DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.
RESULTS: Generally, patients with LCH can be divided into two groups based on the extent of involvement at diagnosis, namely, single-system LCH and multisystem LCH. The involvement may be unifocal or multifocal. Patients with isolated bone lesions typically present between 5 and 15 years of age, whereas those with multisystem LCH tend to present before 5 years of age. The clinical spectrum is broad, ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition. Clinical manifestations include, among others, "punched out" lytic bone lesion, seborrheic dermatitis-like eruption, erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches, and eczematous lesions, diabetes insipidus, hepatosplenomegaly, cytopenias, lymphadenopathy, and an acute fulminant disseminated multisystem condition presenting with fever, skin rash, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. The diagnosis is clinicopathologic, based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue. Positive CD1a, S100, and/or CD207 (Langerin) immunohistochemical staining of lesional cells is required for a definitive diagnosis. Watchful waiting is recommended for patients with skin-only LCH. Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids, topical nitrogen mustard, oral methotrexate, or oral hydroxyurea. The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine. Mercaptopurine is added for patients with risk organ involvements.
CONCLUSIONS: Because of the broad spectrum of clinical manifestations and the extreme diversity of disease, LCH remains a diagnostic dilemma. Morphological identification of LCH cells and positive immunochemical staining with CD1a, S100, and/or CD207 (Langerin) of lesional cells are necessary for a definitive diagnosis.
Objective: This article aimed to provide a narrative updated review on the evaluation, diagnosis, and treatment of tinea corporis.
Methods: A PubMed search was performed with Clinical Queries using the key term 'tinea corporis.' The search strategy included clinical trials, meta-analyses, randomized controlled trials, observational studies, and reviews. The search was restricted to the English language. The information retrieved from the mentioned search was used in the compilation of the present article.
Results: Tinea corporis typically presents as a well-demarcated, sharply circumscribed, oval or circular, mildly erythematous, scaly patch or plaque with a raised leading edge. Mild pruritus is common. The diagnosis is often clinical but can be difficult with prior use of medications, such as calcineurin inhibitors or corticosteroids. Dermoscopy is a useful and non-invasive diagnostic tool. If necessary, the diagnosis can be confirmed by microscopic examination of potassium hydroxide wet-mount preparations of skin scrapings from the active border of the lesion. Fungal culture is the gold standard to diagnose dermatophytosis especially if the diagnosis is in doubt and results of other tests are inconclusive or the infection is widespread, severe, or resistant to treatment. The standard treatment of tinea corporis is with topical antifungals. Systemic antifungal treatment is indicated if the lesion is multiple, extensive, deep, recurrent, chronic, or unresponsive to topical antifungal treatment, or if the patient is immunodeficient.
Conclusion: The diagnosis of tinea corporis is usually clinical and should pose no problem to the physician provided the lesion is typical. However, many clinical variants of tinea corporis exist, rendering the diagnosis difficult especially with prior use of medications, such as calcineurin inhibitors or corticosteroids. As such, physicians must be familiar with this condition so that an accurate diagnosis can be made and appropriate treatment 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.
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.
DATA SOURCES: A PubMed search was completed in Clinical Queries using the key terms "erythema nodosum".
RESULTS: Clinically, erythema nodosum presents with a sudden onset of painful, erythematous, subcutaneous nodules mainly localized to the pretibial areas. Lesions are usually bilateral and symmetrical, ranging from 1 to 5 cm in diameter. Erythema nodosum may be associated with a variety of conditions such as infection, medications, sarcoidosis, pregnancy, inflammatory bowel disease, vaccination, autoimmune disease, malignancy, and miscellaneous causes. The condition is idiopathic in approximately 50% of cases. The diagnosis is mainly clinical with biopsy reserved for atypical cases. To evaluate for the underlying cause, some basic laboratory screening studies are worthwhile in most cases and include a complete blood cell count, erythrocyte sedimentation rate and/or C-reactive protein, throat swab culture, antistreptococcal O titers, and a chest radiograph. Other tests should be individualized, guided by the history and physical examination results. Most cases of erythema nodosum are self-limited and require no treatment. Bed rest and leg elevation are generally recommended to reduce the discomfort. Nonsteroidal anti-inflammatory drugs are the first-line treatment for pain management.
CONCLUSIONS: As erythema nodosum is often a cutaneous manifestation of a systemic disease, a thorough search should be performed to reveal the underlying cause.
OBJECTIVE: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of a solitary cutaneous mastocytoma.
METHODS: A PubMed search was completed in Clinical Queries using the key term "solitary cutaneous mastocytoma". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English language were included. The information retrieved from the above search was used in the compilation of the present article.
RESULTS: Typically, a solitary cutaneous mastocytoma presents as an indurated, erythematous, yellow- brown or reddish-brown macule, papule, plaque or nodule, usually measuring up to 5 cm in diameter. The lesion often has a peau d'orange appearance and a leathery or rubbery consistency. A solitary cutaneous mastocytoma may urticate spontaneously or when stroked or rubbed (Darier sign). Organomegaly and lymphadenopathy are characteristically absent. The majority of patients with skin lesions that erupt within the first two years of life have spontaneous resolution of the lesions before puberty. Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.
