Margarita Larralde

Propranolol for Infantile Hemangiomas

Abstract:  Propranolol has been used successfully in a limited number of children with infantile hemangiomas. This multicenter retrospective study describes the efficacy and adverse effects of propranolol in infantile hemangioma. Seventy‐one infants with infantile hemangiomas were treated with oral propranolol, 1 mg/kg/12 hours, for at least 12 weeks. A photograph based severity scoring assessment was performed by five observers to evaluate efficacy, utilizing a scoring system of 10 as the original infantile hemangioma before treatment and 0 as completely normal skin. The mean of the five independent measurements was used in the analysis. Propranolol was a rapid and effective treatment for infantile hemangiomas at 4 weeks (p < 0.001), at 8 weeks (p < 0.001 compared to the 4 wks value), at 12 weeks (p < 0.05 compared to the 8 wks value), and thereafter up to 32 weeks (p < 0.01 compared to the 16 wks value). The response of infantile hemangiomas to propranolol was similar regardless of sex, age at onset of treatment, type of involvement (segmental and nonsegmental), facial segments affected, special locations (eyelid, nasal tip, and parotid region), ulceration, and depth of infantile hemangiomas. Very few side effects were reported; mainly agitated sleep in 10 of 71 patients. In the series of patients in this study, oral propranolol 2 mg/kg/day was a well‐tolerated and effective treatment for infantile hemangiomas. Prospective studies are needed to establish the exact role of propranolol in the treatment of infantile hemangiomas.

Congenital self-healing histiocytosis (Hashimoto–Pritzker)

Background Congenital self‐healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months.

Eosinophilic pustular folliculitis in infancy: report of two new cases.

Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features. We describe two patients who had recurrent episodes of pruritic follicular papular and pustular lesions on the face, extremities, and trunk. The eruptions lasted for 1 month with intermittent remissions. Laboratory tests disclosed no infectious or parasitic etiology in patient 2. In patient 1 we isolated methicillin‐resistant Staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self‐limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). Histologically both of our patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Their lesions responded well to topical corticosteroids.

Lymphedema as a postulated cause of cutis verticis gyrata in Turner syndrome.

Abstract: Unusual skin lesions were present at birth in four infants with Turner syndrome. The skin changes in these patients appear to have resulted either from in utero entrapment or pinching of edematous skin or from redundant skin remaining after in utero resolution of lymphedema. Distention by lymphedema is thought to cause several of the phenotypic characteristics seen in patients with Turner syndrome, including nuchal webbing and nail changes. In three of these patients the clinical appearance of the skin changes was similar to cutis verticis gyrata, marked by fixed thickened plaques in folds.

Pseudo-Kaposi Sarcoma with Arteriovenous Malformation

Pseudo‐Kaposi sarcoma with vascular malformation (Stewart–Bluefarb syndrome) is an uncommon and important entity characterized by congenital arteriovenous malformation and skin lesions that may resemble Kaposi sarcoma. This is usually seen in the lower limb of young people. We report a case of this syndrome in a 17‐year‐old boy who had skin lesions on the right leg and foot.

Infantile Myofibromatosis: Report of Nine Patients

Abstract:  Infantile myofibromatosis is a rare fibrous tumor of infancy that can be solitary or multiple. Although most of the cases are limited to the skin, in some instances systemic involvement can be present. Solitary tumors limited to the skin usually present a good prognosis with spontaneous regression. We performed a retrospective observational review of the clinical and pathologic characteristics of nine patients diagnosed as having infantile myofibromatosis, followed during a 10‐year period in a Pediatric Dermatology Department.

Scurvy in a 10-month-old boy

We report a 10‐month‐old boy with inflammatory and necrotic gingival lesions, fever, irritability, and pseudoparalysis of the legs. Laboratory examinations revealed moderate anemia and skeletal X‐rays showed osteopenia, scorbutic rosary at the costochondral junctions, and “corner sign” on the proximal metaphyses of the femora. The boy had been fed only with diluted cows milk. He had never taken solid food, vitamin C, or iron complement. Seventy‐two hours after starting oral vitamin C supplementation, there was significant improvement in the patients gingival lesions and general health. The clinical presentation and laboratory and imaging findings, together with the dramatic response to ascorbic acid intake, allowed us to confirm the diagnosis of infantile scurvy.

Eccrine Angiomatous Hamartoma: Report of Five Congenital Cases

Abstract:  Eccrine angiomatous hamartoma is a rare entity characterized histologically by the combination of proliferative eccrine and vascular elements. It generally arises before puberty, as solitary or multiple lesions, with a heterogeneous clinical appearance, affecting predominantly the distal extremities, with or without associated pain or hyperhidrosis. It may require surgical treatment due to cosmetic concern, progressive enlargement or the presence of pain or excessive hyperhidrosis. We report five congenital cases of eccrine angiomatous hamartoma, emphasizing a clinically uncommon tumor‐like appearance, with numerous telangiectasias on their surfaces resembling vascular lesions, in two of them.

Palmoplantar Eccrine Hidradenitis: Seven New Cases

Abstract:  Palmoplantar eccrine hidradenitis is a self‐limited disease characterized by painful erythematous papules and nodules of abrupt onset on the soles, and less frequently on the palms, of young individuals in good health. We describe seven children, four girls and three boys, between 4 and 12 years of age, with characteristic cutaneous and histopathologic findings of palmoplantar eccrine hidradenitis. All patients had complete resolution of their lesions within 2–4 weeks without treatment, however, one child experienced recurrences. All skin biopsy specimens showed a deep dermal mixed infiltrate with abundant neutrophils surrounding eccrine sweat glands, the histologic hallmark of the disease. Palmoplantar eccrine hidradenitis is a distinct clinical entity in which physical activity, excessive sweating, and prolonged wetness are possible triggering factors. The regression of the lesions is usually rapid, with complete clearance after 1 month, although there may be recurrent episodes.

Capillary malformation-arteriovenous malformation: a clinical review of 45 patients.

Capillary malformation–arteriovenous malformation (CM‐AVM) is a recently described autosomal dominant disorder that results from mutations in RASA1. It has been initially described as multiple CMs affecting several members of the same family, associated with fast‐flow malformations in at least one family member.