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At a glance

Genetic disorder characterized by facial follicular skin atrophy, milia, telangiectasias, absent eyelashes and eyebrows, and basal cell carcinomas later on in life.

Incidence

Less than 20 cases reported.

Genetic inheritance

Autosomal dominant.

Clinical aspects

The skin changes usually start between the ages of 7 and 10 years with follicular atrophy (so-called atrophoderma vermiculatum), particularly on the cheeks, and with discoloration of the lips and hands (cyanosis-like). Milia-like papules with telangiectasias develop in adulthood, and basal cell carcinomas are a frequent complication of this syndrome, but appear later in life than in Bazex Syndrome. Basal cell carcinomas with milia and coarse, Sparse Hair Syndrome or congenital hypotrichosis with Milia Syndrome. Microscopically, a widespread lack of elastin in many skin areas with clumping of elastic components in other areas can be identified. Dermal collagen fibrils may have signs of hyalinization and vacuolization. Skin can get the aspect of solar elastosis. The eyebrows and eyelashes may either show an irregular, patchy distribution with distorted and maldirected growth, or may be missing completely.

Anesthetic considerations

No anesthetic complications are expected to arise from this syndrome, and no literature is available.

Other condition to be considered

  • Bazex Syndrome: Characterized by lesions suggesting “multiple ice-pick marks” on the dorsum of the hands and elbows dating from early infancy. Basal cell carcinomas may develop on the face between ages 15 and 26 years. Hypotrichosis is identical to the skin lesions seen in Conradi Disease and in Basal Cell Nevus Syndrome. It is considered X-linked dominant and presents similar features to the Rombo Syndrome. Other features include the presence of a “pinched” nose with hypoplastic alae and prominent columella. This medical condition has been reported mainly in France and all patients were Caucasians.

References

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Michaelsson  G, Olsson  E, Westermark  P: The Rombo syndrome: A familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. Acta Derm Venerol 61:497, 1981.  [PubMed: 6177160]
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Parren  LJ, Frank  J: Hereditary tumour syndromes featuring basal cell carcinomas. Br J Dermatol 165:30, 2011.  [PubMed: 21428980]
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Van Steensel,  MA, Jaspers,  NG, Steijlen  PM: A case of Rombo syndrome. Br J Dermatol 144:1215, 2001.  [PubMed: 11422044]

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