Characterizing Granuloma Annulare in 73 Pediatric Patients.

Background: Granuloma annulare (GA) is a common, benign, idiopathic inflammatory dermatosis. Aside from case reports and small studies, there are limited data about the characteristics of GA in children. Objective: This study aimed to better characterize the epidemiologic and clinical features, triggering factors, disease associations, and outcomes of GA in the pediatric population. Methods: We conducted a retrospective study of 73 pediatric patients diagnosed with GA at the University of Rochester Medical Center over a 7-year period. Results: The most common subtype was localized GA (71.2%, n = 52), followed by subcutaneous (also known as "deep GA"; 16.4%, n = 12) and generalized (12.3%, n = 9) subtypes. Over 90% of patients had idiopathic GA, with the remaining patients reporting viral infection or trauma as triggers. Half of the patients studied had comorbid conditions, most frequently atopic dermatitis (17.8%, n = 13), obesity (9.59%, n = 7), asthma (6.85%, n = 5), and allergic rhinitis (6.85%, n = 5). The median duration of the disease was 11.00 months (interquartile range (IQR) 15.75 months); generalized GA had the shortest duration (median 10.00 months, IQR 15.50 months), while subcutaneous GA had the longest duration (median 12.00 months and IQR 29.00 months). Although recurrence rates for subcutaneous and generalized GA were high at 45.5% and 33.3%, respectively, most patients achieved clearance or improvement with treatment. Conclusion: Most cases of GA in our study were idiopathic, with no clear differences between GA subtypes and associated comorbidities. Topical steroids were the most prescribed treatment with mixed efficacy.

A case of severe nivolumab-induced lichen planus pemphigoides in a child with metastatic spitzoid melanoma.

Dermatologic reactions are among the most common adverse events of antiprogrammed cell death-1 (anti-PD-1) monoclonal antibodies agents and include maculopapular rash, psoriasiform rash, lichenoid eruptions, autoimmune bullous disorders, and vitiligo. Here, we present a case of a 12-year-old African American male with metastatic spitzoid melanoma treated with nivolumab who developed a mild lichenoid eruption that progressed to a severe case of lichen planus pemphigoides (LPP). Management was complex given the patient's age and history and included hospitalization for intravenous steroids, an intensive topical steroid regimen, methotrexate, and discontinuation of nivolumab. This case illustrates a rare but dramatic progression from a mild LP-like eruption to severe bullous lichenoid eruption, most consistent with LPP, as well as the diagnostic and treatment challenges in the setting of a pediatric patient on nivolumab.

Congenital cardiac, aortic arch, and vascular bed anomalies in PHACE syndrome (from the International PHACE Syndrome Registry).

PHACE syndrome represents the association of large infantile hemangiomas of the head and neck with brain, cerebrovascular, cardiac, ocular, and ventral or midline defects. Cardiac and cerebrovascular anomalies are the most common extracutaneous features of PHACE, and they also constitute the greatest source of potential morbidity. Congenital heart disease in PHACE is incompletely described, and this study was conducted to better characterize its features. This study of the International PHACE Syndrome Registry represents the largest central review of clinical, radiologic, and histopathologic data for cardiovascular anomalies in patients with PHACE to date. Sixty-two (41%) of 150 subjects had intracardiac, aortic arch, or brachiocephalic vessel anomalies. Aberrant origin of a subclavian artery was the most common cardiovascular anomaly (present in 31 (21%) of 150 subjects). Coarctation was the second most common anomaly, identified in 28 (19%) of 150 subjects, and can be missed clinically in patients with PHACE because of the frequent association of arch obstruction with aberrant subclavian origin. Twenty-three (37%) of 62 subjects with cardiovascular anomalies required procedural intervention. A greater percentage of hemangiomas were located on the left side of the head and neck in patients with coarctation (46% vs 39%); however, hemangioma distribution did not predict the presence of cardiovascular anomalies overall. In conclusion, PHACE is associated with a high risk of congenital heart disease. Cardiac and aortic arch imaging with detailed assessment of arch patency and brachiocephalic origins is essential for any patient suspected of having PHACE. Longitudinal investigation is needed to determine the long-term outcomes of cardiovascular anomalies in PHACE.

Infantile hemangiomas with unusually prolonged growth phase: a case series.

