Role of propranolol in ulcerated haemangioma of head and neck: a prospective comparative study.

PURPOSE: Infantile haemangiomas comprise the majority of vascular anomalies and are considered the predominant vascular tumour type 1. We performed this prospective study to evaluate the therapeutic response and propranolol tolerance in infants with ulcerated infantile haemangioma of head and neck region. METHODS: Sixty-four patients with ulcerated infantile haemangiomas (IHs) of head and neck region, without any prior treatment and with age older than 1 month, were included in the study, after informed consent was obtained, and were randomly divided into groups A and B. Group A patients were given oral propranolol at a dose of 2 mg/kg per day in three divided doses as outpatients. Group B patients were given oral ibuprofen at a dose of 10 mg/kg 8-hourly and paracetamol at dose of 16.2 mg/kg 8-hourly. Documentation of gender, age, haemangioma location, duration of ulceration and pain was measured on the second and fifth day after commencement of treatment in both groups using the Children's Hospital of Eastern Ontario Pain Scale. RESULTS: There was no difference in pain score between the two groups (P value 0.074). Mean duration of healing of ulceration in group A was 17.93 ± 2.22 days and in group B was 27.71 ± 2.33 days (P value <0.001). In group A, out of 28 patients, 8 (28.5 %) were complete responders, 16 (57.1 %) were partial responders and 4 (14.2 %) were non-responders. CONCLUSION: Propranolol is a valuable therapeutic alternative for treatment of ulcerated haemangiomas and effectively reduces pain.

Congenital ulcer of the buttock.

We present the case of a congenital, deep ulcer of the buttock in an otherwise healthy newborn girl. Histopathology excluded any specific cause of ulceration. The ulcer healed spontaneously at 4 weeks of age, leaving a scar. Because the patient also had symmetrical pits on her knees and because the ulcer was located in an area typically affected by dimples, we interpret this unique manifestation as a congenital defect in the spectrum of congenital dimples.

Emergency surgical treatment of an ulcerative and hemorrhagic congenital/infantile fibrosarcoma of the lower leg: case report and literature review.

Fibrosarcomas are rare malignant soft-tissue tumors occurring mostly in infants younger than 1 year of age. Fibrosarcomas can ulcerate and cause various complications, which could threaten a fetus in utero or a child in the early neonatal period. We report a unique case of congenital infantile fibrosarcoma of the lower leg, its treatment and pathology. The large expansive and destructive lesion was not appreciated on routine prenatal ultrasound exams at 20 and 33 weeks gestation. The newborn required immediate emergency surgical intervention after delivery to prevent death by hemorrhagic shock. Initial debulking of the tumor was performed and hemostasis was attained on the day of birth. The child was resuscitated and definitive treatment of the leg was deferred until a pathologic diagnosis was obtained. Given the extent of the fibrosarcoma, the lower leg was not salvageable and the patient received a through-the-knee amputation in the neonatal period. The patient is free of disease at 2 years of age.

Congenital erosive and vesicular dermatosis with reticulated scarring in a newborn: an innovative treatment using a silicone dressing.

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is a rare entity presenting in the newborn with crusted erosions and vesicles that heal relatively rapidly, forming unique reticulated scars. We report the case of a premature baby 31 weeks old. Diagnosis was confirmed by skin biopsies, and the clinical improvement was excellent, with complete healing observed within 7 weeks. This report highlights clinical and histopathologic features, and a new successful treatment approach using a silicone dressing.

PHACES syndrome presenting as hemangiomas, sternal clefting and congenital ulcerations on the helices.

Sternal malformation/vascular dysplasia association is a very rare condition comprised of midline defects and hemangiomas of the face and anterior trunk, that can be found as part of the PHACES phenotypic spectrum (posterior fossa malformations, hemangiomas, arterial anomalies, coarctations of the aorta, cardiac defects and eye abnormalities, sternal clefting). Herein, we describe a 6-month-old boy with sternal cleft, extensive segmental hemangiomas, and a depigmented scar on the tip of the xyphoid process, corresponding to a sternal malformation/vascular dysplasia association. He also had bilateral cutaneous ulcerations on the helices. Our case report indicates that ulceration of a hemangioma can occur before significant proliferation and may even be present congenitally.

Response of ulcerated perineal hemangiomas of infancy to becaplermin gel, a recombinant human platelet-derived growth factor.

