[Bloom syndrome. Clinical manifestations and cromosomal study in a Mexican child]. / Síndrome de Bloom. Manifestaciones clínicas y estudio cromosómico en una niña mexicana.

Bloom syndrome is an extremely rare inherited disorder. We present a case of Bloom syndrome with a chromosomal study in a Mexican five-year-old patient who presented growth retardation, narrow facies with poikiloderma, café-au-lait, macules and photosensitivity.

[DRESS syndrome: Report of nine cases]. / Reacción por drogas con eosinofilia y síntomas sistémicos (síndrome de DRESS): Estudio retrospectivo de nueve casos.

BACKGROUND: DRESS Syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is an uncommon disease caused by drugs. It is characterized by a polymorphic disseminated eruption with fever and multiple organ dysfunction. AIM: To report the etiology, characteristics, treatment, prognosis, and follow up of patients with DRESS Syndrome admitted to a clinical hospital. MATERIAL AND METHODS: Review of medical records of patients admitted for drug reactions, selecting those patients complying with clinical criteria for DRESS Syndrome. Drugs used during three months prior to the onset of symptoms were evaluated as possible causes of the disease. RESULTS: Nine patients aged 16 to 68 years (six males) complied with the clinical criteria for the disease. The causative medications were carbamazepine in three patients, phenytoin in three, antituberculous drugs in two and amoxicillin in one. All were treated with systemic steroids with a complete clinical resolution. CONCLUSIONS: DRESS syndrome is usually underdiagnosed and has a good response to systemic steroids.

Disseminated brown macules in an infant.

Urticaria pigmentosa is the most common form of mastocytosis. Mastocytosis usually presents at birth or early childhood, and may involve only the skin or, less commonly, other internal organs. Diagnosis is clinical, but a skin biopsy may be useful. Prognosis is usually good, and treatment focuses on the avoidance of certain triggers and administration of topical and systemic medications. Appropriate counselling of parents regarding the benign nature of this disease is important as most cases resolve by adolescence.

Multiple-Digit Pigmented Bowen's Disease Induced by Human Papillomavirus in an Immunocompetent Child.

Introduction: Bowen's disease is a squamous cell carcinoma in situ, the most common malignancy of the nail unit. Presenting more frequently in the fingernails, common risk factors include ionizing radiation, oral exposure to arsenic or pesticides, dyskeratosis congenita, and quite commonly diverse subtypes of HPV. We report the first case of multiple periungual pigmented Bowen's disease in a pediatric patient. Case Presentation: A healthy 13-year-old boy presented with a 9-month history of a pigmented erythematous patch on the proximal nail fold of his 3rd right finger without associated symptoms. A punch biopsy was taken, and the diagnosis of Bowen's disease was made. The patient received photodynamic therapy and three cycles of imiquimod without response, and two new lesions appeared on the first and second right fingers. Surgical removal was performed on all lesions. A polymerase chain reaction detected an HPV type 16. Discussion/Conclusion: Multiple periungual Bowen's disease is rare, with the most frequent risk factors being HPV infection and chronic immunosuppression. Less than 10% of the cases present as longitudinal melanonychia. To date, there are no previous reports of multiple pigmented periungual Bowen's disease. HPV-induced Bowen's disease is usually present in adults aged between 22 and 89 years as persistent verrucae. In this case, koilocytosis and the fact that all lesions appeared on the right hand are suggestive of HPV infection.

Localized Donor Area Acute Telogen Effluvium following Follicular Unit Extraction: Key Trichoscopic Findings.

Introduction: Telogen effluvium is a form of non-scarring alopecia characterized by an increased hair shedding rate induced by mechanical or inflammatory factors. Case Report: A 27-year-old healthy male patient presented with several itchy alopecic patches in the occipital region. The patient had undergone a follicular unit extraction 6 weeks before with complete recovery after 1 week. Upon trichoscopy, we found empty follicular openings, short regrowing hairs, and coudability hairs. A diagnosis of acute telogen effluvium was made, and the patient was started on betamethasone lotion for daily use as a means to treat pruritus. After 1 month, the patient presented an almost complete response. Conclusion: While acute telogen effluvium is commonly seen in the receptor area after a hair transplant, the incidence of the donor region as a presentation is unknown. Common trichoscopic findings in telogen effluvium include empty follicular openings, short regrowing hairs, and lack of other signs usually seen in other types of alopecia. This description was consistent with what we found in our patient. Trichoscopic findings can help in the diagnosis, and understanding its natural course, physicians can reassure the patient about the self-resolutive outcome of this condition.

Ecthyma gangrenosum: a report of eight cases.

