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LEOPARD syndrome (LS) is a rare inherited autosomal dominant disease with high penetrance and markedly variable expression characterized by a spectrum of somatic abnormalities. In 1971, Gorlin proposed the well-known acronym LEOPARD (lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth, deafness). The nature and clinical importance of cardiovascular phenotypes associated with LS remain uncertain, because few patients with the disease have undergone comprehensive cardiac evaluations. To date, 200 cases have been described and one review has been published. We emphasize that this case is exceptional insofar as life expectancy was longer than other LEOPARD syndrome cases described in previous reports; these have had an early mortality due to cardiopathies. The aim of our study is to report a rare case of a patient affected with LEOPARD syndrome, survived until 67 years with cutaneous associations never described in literature.

Distinguishing keratoacanthoma from well-differentiated squamous-cell carcinoma is often difficult on account of the clinical and histopathological similarities between them. Since the outcome of treatment depends on identifying the correct diagnosis and having the correct treatment on time, it is essential to differentiate keratoacanthoma and squamous-cell carcinoma as soon and accurately as possible. A paradigmatic case is herein reported. An 85 year-old female underwent total parotidectomy and ipsilateral neck dissection due to the squamous-cell carcinoma of the parotid gland. The investigations, in order to determine whether the tumour was a metastatic or a primary one, led to a misdiagnosis. A prior skin lesion, which was excised over her left cheek one year ago in another clinic, was diagnosed as keratoacanthoma. However, the histopathological revision of the specimen revealed that the lesion was in fact a squamous-cell carcinoma. Thus the parotid tumour was accepted as metastatic squamous-cell carcinoma rather than primary squamous-cell carcinoma.

The authors report a case of keratoacanthoma localized on the prolabium of the lower lip. They focus their attention on the difficulty of making a correct diagnosis and on the complexity of differential diagnosis with squamous cell carcinoma. After incisional biopsy of the lesion, treatment took the form of the complete excision of the neoplasm which is indispensable for a definitive diagnosis. The keratoacanthoma, although being an absolutely benign lesion, in fact requires complete exeresis in order to perform an histological examination of the entire neoplastic mass and thus definitively rule put the presence of squamous cell carcinoma. If correctly performed, the operation leaves very little scarring and satisfies the patient's esthetic and psychological expectations.

Four patients affected with keratoacanthoma have been treated with etretinate. In all the cases the tumors were either excessively large or located in peculiar sites. Etretinate was initially administered at the dosage of 1-1.3 mg/kg/die and then reduced at 0.3 mg/kg/die. All the tumors healed completely. During the treatment a slight cheilitis appeared in all the patients. One of them also experienced a transient hypertrigliceridemia and another an erythematous scaling dermatitis of the trunk and of palms.