BACKGROUND: Most infantile hemangiomas (IHs) complete their proliferative growth phase before 9 months of age, but those with unusually prolonged growth create unique clinical challenges. We performed a retrospective case series of IHs with prolonged growth to further characterize these lesions and their treatment. OBSERVATIONS: We identified 23 patients as having IHs with prolonged growth after 9 months of age, with growth to a mean age of 17 months. All of the IHs had a deep dermal to subcutaneous component, all had either segmental or indeterminate morphologic characteristics, and 39% involved the parotid gland. A total of 20 of 23 received prolonged treatment with systemic corticosteroids (mean duration of treatment, 11 months), and 9 of 20 received additional systemic therapies (vincristine sulfate and/or interferon alfa-2a or alfa-2b). CONCLUSIONS: Prolonged growth was observed primarily in IHs with a deep component and segmental morphologic characteristics. Recognition of this subset of hemangiomas is important for clinicians, and further study of IHs may provide clues to their pathogenesis.

A comparison of disease severity among affected male versus female patients with PHACE syndrome.

BACKGROUND: PHACE syndrome (Online Mendelian Inheritance in Man database No. 606519) refers to the association of large, plaquelike, or segmental hemangiomas of the face, with one or more of the following anomalies: posterior fossa brain malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, eye anomalies, and ventral developmental defects, specifically sternal defects, supraumbilical raphe, or both. OBJECTIVE: The underlying pathogenesis of PHACE is unknown. A strong female predominance exists, leading some to suggest the possibility of X-linked dominant inheritance, with lethality in male patients. However, no familial cases have been reported, and disease severity among affected male patients has not been systematically studied. METHODS: We compared the incidence of syndrome-associated anomalies between 17 new and 42 published reports of male patients with PHACE versus 213 published reports of female patients with PHACE. RESULTS: A statistically significant difference was found only for structural brain anomalies, which were somewhat more common in male patients. LIMITATIONS: This was a retrospective study. Information was limited on some new and many previously reported cases. CONCLUSIONS: Overall, our results show no convincing trend toward greater or lesser disease severity among affected male patients with PHACE.

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity.

BACKGROUND: Severe thrombocytopenic coagulopathy may complicate platelet-trapping vascular tumors such as kaposiform hemangioendothelioma and tufted angioma. Low-grade, chronic consumptive coagulopathy may occur with extensive venous and lymphatic malformations. We have also observed patients with rare multifocal, congenital skin and gastrointestinal (GI) tract vascular anomalies of distinctive and remarkably similar appearance, all associated with coagulopathy. We studied the clinical and histopathologic features of 3 patients demonstrating this previously uninvestigated phenomenon. OBSERVATIONS: All 3 patients presented with hundreds of congenital red-brown skin plaques as large as a few centimeters, with similar lesions throughout the GI tract and severe GI tract bleeding. One patient had synovial involvement. All had significant thrombocytopenia, with prothrombin and partial thromboplastin times and fibrinogen levels near the reference range. Corticosteroids and/or interferon alfa treatment resulted in equivocal or no improvement. Skin lesions from all 3 patients were histologically distinctive and similar, including dilated, thin-walled vessels in the dermis and subcutis lined by hobnailed, proliferative endothelial cells (10%-15% immunoreactive for Ki-67), most displaying intraluminal papillary projections. Immunoreaction for the lymphatic marker LYVE-1 was uniformly present. CONCLUSIONS: We propose the term multifocal lymphangioendotheliomatosis with thrombocytopenia to distinguish this newly recognized clinicopathological entity. These congenital lesions, like tufted angioma and kaposiform hemangioendothelioma, show lymphatic differentiation, strengthening the association between abnormal lymphatic endothelium and coagulopathy.

Enfermedad estreptocócica perianal y psoriasis gutata: a propósito de dos casos infantiles / Perianal streptococcal disease and psoriasis guttata: a propos of 2 infantile cases

Se presentan dos casos infantiles de psoriasis gutata y eritema perianal persistente donde se aisló el estreptococo beta hemolítico del grupo A. Se quiere recalcar la importancia de buscar el reservorio perianal del estreptococo en el niño con psoriasis en gotas, además de los clásicos focos faríngeos y nasales. El tratamiento empleado fue penicilina V oral (100.000 U/kg/d) durante 3 semanas más rifampicina (20 mg/kg/d) durante 10 días por la persistencia clínica del eritema perianal y la positividad del cultivo. La duración del tratamiento está determinada por la remisión del cuadro clínico y la negativización de los cultivos perianales