[Antibiotic prophylaxis in dermatologic surgery]. / Profilaxis antibiótica en cirugía dermatológica.

This article discusses factors to take into consideration for the rational use of antibiotics to prevent postoperative infection in dermatologic surgery. The treatment of choice is described.

Congenital multiple clustered dermatofibroma.

Multiple clustered dermatofibroma (MCD) is a rare tumour which usually appears during the first and second decades of life. We report a man in whom the MCD was congenital, although during the first few years of his second decade it extended to involve a broad zone on the left hip, gluteal region and upper thigh.

X-linked ichthyosis: an update.

X-linked ichthyosis is a genetic disorder of keratinization characterized by a generalized desquamation of large, adherent, dark brown scales. Extracutaneous manifestations include corneal opacity and cryptorchidism. Since 1978 it has been known that a deficit in steroid sulphatase enzyme (STS) is responsible for the abnormal cutaneous scaling, although the exact physiological mechanism remains uncertain. The STS gene has been mapped to the distal part of the short arm of the X chromosome. Interestingly, this region escapes X chromosome inactivation and has the highest ratio of chromosomal deletions among all genetic disorders, complete deletions having been found in up to 90% of patients. Diagnosis of patients with X-linked ichthyosis and female carriers is based on biochemical and genetic analysis. The latter currently seems to be the most accurate method in the majority of cases.

Longitudinal, transverse, and diffuse nail hyperpigmentation induced by hydroxyurea.

Hydroxyurea is a cytostatic agent used in patients with myeloproliferative disorders that may produce nail hyperpigmentation, most frequently longitudinal melanonychia. We present a patient treated with hydroxyurea whose nails showed several patterns of nail hyperpigmentation, including longitudinal and transverse bands, and a diffuse darkening of the nail matrix.

Altered expression of immunoreactive involucrin in lamellar ichthyosis.

In some cases of lamellar ichthyosis, mutations in the epidermal transglutaminase gene and a reduction in the thickness of the cornified envelope have been documented. Involucrin is a major component of the cornified envelope and a substrate for epidermal transglutaminase. The aim of the present work was to analyse the expression of involucrin in lamellar ichthyosis. An ultrastructural study and/or immunohistochemical and biochemical techniques with anti-involucrin antibody were carried out on the epidermis of fifteen patients (12 families) suffering from lamellar ichthyosis. The effect of in vivo retinoid treatment on the involucrin epidermal expression was also investigated. Four cases with normal skin, seventeen cases of other ichthyoses and ten cases of psoriasis were used as controls. In all these cases of lamellar ichthyosis, a thin or absent cornified envelope, electron-dense granules inside corneocytes and a decrease of the epidermal involucrin expression were observed. In the patients receiving treatment with retinoids, western blot and ELISA revealed an increase in the involucrin expression. The decreased expression of involucrin in lamellar ichthyosis could contribute to the altered desquamation process accompanying the disease, since the clinical improvement associated with retinoid treatment is accompanied by an increase in the expression of involucrin.

Congenital ulcerated subcutaneous fat necrosis of the newborn.

A neonate, born from a mother with hypertension during the last trimester of gestation, presented with ulcerated lesions on the right lower limb that had been present since birth. The baby also had necrosis of the fifth toe of the right foot and generalized muscle hypoplasia of the entire limb. The histologic appearance of the cutaneous lesions was diagnostic of subcutaneous fat necrosis of the newborn. The congenital presence of subcutaneous fat necrosis is exceptional, and to date, ulcerated forms have not been reported in the literature.

Verrucous plaque on the back of a hand.

We report the case of a 44-year-old slaughter-house operator with a skin lesion that had been present for two years on the back of his left hand. The lesion had increased progressively in size despite numerous topical treatments. Physical examination revealed an infiltrated erythematous-violet plaque with a verrucous surface featuring numerous orifices draining purulent material. Histologic study of the lesion disclosed tuberculoid granulomatous infiltrates at the dermoepidermal limit. Presence of Mycobacterium tuberculosis, together with other epidemiologic, clinical, histologic, and immunologic data, permitted a diagnosis of tuberculosis verrucosa cutis to be made. The excellent response of the patient to treatment confirmed this hypothesis. However, polychemotherapy withdrawal was temporarily needed due to analytical abnormalities.

