Anthony J. Mancini

Infantile Hemangiomas: Current Knowledge, Future Directions. Proceedings of a Research Workshop on Infantile Hemangiomas

Ilona J. Frieden, M.D.,* Anita N. Haggstrom, M.D.,† Beth A. Drolet, M.D.,‡ Anthony J. Mancini, M.D.,§ Sheila Fallon Friedlander, M.D.,¶ Laurence Boon, M.D., Ph.D.,** Sarah L. Chamlin, M.D.,§ Eulalia Baselga, M.D.,†† Maria C. Garzon, M.D.,‡‡ Amy J. Nopper, M.D.,§§ Dawn H. Siegel, M.D.,* Erin W. Mathes, M.D.,* Deborah S. Goddard, M.D.,¶¶ Joyce Bischoff, Ph.D.,¶¶ Paula E. North, M.D., Ph.D.,*** and Nancy B. Esterly, M.D.†††

Nail matrix arrest following hand-foot-mouth disease: a report of five children.

Abstract: Hand‐foot‐mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by a vesicular palmoplantar eruption and erosive stomatitis. Nail matrix arrest has been associated with a variety of drug exposures and systemic illnesses, including infections, and may result in a variety of changes, including transverse ridging (Beaus lines) and nail shedding (onychomadesis). The association of HFMD with Beaus lines and onychomadesis has not been reported previously. Five children, ages 22 months–4 years, presented with Beaus lines and/or onychomadesis following physician‐diagnosed HFMD by 3–8 weeks. Three of the five patients experienced fever with HFMD, and none had a history of nail trauma, periungual dermatitis, periungual vesicular lesions, or a significant medication intake history. All patients experienced HFMD within 4 weeks of one another, and all resided in the suburbs of the Chicago metropolitan area. In all patients the nail changes were temporary with spontaneous normal regrowth. The mechanism of the nail matrix arrest is unclear, but the timing and geographic clustering of the patients suggests an epidemic caused by the same viral strain.

Propranolol Use in PHACE Syndrome with Cervical and Intracranial Arterial Anomalies: Collective Experience in 32 Infants

The objective of this retrospective study of patients evaluated between July 2008 and October 2011 in seven pediatric dermatology centers was to combine collective clinical experience using oral propranolol therapy in 32 infants with PHACE syndrome (Posterior fossa [brain malformations present at birth], Hemangioma [usually covering a large area of the skin of the head or neck >5 cm]; Arterial lesions [abnormalities of the blood vessels in the neck or head]; Cardiac abnormalities or aortic coarctation [abnormalities of the heart or blood vessels that are attached to the heart]; Eye abnormalities) with cervical or intracranial arterial anomalies. Patients were given an average daily dose of oral propranolol of 1.8 mg/kg divided two or three times per day for an average duration of 12.3 months. The main outcome measure was adverse neurologic events. Seven (22%) patients were categorized as being at higher risk for stroke, defined on magnetic resonance imaging as severe, long‐segment narrowing or nonvisualization of major cerebral or cervical vessels without anatomic evidence of collateral circulation, often in the presence of concomitant cardiovascular comorbidities. Only one patient developed a change in neurologic status during propranolol treatment: mild right hemiparesis that remained static and improved while propranolol was continued. An additional three patients had worsening hemangioma ulceration or tissue necrosis during therapy. This is the largest report thus far of patients with PHACE syndrome treated with propranolol. Although no catastrophic neurologic events occurred, serious complications, particularly severe ulcerations, were seen in a minority of patients, and given the sample size, we cannot exclude the possibility that propranolol could augment the risk of stroke in this population. We propose radiologic criteria that may prove useful in defining PHACE patients as being at high or standard risk for stroke. We continue to advise caution in using systemic beta‐blockers, particularly for children with vascular anomalies at higher risk for stroke. Use of the lowest possible dosage, slow dosage titration, three times per day dosing to minimize abrupt changes in blood pressure, and close follow‐up, including neurologic consultation as needed, are recommended.

Retrospective Analysis of 32 Pediatric Patients with Anticonvulsant Hypersensitivity Syndrome (ACHSS)

Abstract:u2002 Objectıve:u2002 To review 32 pediatric patients with anticonvulsant hypersensitivity syndrome.

Infantile Acropustulosis Revisited: History of Scabies and Response to Topical Corticosteroids

Abstract: Infantile acropustulosis (IA) is a condition of young children characterized by recurrent episodes of pruritic vesicles and pustules in an acral distribution. Several reports describe patients with scabies infestation prior to the diagnosis of IA, although the relationship between the two remains unclear. Furthermore, optimal therapy is controversial. We reviewed the history of scabies and response to therapy in 21 patients diagnosed with IA at two institutions between 1983 and 1997. A history of prior treatment for scabies was noted in 14 patients, although only two had mites, feces, or ova detected on microscopic examination for diagnostic verification. All patients were treated with topical corticosteroids (4 with class I, 12 with class II, 3 with class III, 1 with class IV, and 1 with class VI). All 18 patients who returned for follow‐up experienced significant improvement or cleared completely with treatment. There were no observed cutaneous or systemic side effects from corticosteroid therapy. We conclude that a history of preceding scabies is common in patients with IA, but often this diagnosis is made without microscopic confirmation. We also demonstrate that mid‐ to high‐potency topical corticosteroids are a safe and effective first‐line therapy for patients with IA.

