A case of dyschromatosis symmetrica hereditaria with an associated eyelid hemangioma

Dyschromatosis symmetrica hereditaria (DSH) are rare autosomal dominant pigmentary genodermatosis characterized by reticular hyper- and hypopigmented skin macules on the dorsal aspect of the extremities and freckle-like spots on the face, sparing the palms and soles. Cutaneous hemangiomas were not reported in the literature with DSH. We describe for the first time to the best of our knowledge a case of DSH with histopathologically confirmed eyelid hemangioma.

A 25-year-old female was diagnosed with DSH in her childhood by a dermatologist then later developed cutaneous lupus erythematosus (CLE). Four years later she presented to our clinic with right lower eyelid painless mass. The histopathological examination showed inflamed epidermis overlying a mixed capillary and cavernous hemangioma. The patient had complete healing of the skin post-operatively with excellent cosmetic result.

DSH is usually isolated, however, acral hypertrophy, psoriasis, dental anomalies, aortic valve sclerosis, dystonia and intracranial hemangiomas have been reported in association with the disease. The types of the hemangiomas reported were not specified with lack of tissue diagnosis. Our case is unique because of the late occurrence of this eyelid skin hemangioma, the concomitant CLE, the history of hyperthyroidism, and the positive family history of consanguinity.

The pathogenesis of DSH is not well understood, however the previously reported intracranial hemangiomas and the currently reported skin vascular lesion would raise the role of inheritance and variable expression of such an association especially with concomitant CLE. This may warrant further studies on the etiology of DSH.