Retrospective study of pilomatricoma: 261 tumors in 239 patients.

INTRODUCTION AND OBJECTIVES: Pilomatricoma is the second most common skin tumor in childhood and youth and it has a broad differential diagnosis. The main objective of the present study was to determine the incidence of pilomatricomas in our hospital and to analyze the following variables: patient age and sex, tumor site, symptoms, previous trauma, clinical diagnosis, associated diseases, number of cases of multiple tumors, ultrasound findings, anesthetic and surgical techniques, and postoperative complications. MATERIAL AND METHOD: This was a retrospective study of the pilomatricomas surgically excised between January 2004 and December 2012 and registered in the database of the pathology department of our hospital. RESULTS: We found 261 pilomatricomas in 239 patients (120 female and 119 male) between 1 and 83 years of age (mean age, 26.4 years). The most common presentation was as a firm subcutaneous nodule, which was asymptomatic in 82% of cases. Tumors most commonly affected the head and neck (49.81%). Preoperative diagnosis was correct in 54.4% of cases, concomitant diseases were present in 59 cases, and 7 patients reported a history of trauma in the area of the tumor. There were 17 cases of multiple tumors, 1 case in a patient with familial disease and 2 in patients with Steinert disease. Soft-tissue ultrasound was performed on 57 lesions; images were compatible with pilomatricoma in 48 cases. Tumor excision was performed under local anesthesia in 185 cases and under local and general anesthesia in 76. The postoperative complications were hypertrophic scarring and wound dehiscence (1 case each). CONCLUSIONS: This was a retrospective study with the largest series recorded in Spain and with one of the highest incidences. In addition, we report on variables not described in other studies.

[Juvenile xanthogranuloma of the central nervous system]. / Xantogranuloma juvenil del sistema nervioso central.

INTRODUCTION: Juvenile xanthogranuloma (JXG) is a benign histiocytosis affecting mostly skin, although it may also be extracutaneous. Lesions tend to regress spontaneously, but there are cases of bad prognosis. CASE REPORT: We report the case of a girl aged 3 months with JXG involving skin and, asymptomatically, central nervous system (CNS) and lungs. At age 4 months, the bigger CNS lesion, placed in the right cavernous sinus and whose growth might have had important neurologic repercussions, was excised. After surgery she presented seizures with initial response to antiepileptic drugs. At age 8 and 10 months surgery was again necessary for appearance of a tension pseudoyst in postsurgical bed; a cystoperitoneal shunt was instaured and seizures were controlled. When the patient was 12 months old, the diagnosis of hypothyroidism was established, with a favourable response to levothyroxine. At present, the patient is 2 years 8 months and has a slightly slow neurologic development, with occasional short seizures. Skin lesion is smaller, lung nodules have regressed and, regarding CNS lesions, the bigger has not recurred and the other one remains stable. CONCLUSIONS: Some authors propose doing JXG s extension study only when suggested by clinical findings, so long as an effective therapy and the advantage to presymptomatic treatment are not well established. We wonder whether early diagnostic and therapeutic approach to silent deep lesions might lead to a better outcome of patients with JXG, particularly those with CNS involvement.

[Familial acanthosis nigricans]. / Acantosis nigricans familiar.

After a brief introduction about the classification and clinicopathological findings of acanthosis nigricans, we present a clinical case of the familial type of acanthosis nigricans in a woman with lesions beginning in childhood and with three family members affected. We comment the characteristics that permit us to individualize this condition from the other forms of benign acanthosis nigricans.

[Progressive symmetric erythrokeratodermia. Report of a case with delayed onset treated by etretinate]. / Eritroqueratodermia simétrica y progresiva. Aportación de un caso de inicio tardío tratado con etretinato.

A case of delayed onset (17 years) progressive symmetric erythrokeratodermia is presented. The treatment with etretinate initially improved the clinical picture, but it had to be discontinued because of personality changes, particularly depressive crisis. In addition, clinical manifestations, histology, kinetics and ultrastructural changes in this condition are also commented on.

[Multifocal epithelial hyperplasia: a familial case]. / Hiperplasia epitelial multifocal: un caso familiar.

UNLABELLED: Multifocal epithelial hyperplasia (MEH), or Heck disease, is a disease of the oral mucosa rarely seen in Spain. It is caused by the human papillomavirus (HPV), and is mainly associated with types 13 and 32. It appears in childhood (3-18 years) and a higher prevalence is reported in women and certain ethnic groups (American Indians and Eskimos). It consists of a benign, asymptomatic epithelial proliferation in the form of multiple papules of 3-10mm with a normal oral mucosa colour. The case involves an Ecuadorian male of 14 years, with papulous lesions in the oral mucosa with onset at 4 years of age. His 32 year-old mother had identical lesions. CONCLUSION: We present two members of an ecuadorian family with MEH. Although it is a rare disease in Spain, we must take this disease into account, given the increase in the immigrant population in Spain.

[Leprosy: identification of an isolated case]. / Lepra: detección de un caso aislado.

Leprosy is an infrequent disease in Spain. 4,714 cases of leprosy was registered in the last national census (1979). The disease is still common in certain regions, such as Levant, Andalusia, Extremadura, Canary Island and Galicia. Lepromatous type is more frequent, a typical hallmark of endemies with low activity or characterized by regression. We report a patient affected of borderline lepromatous leprosy with the following characteristics: 1) the only known case in his locality, 2) lack of reaction after the treatment, and 3) due to its clinic, histological, and bacteriological characteristics was quite difficult to determine the leprosy's type. Even though the leprosy is an infrequent disease, it can be seen in everyday's practice, so that the internist and specialist. Have to know the disease in order to make an early diagnosis and to treat the patient effectively.