CONCLUSION: The diagnosis is mainly clinical, based on the morphology of the lesion, the presence of a positive Darier sign, and the absence of systemic involvement. A skin biopsy is usually not necessary unless the diagnosis is in doubt.
DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key terms "Gianotti-Crosti syndrome" OR "papular acrodermatitis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.
RESULTS: The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks, extensor surfaces of the extremities, and buttocks. There is a sparing of antecubital and popliteal fossae as well as palms, soles, and mucosal surfaces. Although often asymptomatic, the lesions may be mildly to moderately pruritic. Gianotti-Crosti syndrome is most common in children between 1 and 6 years of age. The Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti syndrome. No treatment for Gianotti-Crosti syndrome is necessary because it is self-limited. In an era of vaccine hesitancy and refusal, Gianotti-Crosti syndrome may be important to mention to parents, because it can occur and trigger alarmism.
CONCLUSIONS: Gianotti-Crosti syndrome is mainly a disease of early childhood, characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution. With the advent of more universal vaccination against hepatitis B virus, Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti syndrome. Few cases of post-vaccination Gianotti-Crosti syndrome have been reported. Currently, the emphasis should be placed on its self-limiting attribution.
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.
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.
OBJECTIVE: This article aims to familiarize physicians with the clinical manifestations, evaluation, diagnosis, and management of vitiligo.
METHODS: A Pubmed search was conducted in Clinical Queries using the key term "vitiligo". The search included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English language. The information retrieved from the above search was used in the compilation of the present article.
RESULTS: Approximately one quarter of patients with vitiligo have the onset before 10 years of age. Genetic, immunological, neurogenic and environmental factors may have a role to play in the pathogenesis. Vitiligo typically presents as acquired depigmented, well-demarcated macules/patches that appear milk- or chalk-white in color. Lesions tend to increase in number and enlarge centrifugally in size with time. Sites of predilection include the face, followed by the neck, lower limbs, trunk, and upper limbs. The clinical course is generally unpredictable. In children with fair skin, no active treatment is usually necessary other than the use of sunscreens and camouflage cosmetics. If treatment is preferred for cosmesis, topical corticosteroids, topical calcineurin inhibitors, and narrowband ultraviolet B phototherapy are the mainstays of treatment.
CONCLUSION: The therapeutic effect of all the treatment modalities varies considerably from individual to individual. As such, treatment must be individualized. In general, the best treatment response is seen in younger patients, recent disease onset, darker skin types, and head and neck lesions. Topical corticosteroids and calcineurin inhibitors are the treatment choice for those with localized disease. Topical calcineurin inhibitors are generally preferred for lesions on genitalia, intertriginous areas, face, and neck. Narrowband ultraviolet B phototherapy should be considered in patients who have widespread vitiligo or those with localized vitiligo associated with a significant impact on the quality of life who do not respond to treatment with topical corticosteroids and calcineurin inhibitors.
OBJECTIVE: This article aimed to familiarize pediatricians with clinical manifestations, evaluation, diagnosis, and management of pityriasis rosea.
METHODS: A search was conducted in March 2020 in Pubmed Clinical Queries using the key term " pityriasis rosea". The search strategy included all clinical trials (including open trials, non-randomized controlled trials, and randomized controlled trials), observational studies, and reviews (including narrative reviews and meta-analyses) 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: Pityriasis rosea occurs mainly in individuals between 10 and 35 years of age with a peak during adolescence. Human herpesvirus (HHV)-7 and HHV-6 have been implicated as the causative agents in some patients with pityriasis rosea. A mild prodrome consisting of headaches, fever, malaise, fatigue, anorexia, sore throat, enlarged lymph nodes and arthralgia is present in about 5% of patients. The most common presenting sign, found in approximately 80% of patients, is a "herald" or "mother" patch which is larger and more noticeable than the lesions of the later eruption. A generalized, bilateral, symmetrical eruption develops in approximately 4 to 14 days and continues to erupt in crops over the next 12 to 21 days. Typical lesions are 0.5 to 1 cm, oval or elliptical, dull pink or salmon-colored macules with a delicate collarette of scales at the periphery. The long axes tend to be oriented along the skin lines of cleavage (Langer lines). Lesions on the back may have a characteristic "Christmas tree", whereas lesions on the upper chest may have a V-shaped pattern. There are many conditions that may mimic pityriasis rosea. Pityriasis rosea in the absence of the herald patch and its variants may pose a diagnostic challenge. The typical course is 6 to 8 weeks. In the vast majority of cases, reassurance and symptomatic treatment should suffice. Active intervention may be considered for individuals with severe or recurrent pityriasis rosea and pregnant women with the disease. Treatment options include acyclovir, macrolides (in particular, erythromycin), and ultraviolet phototherapy. If active intervention is needed, there is evidence supporting the use of oral acyclovir to shorten the duration of illness.
CONCLUSION: Pityriasis rosea is a common, acute, self-limiting exanthematous skin disease that primarily affects children and young adults. The condition is characterized by a "herald patch" after which oval erythematous squamous lesions appear along Langer's lines of cleavage on the trunk and proximal extremities, giving it a "Christmas tree" appearance. The disease presenting in its classical form can easily be diagnosed. Clinical variants of the disease may pose a diagnostic challenge for the general pediatrician. Knowledge of the disease is essential to allow prompt diagnosis and to avoid unnecessary investigations.