Hepatic Infantile Hemangiomas Treated with Oral Propranolol—A Case Series

Abstract:u2002 Hepatic infantile hemangiomas may be associated with morbidity and mortality, and traditional therapies may be associated with significant side effects. Since propranolol has been recently used successfully to treat cutaneous infantile hemangiomas, we decided to use it in three patients who presented with hepatic and skin hemangiomatosis. Three patients with skin and hepatic infantile hemangiomas, two of whom had evidence of cardiovascular compromise and one of whom had extensive liver involvement and hypothyroidism, were treated with oral propranolol. Regression of both skin and hepatic hemangiomas was noted in all patients, as was resolution of the cardiac symptoms and decreased thyroid requirement in two patients each. Propranolol was well tolerated without any adverse effects. Propranolol should be considered as a potential first‐line therapy in patients with symptomatic hepatic hemangiomatosis.

A Child with Both Langerhans and Non-Langerhans Cell Histiocytosis

Abstract: The histiocytic syndromes consist of a group of disorders that share in common the proliferation of cells of the monocytic/macrophage lineage. It has been conventional to divide the histiocytoses into two separate groups: Langerhans cell histiocytosis (LCH) and non‐LCH. We present a 2‐year‐old Hispanic boy who was referred to the dermatology clinic for evaluation of an asymptomatic cutaneous eruption of the head and upper trunk. In addition, he had a 3‐week history of pain in his right leg and difficulty in walking. The patients physical examination was normal, excluding the skin findings. On plain radiography, multiple lytic lesions in the skull, lumbar spine, and right tibia were seen. Histopathologic examination of a skin biopsy specimen revealed a predominantly histiocytic infiltrate in the dermis which was negative for S‐100 and CD1a stains. A tibial biopsy specimen showed a monomorphous infiltrate of histiocytes that were S‐100 and CD1a positive. This patients concomitant findings of both LCH and non‐LCH histiocytoses further support a potential overlap within the histiocytic syndromes, as has been suggested by others.

Hemifacial infantile hemangioma with intracranial extension: a rare entity.

Abstract:u2002 Intracranial hemangiomas are uncommon, especially in the absence of diffuse hemangiomatosis or the syndrome consisting of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal clefting, and/or supra‐umbilical raphe (PHACES). We saw an 8‐month‐old ex‐premature girl with a large left‐sided ocular and facial hemangioma that had been growing since early infancy. Examination revealed a 7 × 13 cm violaceous tumor involving the left periocular region and face. Ophthalmologic examination revealed deprivation amblyopia, anisometropia with myopia and astigmatism. Magnetic resonance imaging demonstrated a vascular tumor mass involving the scalp, face, and calvarium with extension into the orbit, infratemporal fossa, nasopharynx, lateral medullary cistern, internal auditory canal, and fourth ventricle. Marked enhancement was seen with contrast, and no posterior fossa malformations were noted. She was treated with prednisolone, which was tapered over 12 months. Follow‐up magnetic resonance imaging examination at 25 months showed a marked decrease in the size of all lesions, with residual hemangioma in the periorbital soft tissues and small foci in the orbit and intracranial sites. The brain and ventricular system were normal. Intracranial hemangioma may occur in the setting of a large facial hemangioma (especially segmental) in the absence of the PHACES syndrome or diffuse hemangiomatosis. Radiological imaging should be considered to assess for intracranial hemangioma as well as posterior fossa or arterial anomalies.

Intussusception in an infant with acute hemorrhagic edema of infancy

Abstract:u2002 Acute hemorrhagic edema of infancy typically occurs in infants less than 2u2003years of age and follows a benign clinical course. Gastrointestinal complications are unusual in this entity, in contrast to children with Henoch–Schönlein purpura, who are predominantly older than 2u2003years and at risk for potential morbidity from gastrointestinal tract and kidney involvement. We describe an infant with acute hemorrhagic edema of infancy who developed intussusception.

PHACE without Face? Infantile Hemangiomas of the Upper Body Region with Minimal or Absent Facial Hemangiomas and Associated Structural Malformations

Abstract:u2003 Infantile hemangiomas can be associated with congenital anomalies such as PHACE syndrome with facial hemangiomas and genitourinary and spinal anomalies in the setting of lower body hemangiomas. We describe five infants in whom segmental hemangiomas involving the upper torso and extremities with absent or small facial hemangiomas were associated with structural anomalies similar to those reported with PHACE syndrome, including three with structural arterial anomalies of the subclavian arteries, three with aortic arch anomalies (right sided or narrowed arch), two with congenital heart disease (atrial septal defect and ventricular septal defect; tetralogy of Fallot), one with a retinal scar, and one with a sternal defect (scar). Two of five had small facial hemangiomas of the lower lip, but none had large segmental hemangiomas of the face. Three of five would have met diagnostic criteria for PHACE but lacked a facial hemangioma of 5u2003cm in diameter or greater. Patients with segmental arm and thorax hemangiomas may have associated structural abnormalities with overlapping features of PHACE, suggesting that a similar syndrome can occur in this clinical setting.