[Bowenoid papulosis. Review of 4 cases in a health center]. / Papulosis bowenoide. Revisión de cuatro casos en un centro de salud.

OBJECTIVE: To check the clinical, histological, evolutionary and therapeutic characteristics of Bowen's Disease. DESIGN: A descriptive study of a crossover type. SETTING: Primary Care and a Dermatology clinic at a Health Centre. PATIENTS: There were 4 adult males, aged between 25 and 31, with pigmented papulosis lesions on their external genitals. Two of them were HIV-positive. MEASUREMENTS AND MAIN RESULTS: Clinical records were used. The diagnosis was confirmed through histological analysis. In all cases the lesions were papulosis, multiple and affecting the external genitals. The patients were clinically controlled until the lesions disappeared. The patients' sexual partners were referred to Gynaecology clinics for examination, but no pathology was found in any of them. CONCLUSIONS: Basic knowledge of Bowen's Disease in Primary Care is important given that it is essential to reach a diagnosis as early as possible, in order to monitor the patient and his partner and so avoid any future complications arising from the pathology's presence, such as tumour processes affecting the uterine cervix. The performance of histological analyses when faced with any suspicious genital lesion must be encouraged, given that this technique is non-aggressive.

[Soft chancroid: 4 clinical cases]. / Chancro blando: cuatro casos clínicos.

OBJECTIVE: The presentation of four clinical cases of chancroid confirmed by means of a culture for Haemophiullus Ducrey and the checking of its clinical and epidemiological characteristics. SETTING: Dermatology Clinic at the "V Centenario" Health Centre, San Sebastián de los Reyes, Madrid. PATIENTS AND OTHER PARTICIPANTS: Four clinical cases collected between 1988 and 1992. They were characterised by the very painful ulcers on the penis which appeared from 10 to 15 days after possible infection. Two of them had been previously treated with penicillin but showed no improvement. MEASUREMENT AND MAIN RESULTS: Clinical records were consulted and the diagnosis was confirmed by means of microbiological culture. Analyses including VDRL and HIV serologies were performed. All the patients were male: the location was the balanopreputial furrow. In two cases the lesion and the secondary adenopathy were single. In the four the general analysis was normal and serology for syphilis negative. Two were HIV positive. All were cured with a single dose of Ceftriaxon. CONCLUSIONS: Even though the chancroid is not common in our field, it must be identifiable so that a proper differential diagnosis of all genital ulcers can be made, with confirmation by means of a culture in a specific medium. The present first line treatment should be in reach of the Public Health System's family doctors and be initiated at once, given that genital ulcers are a risk factor in HIV transmission. Additionally these patients' HIV antibodies should be studied.

[Suppurative hidradenitis in Down's syndrome. Apropos of three cases]. / Hidradenitis supurativa en el síndrome de Down. A propósito de tres casos.

We present three patients affected by the Down's Syndrome who suffered hidradenitis suppurativa in perianal region for a long period of time with poor response to topical and systemic treatments. We describe both processes and the immunological alterations described in both groups. We conclude that hidradenitis suppurative may be a cutaneous manifestation relate to Down's Syndrome, but further research is necessary to clarify this associations.

[Pink pityriasis in primary care. Review of 46 cases]. / Pitiriasis rosada en atención primaria. Revisión de 46 casos.

OBJECTIVE: We pretend to improve the knowledge and management of pityriasis rosea by family practitioners. DESIGN: A retrospective qualitative study by the review of the registry in a outpatient Dermatology clinic. SETTING: This work is made in the Dermatology clinic attending to San Sebastián de los Reyes population (Madrid) that's situated in the V Centenario Center. MEASUREMENTS AND MAIN RESULTS: 46 patients (28 females) were diagnosticated (mean age 25 +/- 13). Incidence was 85/100,000 hab; 12 patients were derivated urgently; 38 cases showed herald patch (82.6%). Lesions had characteristical distribution in 36 patients (78.3%); 43 cases had a clinic diagnosis (93.5%). Treatment wasn't necessary in 20 patients (43.5%). Most usual General Practitioner's presumption diagnosis was pityriasis rosea (21.7%); there was no diagnosis in 36.9%. CONCLUSIONS: We find inexperience in general practitioners about pityriasis rosea. It's necessary a correct knowledge and management of this pathology in Primary Care.

[Pigmented basocellular epithelioma. Presentation of 7 cases]. / Epitelioma basocelular pigmentado. Presentación de 7 casos.

OBJECTIVE: To review the clinical, histological, evolutive and therapeutical characteristics of basal cell carcinoma and the pigmented subtype. DESIGN: Descriptive transversal study. LOCATION: Primary Care; Clinic of Dermatology in a Primary Care of Madrid. PARTICIPANTS: Seven patients (4 males, 3 females) over 60 years old with pigmented lesions and skin little pigmented. MEASUREMENTS AND MAIN RESULTS: Medical records and the histological examination were reviewed in all the cases. The clinical evolution had been the same in all the patients starting with an asymptomatic papular lesion of dark pigmentation growing slowly. Finally the lesions were transformed to a plaque or a shining nodule. Prolonged exposure to sunlight was present in five cases. All the patients were referred to the Dermatology Clinic with the presumptive diagnosis of melanoma. The lesion histological examination was diagnostic. CONCLUSIONS: It is very important the knowledge of basal cell carcinoma in Primary Care due the elevated incidence mainly among the geriatric population and the immunosupressed subjects. We must think in this type of neoplasia, doing the differential diagnosis with the other pigmented lesions. It is necessary to perform the histological examination of the suspects lesions.