Biomedical subjects
Publications and source records attributed to D M Bronson.
Woolly-hair is a congenital defect that produces a localized patch of lightly colored, curly scalp hair. Scanning electron microscopy of the abnormal hairs demonstrates twisting of the hair shaft and abnormal cuticle formation.
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Three patients had epidermolysis bullosa acquisita in association with Crohn's disease. We believe that epidermolysis bullosa acquisita should be included among the extraintestinal manifestations of inflammatory bowel disease. This article describes the diagnostic criteria, treatment, and other systemic diseases associated with epidermolysis bullosa acquisita.
A case of congenital pilar and smooth muscle nevus (CPSMN) is reported. CPSMN is a distinct clinical and histologic entity characterized by prominent vellus hairs accompanying a proliferation of smooth muscle within the reticular dermis. Plaquelike elevation and mild hyperpigmentation are variable features of this unusual organoid nevus.
Trichophyton tonsurans has long been recognized as an important, but hitherto uncommon, cause of tinea capitis in the United States. Today it is known to be more common, but infection of the glabrous skin by this organism is still not well appreciated. In an epidemiologic study during the two decades from 1961 to 1980, 1,292 isolates of thirteen species of dermatophytes were obtained from patients at Cook County Hospital, Chicago. During the last 3 years of study, T. tonsurans accounted for 207 cases (96%) of tinea capitis and ninety-seven cases (75%) of tinea corporis. Ninety-five percent of cases of tinea capitis caused by T. tonsurans occurred in children (59% girls, 41% boys). By contrast, 62% of cases of tinea corporis caused by that organism occurred in adults. Substantially more women than men were affected in a ratio of nearly six to one. The majority of the women were between 15 and 29 years of age; about half of all had lesions on the arm. Over the past 20 years, T. tonsurans has emerged as a major cause of both tinea capitis and tinea corporis in Chicago. The disproportionate number of cases of tinea corporis in women of childbearing age may be due to their more frequent contact with infected children.
A case of acrodermatitis enteropathica belatedly recognized in a pregnant 23-year-old woman is reported. The condition was not specifically diagnosed during childhood. It cleared at puberty but recurred during two of three pregnancies in the form of pustular, vesiculobullous, and psoriasiform lesions. There were no associated signs or symptoms in other organs. Initial diagnoses upon the recurrence during the third pregnancy were herpes gestationis and impetigo herpetiformis. A markedly decreased serum zinc level (18 micrograms/dl) was found. Treatment with zinc sulfate was instituted, and within 3 days the cutaneous lesions began to clear. Two months after the birth of a healthy child, and without further therapy, all lesions had resolved and the serum zinc level was nearly normal. Acrodermatitis enteropathica should be considered in the differential diagnosis of unresponsive bullous dermatoses occurring during pregnancy.
Two children with pili torti (twisted hairs) are reported, one of whom had sensorineural hearing loss. This combination of pili torti and deafness was described as a new genetic entity by Björnstad in 1965. Although light microscopy demonstrated variable numbers of twists along the shafts of hairs obtained from both patients, scanning electron microscopy in both cases revealed flattening and diagonally arranged scalloped defects on only one side of the involved hair shafts rather than true twists. Early auditory testing should be performed in children with pili torti in order to secure early treatment of possible associated hearing loss.
The primary form of cutis verticis gyrata, a rare skin lesion, is described for the first time in a neonate in whom it was associated with other congenital anomalies. The combination of a histologically normal skin biopsy from the lesion and neurologic deficit supported the diagnosis; follow-up at 7 months of age showed persistent hypotonia and significant developmental delay. Differentiation of primary and secondary forms of cutis verticis gyrata and currently recognized etiologies are also briefly reviewed.
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