OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations, evaluation, diagnosis, and management of acanthosis nigricans.
METHODS: A search was conducted in November 2021in PubMed Clinical Queries using the key term "acanthosis nigricans". 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: Acanthosis nigricans is characterized by symmetric, hyperpigmented, and velvety plaques with ill-defined borders, typically involving intertriginous areas. Obesity is the most common cause of acanthosis nigricans which is increasingly observed in obese children and adolescents and can serve as a cutaneous marker of insulin resistance. Early recognition of acanthosis nigricans is important because acanthosis nigricans can also be a cutaneous manifestation of a variety of systemic disorders and, rarely, as a sign of internal malignancy. This may consist of weight reduction, discontinuation of causative drugs, treatment of underlying endocrinopathy, or treatment of an underlying malignancy. For patients with isolated acanthosis nigricans and for those whose underlying cause is not amenable to treatment, treatment of the lesion may be considered for cosmetic reasons. Topical retinoids, vitamin D analogs, chemical peels, and other keratolytics are often used for the treatment of localized lesions. Seldom, systemic therapy such as oral retinoids may be considered for extensive or generalized acanthosis nigricans and acanthosis nigricans unresponsive to topical therapy. Other uncommon treatment modalities include dermabrasion, laser therapy, and surgical removal.
CONCLUSION: Although acanthosis nigricans is treatable, a complete cure is difficult to achieve. The underlying cause should be treated, if possible, to resolve and prevent the recurrence of acanthosis nigricans. The diagnosis is mainly clinical, based on the characteristic appearance (symmetrically distributed, hyperpigmented, velvety, papillomatous, hyperkeratotic plaques with ill-defined borders) and the typical sites (intertriginous areas, flexural area, and skin folds) of the lesions. The diagnosis might be difficult for lesions that have atypical morphology or are in an unusual location. Clinicians should be familiar with the clinical signs, evaluation, diagnosis, and therapy of acanthosis nigricans because of the link between it and underlying diseases.
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.
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: 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 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.
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.