The vast majority of hemangiomas, the most common skin tumor of infancy, are small lesions, easily recognized by their clinical features, and left to involute spontaneously. Hemangiomas also grow in a number of visceral locations, although rarely. In addition, associated malformations are reported. We analyzed 175 cases of severe superficial hemangiomas that represented approximately 10% of all hemangiomas evaluated from 1980 to 1995. In this particular group of severe hemangiomas, with marked female preponderance (6.6:1), symptomatic visceral hemangiomas were present in 20 of 175 patients (11.4%) and associated malformations were present in 12 patients (6.9%), with both present in 4 patients. We describe these associations and discuss which hemangiomas required active treatment and which therapeutic modalities can be used. Progress has been made in the management of problem hemangiomas.

Congenital Abnormalities↗

PubMedDate not supplied

[Congenital homolateral epidermal hyperplasia and hypoplastic hemidysplasia (splitting of the Solomon's syndrome) (author's transl)].

The observation of a 16-year-old girl born with an ectromelia and an ipsilateral inflammatory verrucous epidermal nevus led us to a synthetic study of 17 similar cases already published since 1927: all these cases concern female patients and are characterized by a unilateral hypoplastic dysplasia, most often of limbs, and inflammatory epidermal hyperplastic lesions described as ichthyosiform, psoriasiform or verrucous, usually distributed on the same side on the skin overlying the dysplastic body areas. The skin lesions may be partly regressive after birth and their histological features are suggestive of inflammatory linear verrucous epidermal nevus (I. L. V. E. N.). These associations may be representative of a special form of Solomon's syndrome whose heterogeneity has be recently emphasized. We propose to subdivide it in 3 forms: the epidermal nevus syndrome (Solomon's syndrome)--the organoid nevus syndrome (Schimmelpenning's syndrome)--the I. L. V. E. N. syndrome, probably X-linked dominant inherited (lethal for hemizygous males), associated with ipsilateral hypoplastic body lesions and, however less frequently than in the epidermal nevus syndrome, with ocular and nervous abnormalities. The distribution of cutaneous lesions has some similarities with the pattern of skin symptoms of X-linked dominant traits such as chondrodysplasia punctata, focal dermal hypoplasia or incontinentia pigmenti. The most typical feature of this syndrome is the strong inflammatory aspect of the epidermal nevus erroneously described in previous cases as unilateral psoriasis or ichthyosiform erythroderma.

Adolescent↗