Diffuse whitening of the oral mucosa in a child.

We report a healthy 16-year-old Caucasian boy, who consulted us for white, asymptomatic lesions in the mouth. The lesions were stable and had been present for 6 years. On physical examination, there were diffuse white, soft, corrugated plaques involving the buccal and labial mucosa, oral commissures, and floor of the mouth. No other mucosae were affected and there were no skin or nail abnormalities. The histologic findings revealed epidermal hyperplasia with parakeratosis and intracellular edema in the squamous cell layer. No nuclear atypia was observed. A differential diagnosis of three entities is proposed: white sponge nevus, leukoedema, and focal epithelial hyperplasia.

Incontinentia pigmenti: XXY male with a family history.

We report on the case of a male who from the start of life displayed vesicular lesions; on the trunk these were clustered and on the limbs they adopted a linear configuration. After biopsy of one such lesion, the histopathological study was compatible with a diagnosis of incontinentia pigmenti (IP). In the following months, hyperkeratotic lesions appeared which later became pigmented. The mother and other female members of the family also showed different degrees of alteration related to the same disease. The karyotype study showed the existence of 47,XXY (Klinefelter syndrome). The exceptional nature of this case is that although it is the third case reported in the literature of a male affected by incontinentia pigmenti with a previous family history, it is the only one combining this characteristic with the presence of a 47,XXY karyotype.

Subcutaneous fat necrosis of the newborn and idiopathic hypercalcemia.

A newborn with important signs of fetal disturbance and respiratory distress and respiratory distress developed plaques on both buttocks 14 days after birth, with the clinical and histological characteristics of subcutaneous fat necrosis of the newborn. Fifteen days after the onset of the cutaneous signs, a discrete degree of hypercalcemia was detected. Its outcome was favorable due to the early establishment of a diet rich in medium-chain triglycerides and devoid of calcium and vitamin D. Sixteen cases of idiopathic hypercalcemia in newborn with subcutaneous fat necrosis have been reported of which 3 died. Serial serum calcium determinations should be made in such infants and they should be observed closely for signs and symptoms of hypercalcemia.

[Apocrine cystadenoma. About 3 observations with one balanic localization (author's transl)]. / Cystadénoma apocrine. A propos de 3 observations dont une de siège balanique.

Description of three apocrine cystadenomas: two of the face and scalp and one of the glans (urethral meatus and left anterior surface of the navicular fossa) with no bibliographical precedent. From a clinico-histological standpoint the importance of objective data which make possible the clinical diagnosis is stressed allowing light microscope differentiation from eccrine hydrocystoma. The histogenesis is exposed and it appears convenient to name (eccrine or apocrine) cystadenoma the authentic cystic adenoma (among the organoid hamartoma of the cutaneous appendages, while reserving the term (eccrine or apocrine) hydrocystoma for glandular cysts. Due to the esthetic and occasionally functional repercussion, surgical excision is indicated.

X-linked ichthyosis.

In the period 1962-1974, 36 patients with X-linked ichthyosis, belonging to 22 families, were selected for study. The frequency of this genodermatosis is higher in the province of Salamanca than in other countries. In most of the cases, the lesions were apparent at birth or shortly after. In 41% of the patients studied by slit-lamp microscopy, posterior embriotoxom was seen. Hyperkeratosis was seen in follicular and sweat duct orifices. Statistical analysis of the pedigrees showed a higher proportion of affected males among the offspring of carriers and also among the offspring of the ichthyotics' sisters. In addition, among the patients' descendants, the higher ratio of females is statistically significant, showing that there is likely to be selection in favour of the X chromosome.

[Ehlers-Danlos syndrome (1 case)]. / Sindrome de Ehlers-Danlos.

A male patient is presented is clinical and histopathological characteristics are typical of Ehlers-Danlos Syndrome (cutaneous and articular hyperelasticity and cutaneous fragility with the presence of atrophic scars). Looking into the family history we think that other relatives had the same disease and they had died during their childhood due to internal hemorrhages. The patient we present did not have significant hemorrhages in any moment. The elastic fibers in the dermal papilla are reduced giving rise to the appearance of being increased in the middle and deep dermis.