper respiratory infection. Fever is typically low grade and is present in approximately 50% of cases. The cutaneous lesions are characterized by rapid onset of small erythematous macules or papules that progress to well demarcated, annular, rosette, medallion-like, or targetoid purpuric plaques or ecchymosis in 24 to 48 hours. The skin lesions are typically palpable, nonpruritic, and symmetrically distributed. Sites of predilection include the face, auricles, and extremities. Edema is typically nonpitting and asymmetrical and occurs primarily on the dorsum of the hands and feet, the face, and the auricles. In spite of the acuteness and extent of the cutaneous findings, the child looks well and nontoxic. Systemic and/or visceral involvement are rare. The differential diagnosis is broad and includes, among others, Henoch-Schönlein purpura. It is crucial to distinguish AHEI from the other diseases since the management of these diseases is quite different. The clinical features of mimickers of AHEI are reviewed and clues to differentiate AHEI from these mimickers are highlighted..AHEI is a benign, self-limited disease with complete spontaneous recovery in one to three weeks in the majority of cases. CONCLUSION: Recognizing this rare disease is important for the pediatrician to rapidly differentiate AHEI from other potentially serious diseases that require prompt therapy and monitoring. With rapid recognition of AHEI, unnecessary investigations and inappropriate interventions can be prevented and parental anxiety can be avoided.

Tinea Capitis: An Updated Review.

BACKGROUND: Tinea capitis is a common and, at times, difficult to treat, fungal infection of the scalp. OBJECTIVE: This article aimed to provide an update on the evaluation, diagnosis, and treatment of tinea capitis. METHODS: A PubMed search was performed in Clinical Queries using the key term "tinea capitis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article. Patents were searched using the key term "tinea capitis" at www.freepatentsonline.com. RESULTS: Tinea capitis is most often caused by Trichophyton tonsurans and Microsporum canis. The peak incidence is between 3 and 7 years of age. Non-inflammatory tinea capitis typically presents as fine scaling with single or multiple scaly patches of circular alopecia (grey patches); diffuse or patchy, fine, white, adherent scaling of the scalp resembling generalized dandruff with subtle hair loss; or single or multiple patches of well-demarcated area (s) of alopecia with fine-scale, studded with broken-off hairs at the scalp surface, resulting in the appearance of "black dots". Inflammatory variants of tinea capitis include kerion and favus. Dermoscopy is a highly sensitive tool for the diagnosis of tinea capitis. The diagnosis can be confirmed by direct microscopic examination with a potassium hydroxide wetmount preparation and fungal culture. It is desirable to have mycologic confirmation of tinea capitis before beginning a treatment regimen. Oral antifungal therapy (terbinafine, griseofulvin, itraconazole, and fluconazole) is considered the gold standard for tinea capitis. Recent patents related to the management of tinea capitis are also discussed. CONCLUSION: Tinea capitis requires systemic antifungal treatment. Although topical antifungal therapies have minimal adverse events, topical antifungal agents alone are not recommended for the treatment of tinea capitis because these agents do not penetrate the root of the hair follicles deep within the dermis. Topical antifungal therapy, however, can be used to reduce transmission of spores and can be used as adjuvant therapy to systemic antifungals. Combined therapy with topical and oral antifungals may increase the cure rate.

Scabies: A Neglected Global Disease.

BACKGROUND: Scabies is a skin disease caused by an obligate human parasite mite Sarcoptes scabiei var. hominis. Children under the age of two and elderly individuals are at the greatest risk. Knowledge of this condition is important for an early diagnosis to be made and treatment to be initiated. OBJECTIVE: The review aimed to familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of scabies. METHODS: A search was conducted using Pubmed with the built-in "Clinical Queries" tool. The search term "Scabies" was used. The categories of "epidemiology", "diagnosis", "therapy", "prevention" and "prognosis" had a limited scope for primary clinical studies. Meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews were included. Only papers published in the English language were included. A descriptive, narrative synthesis was provided of the retrieved articles. RESULTS: Worldwide, scabies affects 200 to 300 million individuals annually. The average prevalence is estimated to be 5 to 10% in children of developing countries. Transmission usually occurs after close prolonged skin-to-skin contact. Classic scabies is characterized by an erythematous papular eruption, serpiginous burrows, and intense pruritus. Sites of predilection include the webs of the fingers, volar wrists, lateral aspects of fingers, extensor surfaces of elbows and knees, waist, navel, abdomen, buttocks, groins, and, genitals. A clinical diagnosis of classic scabies can be made on the basis of the history and clinical findings. Other clinical variants include crusted scabies, nodular scabies, and bullous scabies. Finding the mite, ova, or fecal pellets on microscopic examination of scrapings taken from skin lesions confirms the diagnosis of scabies infestation. For eradication of scabies mites, the drugs of choice are topical permethrin and oral ivermectin. CONCLUSION: Scabies is a highly contagious parasitic cutaneous disease that is stigmatising and debilitating. Increased awareness, accurate diagnosis, and prompt treatment are essential for the effective control of scabies and for the prevention of the spread of the disease.