BACKGROUND: Hemangiomas of infancy are the most common tumors of childhood, and ulceration is the most common complication. Many treatments have been used for hemangioma ulceration, although none are uniformly effective. A recent report described the successful use of 0.01% becaplermin gel, a recombinant human platelet-derived growth factor, for an ulcerated hemangioma refractory to standard care. We sought to further assess the responsiveness of hemangioma ulceration to 0.01% becaplermin gel and to compare its cost to that of conventional modalities. OBSERVATIONS: We report a case series of 8 infants treated with becaplermin gel for ulcerated perineal hemangiomas of infancy. All infants were seen between January and June 2003 in the pediatric dermatology clinic at Texas Children's Hospital. Six female and 2 male infants were included. All of the hemangiomas were large (> or =6 cm(2)), and of superficial or mixed superficial and deep morphology. Rapid ulcer healing occurred in all patients within 3 to 21 days (average, 10.25 days). CONCLUSIONS: In this small series, 0.01% becaplermin gel was a safe and effective treatment for perineal hemangioma ulceration. The rapid healing achieved with 0.01% becaplermin gel allows a reduction in the risk of secondary infection, pain, and need for hospitalization, as well as in the costs that often accumulate from multiple follow-up visits and long-term therapy.

A new case of extensive congenital erosions and vesicles healing with reticulate scarring.

Congenital erosions and vesicles that heal with reticulate scarring comprise a new entity first described in 1985 in 3 girls aged 3, 5 and 6 years. Two years later, an 8-year-old boy in whom the skin lesions were accompanied by neurological disorders was described, as in one of the patients described in the 1985 study. In 1990, similar findings were reported in a neonate, and recently the 7th case has been reported in the American literature. We describe an 8-month-old infant born with cutaneous lesions that subsequently formed reticulate scars peculiar to this clinical entity, which is characterized by variable clinical and histological expression.

[Large nodular, multicenter, juvenile xanthogranuloma with ulceration]. / Grossknotiges, multizentrisches, juveniles Xanthogranulom mit Ulzeration.

We report on a patient with multicentric juvenile xanthogranuloma, whose unusual clinical and histological pattern initially obscured the diagnosis. Spontaneous remission of the tumour, which has now been maintained for over 2 years, the change from the primary monomorphological fibrocytic appearance to a polymorphological histiocytic picture with multiple fat-storing giant cells, and the lacking dermonstration of specific cell organelles finally allowed the nature of the disease to the definitely diagnosed.

Congenital varicella of the upper limb. A preventable disaster.

Two patients with a rare form of congenital varicella involving the upper limb are described. The spectrum of disease, pathophysiology, principles of management and prevention are discussed.

Adams-Oliver syndrome: aplasia cutis congenita, terminal transverse limb defects and cutis marmorata telangiectatica congenita.

A male infant with the features of Adams-Oliver syndrome is described. These features included severe aplasia cutis congenita (ACC) of the scalp involving both skin and cranium, a shortened right foot with talipes equinovarus, extensive cutis marmorata telangiectatica congenita and also haemangiomas and ulceration of the abdominal skin. Exposure of the dural membrane was associated with infection, hyponatraemia and finally localised necrosis with herniation of brain and fatal cerebral haemorrhage.

Cutis marmorata telangiectatica congenita with terminal transverse limb defects.

We describe a girl with clinical and histopathological manifestations characteristic of Cutis Marmorata Telangiectatica Congenita (CMTC) associated with Terminal Transverse Limb Defects (TTLD). In contrast to previous reports on this rare syndrome, no cutaneous atrophy or aplasia was evident in our patient.

Aplasia cutis congenita associated with valvular heart disease.

We present a patient with a congenital scalp lesion compatible with aplasia cutis congenita (ACC) and associated congenital heart disease (CHD). This interesting combined entity is briefly reviewed and associated defects that can occur are discussed.

[Aplasia cutis congenita. Commentary on 5 cases]. / Aplasia cutis congénita. Comentario sobre cinco casos.

Five cases of aplasia cutis congenita are presented, three of them with the classic localization of the scalp. The fourth case involves the lower extremities and the fifth, the trunk. The malformations associated to each case are described. A review of the existing literature and of possible etiologies is done.

Cutis marmorata telangiectatica congenita: a case report.

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disorder that was first described in 1922 by Van Lohuizen (1). The clinical presentation is one of persistent cutis marmorata, phlebectasia, telangiectasia, and areas of ulcerations. The disorder shows slow clinical improvement over time. Previously, CMTC has also been described as congenital generalized phlebectasia (2-7), congenital phlebectasia (8), nevus vascularis reticularis (9), congenital livedo reticularis (10), and livedo telangiectatica (11). The following is a case report.