IN 1951 Binkley and Johnson1 reported the case of a thirty-one-year-old woman with basal-cell nevi, agenesis of the corpus callosum and dental cysts. The skin lesions, appearing in childhood, exten...

Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib, A syndrome

A Review of its Symptomatology, Natural History, Pathologic Features, Genetics And Relationship to the Natural Aging Process

Werner’s Syndrome: A Review of its Symptomatology, Natural History, Pathologic Features, Genetics and Relationship to the Natural Aging Process

Nevoid basal-cell carcinoma syndrome.

NEMO/IKKγ-Deficient Mice Model Incontinentia Pigmenti

• Incontinentia pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis that usually affects female infants. I reviewed 464 references from the world literature and found 653 apparently valid reports of patients with incontinentia pigmenti. Skin manifestations were found to be somewhat more common than previously reported, and systemic manifestations were found in 79.8% of the patients. (Arch Dermatol112:535-542, 1976)

Incontinentia pigmenti. A world statistical analysis.

ALTHOUGH nevi have been the subject of a great many papers, much interest, and many philosophical arguments since the earliest days of medicine, it appears that there are still "new" nevi which have escaped detection and description in the past. I have recently studied a singular case of the nevus-unius-lateralis type which proved to be a systematized basal-cell nevus. REPORT OF CASE O. J., a 69-year-old white man, was seen in the department of dermatology and syphilology of the University Hospitals on May 16, 1951, on referral from the department of surgery, where he was being treated for an adenocarcinoma of the colon. He was a small, emaciated man with a marked congenital dorsal scoliosis and peculiar smooth growths on the skin of the left side of the body, also present since birth. Examination of the skin showed bands of varying width on the left side of the chest

Linear unilateral basal-cell nevus with comedones; report of a case.

INCONTINENTIA pigmenti is a peculiar skin disease, probably developmental in origin, characterized by an extremely striking and bizarre arrangement of pigmented macules in polyangular flecks, whorls, spidery forms, lines, and patches. These chocolate-brown designs do not follow lines of cleavage or distributions of nerves, nor do they respect the limitations of the midline. Areas of alopecia in the scalp appear frequently, and many other ectodermal and mesodermal defects occur, although they are less well known. One feature of this odd cutaneous anomaly has only been touched on in the literature, and yet it is perhaps the most peculiar aspect of the disease: the pigmentary anomaly is frequently heralded by inflammatory lesions, particularly bullae, in lines and patches. The bullae disappear and recur for weeks or months and then finally give way, either to the pigmented macules directly or to an intermediate temporary stage of linear verrucous lesions which gradually fade

INCONTINENTIA PIGMENTI: A Report of Five Cases and Review of the Literature

BONE formation in the skin, apart from calcinosis cutis, occurs in several forms: (1) in solitary growths in scars of surgical or other trauma; (2) in ossifying plaques in sclerodermatous diseases; (3) in nevi; (4) in epidermoid carcinomas; (5) in multiple small nodules in children, apparently from embryonic cell rests; (6) in metastatic osteogenic sarcoma, and (7) in miliary nodules in skin damaged by previous pathologic processes. It is with the last type that this report is concerned. In the cases previously reported, such as those of Hopkins 1 and Leider, 2 acne was apparently the predisposing disease. In other similar cases, such as those of Virchow 3 and Arzt, 4 no such process was noted. Syphilis has been presumed to be a factor in one case 5 in which bone was formed in a chronic leg ulcer, but otherwise this disease has not been reported in predisposition to osteomatosis.

Multiple miliary osteomas of the skin.

ABNORMALITIES in the distensibility and elasticity of the skin have been described for centuries under a variety of names. Considerable confusion has arisen in the nomenclature concerning diseases reported under such terms as cutis laxa, dermatolysis, cutis hyperelastica, cutis pendula, loose skin, dermatochalasis, pachydermatocele, chalazoderma, chalodermie, cutaneous geromorphism, dermatomegaly, lax skin, Ehlers-Danlos syndrome, elephantiasis and many others. These terms are loosely applied and tend to overlap in meaning. In general, the aforenamed diseases can be separated fairly well into several groups: Dermatolysis is the term first used by Alibert 1 in 1835 to describe hypertrophic disorders of the skin associated with laxness but without hyperelasticity. There seem to be two types (which may be related): first, generalized diffuse hypertrophy of the skin which hangs in heavy folds and is coarse and usually rough and, second, localized neurofibromatosis (von Recklinghausen's disease). Either may be congenital and may be associated with cutis

Robert G. Carney

Papers

Incontinentia pigmenti. A world statistical analysis.

Linear unilateral basal-cell nevus with comedones; report of a case.

INCONTINENTIA PIGMENTI: A Report of Five Cases and Review of the Literature

Multiple miliary osteomas of the skin.

Acquired loose skin (chalazoderma) report of a case.