Localization and age distribution of telangiectases in children and adolescents with hereditary hemorrhagic telangiectasia: A retrospective cohort study.

Background: The location of telangiectases in hereditary hemorrhagic telangiectasia (HHT), as set forth in the consensus diagnostic (Curaçao) criteria, is based primarily on adults.

Objective: Document the locations and numbers of telangiectases in a cohort of pediatric patients with HHT.

Methods: A retrospective chart review using a standardized data collection form for site and number of telangiectases was performed for pediatric patients with HHT (age, 0-18 years) from 2005 to 2016.

Reversible profound sensorineural hearing loss due to propranolol sensitive hemangioma in an infant with PHACE syndrome.

PHACE syndrome is the association of large or segmental infantile hemangiomas of the face or scalp with abnormalities within the posterior fossa, arteries, cardiovascular system, and eyes. We present a case of reversible profound sensorineural hearing loss due to a cerebellopontine angle infantile hemangioma that was successfully treated with propranolol.

Ventral midline blanching in the setting of segmental infantile hemangiomas: clinical observations and pathogenetic implications.

Areas of blanched skin in children may be seen as an independent finding or in association with vascular birthmarks. We performed a retrospective chart review to identify and describe infants with areas of ventral midline blanching in the presence of segmental infantile hemangiomas. We identified nine full-term infants with partial or full segmental hemangiomas and areas of midline ventral blanching.

Intractable localized pruritus as the sole manifestation of intramedullary tumor in a child: case report and review of the literature.

Importance: Persistent localized pruritus is a rare manifestation of central nervous system tumors. Delayed diagnosis can lead to devastating complications.

Observations: We report an otherwise healthy 19-month-old girl who presented with signs of localized intractable pruritus of 6 months' duration on the left side of the neck, shoulder, and arm, resistant to systemic antihistamines and topical corticosteroids.

Rat bite fever: fever, arthritis, and rash in a 4-year-old boy.

Rat bite fever is a rare but potentially fatal Gram-negative infection that predominantly affects populations with exposure to rats, notably children. The clinical presentation is nonspecific and requires a high threshold of suspicion to elicit a history of rat exposure. We report here a case of a child whose diagnosis was made retrospectively but with good outcome.

Basal cell carcinoma arising in a nevus sebaceus in a child with facial trichoepitheliomas.

Nevus sebaceus (NS) is a congenital skin hamartoma that presents in childhood. Tumors may arise within these lesions over time. Mutations in the PTCH gene have been associated with both NS and some of the developing tumors.

Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid.

Neonatal pemphigus is a rarely reported transitory autoimmune blistering disease caused by transfer of maternal IgG autoantibodies to desmoglein 3 to the neonate through the placenta when the mother is affected with pemphigus. It is clinically characterized by transient flaccid blisters and erosions on the skin and, rarely, the mucous membranes. Neonatal pemphigus vulgaris has never been reported to persist beyond the neonatal period and progress to adult disease.

Lipodystrophia centrifugalis abdominalis infantilis in a Caucasian girl: report of a case with perieccrine inflammation.

Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare, self-limiting disease typically affecting Asian children around the age of three. In classic cases, patients present with a hypopigmented patch with central atrophy and an erythematous border in the groin area. We present a case of LCAI affecting a female Caucasian toddler that shows striking perieccrine inflammation, a finding not previously reported in this entity.

Sweet syndrome in two children.

We report a 9-month-old girl and a 4-year-old boy with acute febrile neutrophilic dermatosis (Sweet syndrome). Both children were febrile, had leukocytosis, and exhibited lesions characteristic of Sweet syndrome. Both had an antecedent infection.

Refractory Demodex folliculitis in five children with acute lymphoblastic leukemia.

We report five children with acute lymphocytic leukemia on maintenance chemotherapy who had Demodex folliculitis. None experienced complete clearing when treated with permethrin 5% cream. Topical metronidazole helped to lessen the eruption in four, but did not provide full clearing.

Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.

We report life-threatening febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta in an 8-year-old girl. Hemorrhagic-crusted papules and plaques covered over 90% of the patient's body, leaving her susceptible to Pseudomonas aeruginosa and Staphylococcus epidermidis bacteremia as well as Candida parapsilosis fungemia. Sepsis delayed definitive treatment of the underlying cutaneous disease for 2 weeks.

Vascular stains and hair collar sign associated with congenital anomalies of the scalp.

We reported a series of three meningothelial hamartomas, one benign fibrous tumor, and one aplasia cutis congenita presenting with the hair collar sign and a coexistent vascular stain. Our series highlighted the importance of coexisting cutaneous markers found in the newborn period. The presence of a vascular stain and hair collar sign with or without a congenital scalp nodule should increase suspicion of an associated cranial dysraphism.

Proliferative nodules in congenital melanocytic nevi: a clinicopathologic and immunohistochemical analysis.

Congenital melanocytic nevi (CMN) occur in 1% to 2% of newborns, and the risk of malignant melanoma is increased in patients with large CMN. Appearance at birth or later of a nodular or hyperpigmented area within a CMN simulates malignant melanoma and prompts biopsy. Although their clinical and pathologic features seem ominous, proliferative nodules (PNs) typically are benign and may regress, although atypical features cause greater concern.

Aspergillosis in a 24-week newborn: a case report.

Aspergillosis is an uncommon neonatal infection, diagnosed with an increasing frequency over the last two decades. We report a premature neonate who developed aspergillosis while receiving amphotericin B and fluconazole for candidiasis. Despite early recognition and diagnosis, the infant died.

Tufted angiomas: variability of the clinical morphology.

In 1989 Wilson Jones and Orkin first described tufted angioma, which has the unifying histologic feature of circumscribed angiomatous tufts and lobules within the dermis. Tufted angioma may take unusual forms clinically. We describe five children less than 3 years of age with tufted angiomas, demonstrating the variability of the morphology of this vascular tumor.

Annular elastolytic giant cell granuloma.

Annular elastolytic giant cell granuloma is a granulomatous process that presents as slowly growing annular papules and plaques on sun-exposed skin. It was first described in patients in the fourth and fifth decades of life. These lesions are primarily distributed on the head and neck.