Sclerodermatous changes of face, neck and scalp associated with familial porphyria cutanea tarda.

Porphyria cutanea tarda (PCT), the most common of the porphyrias, is a mainly acquired disease of the liver, which manifests with bullous skin lesions. However, up to 20% of patients with PCT, usually those with chronic untreated disease, are reported to develop some sclerodermatous changes that may affect both light-exposed and nonexposed areas and that can be histologically indistinguishable from true scleroderma. A small number of patients with PCT has severe or generalized scleroderma, which is not necessarily due to coexistent systemic sclerosis. There are few reports in the literature that detail whether the severe sclerodermatous changes respond to control of the porphyria. We report a case of familial PCT with associated severe sclerodermatous changes causing scarring alopecia, cicatricial ectropion and skin thickening over the upper trunk. The scleroderma improved slightly over a 4-year follow-up period after treatment to normalize porphyrin excretion and prevent relapse.

Zinc deficiency: a problem with preterm breast milk.

We report a breast-fed preterm infant (31 weeks gestation) who developed nutritional zinc deficiency 13 weeks post delivery. Serial samples of his mothers breast milk from early lactation (2 weeks post delivery) contained very low zinc concentrations, although her serum zinc and sweat zinc concentrations were normal. Following the birth of her second baby at 38 weeks gestation, her breast milk contained normal amounts of zinc. We conclude that the low breast milk zinc values obtained following the first pregnancy may have been due to immaturity of the milk. Units that feed very low birth-weight babies preterm milk should check its zinc concentration or at least have a high index of suspicion for this nutritional deficiency.

Atypical multicentric reticulohistiocytosis with paraproteinemia.

Two women had multiple subcutaneous nodules that showed features of multicentric reticulohistiocytosis (MR). Neither had joint symptoms. Both had a raised erythrocyte sedimentation rate, an immunoglobulin G paraproteinemia, and raised levels of nonhepatic serum alkaline phosphatase. The skin lesions have been followed up, using light and electron microscopy, immunoperoxidase, and histochemical methods. The material in the giant cells stained positively for gamma heavy chain determinants: the light chain type in each case was that of the paraprotein. An attempt to reproduce the skin lesions in one patient by intradermal injection of her paraprotein failed.

Intra-oral temperatures in man with special reference to involvement of the central incisors and premaxillary alveolar process in lepromatous leprosy.

The predilection of lepromatous leprosy for body sites substantially below core temperature is well known, and within the oral cavity the premaxillary alveolar process and upper central incisor teeth are especially affected. In this study, intra-oral temperatures were recorded by means of a series of thermistor probes applied to the teeth and labial surfaces of the gums of normal subjects. The temperature distribution established showed a close correlation with the known pattern of involvement of teeth and supporting structures derived from clinical, pathological and archeological data. Relatively low temperature of the premaxillary alveolar process was demonstrated, a factor which may be of prime importance for the localization of disease in this region, as it is for other tissues in the lepromatous patient.

Reddening of the upper central incisors associated with periapical granuloma in lepromatous leprosy.

Four years after starting treatment for lepromatous leprosy in England a male Pakistani aged 26 was found to have red discoloration of the upper central incisor teeth. A radiograph suggested periapical abscess on the right with haziness in a corresponding area on the left. Right apicectomy was performed with removal of a solid mass attached to the apex, sections revealing a lepromatous infiltrate with acid-fast fragments of Mycobacterium leprae in the cytoplasm of foamy macrophages. Clinical and archaeological evidence for the frequent involvement of these teeth in lepromatous leprosy is reviewed. The upper incisor area is relatively cool, a factor which may be of critical importance for the lodgement and multiplication of this bacillus, as it is in other body sites in lepromatous leprosy.