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Two patients with Cowden disease, a syndrome consisting of cutaneous papules and nodules on the face, oral mucous membranes and dorsal aspects of the forearms and hands in association with tumours of the thyroid gland, breasts, gastro-intestinal tract, and female reproductive tract, are reported. This disease is important to the physician because recognition of these cutaneous lesions may allow early diagnosis of internal neoplasia.
During the past 2 years we have studied eighty-nine biopsy specimens from nineteen patients with Cowden's syndrome. Among fifty-three facial lesions, twenty-nine were trichilemmomas. Twenty-three of the others were consistent with trichilemmoma, showed a non-specific benign verrucous acanthoma, or were not diagnostic; one was a blue naevus. All fourteen oral mucosal biopsy specimens were benign fibromas. Nineteen of twenty-two biopsy specimens from the hands and feet showed the pattern of benign keratosis. Multiple trichilemmomas were found in all patients with Cowden's syndrome, but at times several biopsy specimens were required before a diagnostic picture was uncovered. All patients with multiple facial trichilemmomas were found to have Cowden's syndrome. The combination of multiple facial trichilemmomas, oral fibromas, and benign acral keratoses enables one to diagnose Cowden's syndrome at a stage before serious internal complications develop.
BACKGROUND: Twenty patients treated for lentigo maligna of the face with cryosurgery developed benign lentiginous hyperpigmentation mimicking a recurrence. OBJECTIVE: When cryosurgery is used in the treatment of lentigo maligna, it is important to know whether repigmentation of the scar represents true recurrence or a benign process. METHODS: Twenty patients were treated with cryosurgery for lentigo maligna of the face. Within a follow-up period of 7 to 80 months, frequent clinical observations were made. RESULTS: Lentiginous hyperpigmentation developed within the treatment area in eight patients. Histologic investigation revealed recurrence of lentigo maligna in three and benign hyperpigmentation in five. CONCLUSION: Genetic factors and UV exposure after cryosurgery may favor the development of benign lentiginous hyperpigmentation. Because recurrence of lentigo maligna must be considered, histologic evaluation of repigmentation is mandatory.
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Different flap techniques for plastic repair of defects are described, namely, local flaps (advancement, transposition and rotation flaps) and regional flaps. Such use of skin of similar structure and pigmentation gives excellent cosmetic results. The indications for using local or regional flaps depends on size and location of the defect. Total extirpation of cutaneous malignancy particularly by microscopically monitored serial excision is a prerequisite to reconstruction.
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We can see that there are a number of conditions that commonly occur on the skin of the face, and that these may be of little or great consequence to our patients. Dentists enjoy an ideal situation for the detection and management or referral of the more worrisome lesions. By paying close attention to the faces of dental patients, asking appropriate questions about the lesions discovered, and following up on the results of advise given to patients, dentists can play an important role as health care advisors concerning dermatologic conditions of the face.
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A case is presented of an otherwise healthy woman with a relatively normal childhood and adult life and no evidence of any congenital anomaly, who at the age of twenty-seven, suddenly, almost explosively, had development of verruca-like lesions of the face. It is well to remember that the clinically obvious is not always the obvious, things are not always what they seem, and verrucae are not always viral warts.
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Enlargement of the cheeks may be due to a multitude of disorders, congenital, neoplastic, and in particular inflammatory. Congenital facial anomalies include cutaneous (and osseous) hemihypertrophy of the face and unilateral angiomatous malformations (e.g. Sturge-Weber-Krabbe Syndrome). Buccal enlargement due to dermal tumours include localized haemangiomas and lymphangiomas, lipomas and other benign connective tissue neoplasms, generalized disorders of the lymphatic or reticuloendothelial system including mycosis fungoides, reticulum cell sarcoma and other soft tissue malignancies, and cutaneous manifestations of malignant haemoblastoses, in particular chronic lymphatic leukaemia. Within the very large group of inflammatory skin swellings of the face a review is made of some bacterial pyodermias, severe forms of acne vulgaris, herpes zoster, lupus vulgaris, erysipelas, rosacea, steroid dermatitis, lupus erythematosus (discoid and systemic), toxic dermatitis, allergic eczema, urticaria, Quincke's oedema, and the Melkersson-Rosenthal syndrome. The importance of prevention and early detection of steroid-induced dermatitis is emphasized. This disorder, which is a pseudo-inflammatory disfiguring complication of prolonged topical steroid abuse, ranks in frequency with the skin problems most often seen in dermatological practice.
The early history of tuberous sclerosis can be traced from European wax models of the skin lesions in the 1830s to its recognition as a systemic disease by the early 1900s. There are various origins for the terms used for tuberous sclerosis, including Pringle's disease, Bourneville's disease, adenoma sebaceum, and epiloia. Since these terms are confusing and inaccurate, we urge that they no longer be used.
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Hydrocortisone 17-butyrate is a new non-fluorinated topical corticosteroid for use in psoriasis, eczema and other inflammatory dermatoses. In double-blind paired comparisons with other topical corticosteroids, the efficacy of hydrocortisone 17-butyrate 0.1% has generally been indistinguishable from that of triamcinolone acetonide 0.1%, fluocinolone acetonide 0.025% or betamethasone 17-valerate 0.1% in patients with eczema or psoriasis. When applied to the face of patients with atrophy superimposed on rosacea and perioral dermatitis resulting from prolonged use of fluorinated topical corticosteroids, hydrocortisone 17-butyrate 0.1% did not prevent the beneficial effect of systemic tetracycline nor the disappearance of telangiectasis, and tended to be more effective than hydrocortisone 1%. This result suggests that hydrocortisone 17-butyrate may be suitable for long-term use on facial lesions, although the occurrence of moderate rebound eruption in about 10% of patients indicates the need for caution. The findings suggest that hydrocortisone 17-butyrate may be less liable to cause skin atrophy and adrenal suppression than some other potent topical corticosteroids, but trials to date have been too short to allow definite conclusions regarding possible long-term effects and have not involved infants or children.
Elastosis perforans serpiginosa (EPS) is a rare entity belonging to the group of primary perforating dermatoses. A 13-year-old male patient with Down's syndrome developed reddish hyperkeratotic papules in a serpiginous and ellipsoid configuration on the face. Histological examination revealed transepidermal elimination of thick coarse elastic fibres from the papillary dermis. The dermal infiltrate showed an immunohistological pattern consistent with an acute cell-mediated immune response. It consisted mainly of activated T-lymphocytes, with a predominance of CD4-positive cells. Considerable numbers of CD1-positive cells were also present. Inflammatory macrophages of the 27E10 phenotype were found in considerable numbers, whereas 25F9-positive resident macrophages were almost completely absent. The role of a cell-mediated immune response in the mechanism of transepithelial elimination is discussed.