Onychomycosis: An Updated Review.

BACKGROUND: Onychomycosis is a common fungal infection of the nail. OBJECTIVE: The study aimed to provide an update on the evaluation, diagnosis, and treatment of onychomycosis. METHODS: A PubMed search was completed in Clinical Queries using the key term "onychomycosis". The search was conducted in May 2019. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 20 years. The search was restricted to English literature. Patents were searched using the key term "onychomycosis" in www.freepatentsonline.com. RESULTS: Onychomycosis is a fungal infection of the nail unit. Approximately 90% of toenail and 75% of fingernail onychomycosis are caused by dermatophytes, notably Trichophyton mentagrophytes and Trichophyton rubrum. Clinical manifestations include discoloration of the nail, subungual hyperkeratosis, onycholysis, and onychauxis. The diagnosis can be confirmed by direct microscopic examination with a potassium hydroxide wet-mount preparation, histopathologic examination of the trimmed affected nail plate with a periodic-acid-Schiff stain, fungal culture, or polymerase chain reaction assays. Laboratory confirmation of onychomycosis before beginning a treatment regimen should be considered. Currently, oral terbinafine is the treatment of choice, followed by oral itraconazole. In general, topical monotherapy can be considered for mild to moderate onychomycosis and is a therapeutic option when oral antifungal agents are contraindicated or cannot be tolerated. Recent patents related to the management of onychomycosis are also discussed. CONCLUSION: Oral antifungal therapies are effective, but significant adverse effects limit their use. Although topical antifungal therapies have minimal adverse events, they are less effective than oral antifungal therapies, due to poor nail penetration. Therefore, there is a need for exploring more effective and/or alternative treatment modalities for the treatment of onychomycosis which are safer and more effective.

Tinea Imbricata: An Overview.

BACKGROUND: Tinea imbricata is a chronic superficial mycosis caused mainly by Trichophyton concentricum. The condition mainly affects individuals living in primitive and isolated environment in developing countries and is rarely seen in developed countries. Physicians in nonendemic areas might not be aware of this fungal infection. OBJECTIVE: To familiarize physicians with the clinical manifestations, diagnosis, and treatment of tinea imbricata. METHODS: A PubMed search was completed in Clinical Queries using the key terms "Tinea imbricata" and "Trichophyton concentricum". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, reviews, and case reports. The information retrieved from the above search was used in the compilation of the present article. RESULTS: The typical initial lesions of tinea imbricata consist of multiple, brownish red, scaly, pruritic papules. The papules then spread centrifugally to form annular and/or concentric rings that can extend to form serpinginous or polycyclic plaques with or without erythema. With time, multiple overlapping lesions develop, and the plaques become lamellar with abundant thick scales adhering to the interior of the lesion, giving rise to the appearance of overlapping roof tiles, lace, or fish scales. Lamellar detachment of the scales is common. The diagnosis is mainly clinical, based on the characteristic skin lesions. If necessary, the diagnosis can be confirmed by potassium hydroxide wet-mount examination of skin scrapings of the active border of the lesion which typically shows short septate hyphae, numerous chlamydoconidia, and no arthroconidia. Currently, oral terbinafine is the drug of choice for the treatment of tinea imbricata. Combined therapy of an oral antifungal agent with a topical antifungal and keratolytic agent may increase the cure rate. CONCLUSION: In most cases, a spot diagnosis of tinea imbricata can be made based on the characteristic skin lesions consisting of scaly, concentric annular rings and overlapping plaques that are pruritic. Due to popularity of international travel, physicians involved in patient care should be aware of this fungal infection previously restricted to limited geographical areas.