Ecthyma gangrenosum is a rare skin infection classically associated with Pseudomonas aeruginosa. We performed a retrospective study of all cases diagnosed with ecthyma gangrenosum from 2004-2010 in a university hospital in Mexico (8 cases, 5 female patients and 3 male patients, ages between 4 months and 2 years). The most common risk factor for ecthyma gangrenosum is neutropenia in immunocompromised patients. In previously healthy patients, immunological evaluation is important to rule out underlying immunodeficiency. Ecthyma gangrenosum in healthy patients has a high mortality rate and early diagnosis and aggressive antibiotic treatment is imperative as it can improve patients' prognosis.

Crusted demodicosis in an immunocompetent pediatric patient.

Demodicosis refers to the infestation by Demodex spp., a saprophytic mite of the pilosebaceous unit. Demodex proliferation can result in a number of cutaneous disorders including pustular folliculitis, pityriasis folliculorum, papulopustular, and granulomatous rosacea, among others. We report the case of a 7-year-old female presenting with pruritic grayish crusted lesions over her nose and cheeks, along with facial erythema, papules, and pustules. The father referred chronic use of topical steroids. A potassium hydroxide mount of a pustule scraping revealed several D. folliculorum mites. Oral ivermectin (200 µg/kg, single dose) plus topical permethrin 5% lotion applied for 3 consecutive nights were administered. Oral ivermectin was repeated every week and oral erythromycin plus topical metronidazole cream was added. The facial lesions greatly improved within the following 3 months. While infestation of the pilosebaceous unit by Demodex folliculorum mites is common, only few individuals present symptoms. Demodicosis can present as pruritic papules, pustules, plaques, and granulomatous facial lesions. To our knowledge, this is the first reported case of facial crusted demodicosis in an immunocompetent child. The development of symptoms in this patient could be secondary to local immunosuppression caused by the chronic use of topical steroids.

Rothmund-thomson syndrome: a 13-year follow-up.

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder presenting with poikiloderma and other clinical features, affecting the bones and eyes and, in type II RTS, presenting an increased risk for malignancy. With about 300 cases reported so far, we present a 13-year follow-up including clinical images, X-rays and genetic analysis. A 13-month-old female started with a facial rash with blisters on her cheeks and limbs at the age of 3 months along with congenital hypoplastic thumbs, frontal bossing and fine hair, eyebrows and eyelashes. The patient was lost to follow-up and returned 12 years later with palmoplantar hyperkeratotic lesions, short stature, disseminated poikiloderma and sparse scalp hair, with absence of eyelashes and eyebrows. Radiographic analysis showed radial ray defect, absence of the thumb and three wrist carpal bones, and reduced bone density. Gene sequencing for the RECQL4 helicase gene revealed a mutation on each allele. RTS is a rare disease, and in this patient we observed the evolution of her skin lesions and other clinical features, which were important for the classification of type II RTS. The next years will provide even more information on this rare disease.

Ulcerated radiodermatitis induced after fluoroscopically guided stent implantation angioplasty.

Cases of radiation-induced skin injury after fluoroscopically guided procedures have been reported since 1996, though the majority of them have been published in Radiology and Cardiology literature, less frequently in Dermatology journals. Chronic radiation dermatitis induced by fluoroscopy can be difficult to diagnose; a high grade of suspicion is required. We report a case of an obese 46-year-old man with hypertension, dyslipidemia, and severe coronary artery disease. He developed a pruritic and painful atrophic ulcerated skin plaque over his left scapula, six months after fluoroscopically guided stent implantation angioplasty. The diagnosis of radiodermatitis was confirmed histologically. We report this case to emphasize the importance of recognizing fluoroscopy as a cause of radiation dermatitis. A good clinical follow-up at regular intervals is important after long and complicated procedures, since the most prevalent factor for injury is long exposure time.

Ecthyma gangrenosum: a report of eight cases

Abstract: Ecthyma gangrenosum is a rare skin infection classically associated with Pseudomonas aeruginosa. We performed a retrospective study of all cases diagnosed with ecthyma gangrenosum from 2004-2010 in a university hospital in Mexico (8 cases, 5 female patients and 3 male patients, ages between 4 months and 2 years). The most common risk factor for ecthyma gangrenosum is neutropenia in immunocompromised patients. In previously healthy patients, immunological evaluation is important to rule out underlying immunodeficiency. Ecthyma gangrenosum in healthy patients has a high mortality rate and early diagnosis and aggressive antibiotic treatment is imperative as it can improve patients' prognosis.

Reacción por drogas con eosinofilia y síntomas sistémicos (síndrome de DRESS): Estudio retrospectivo de nueve casos / Dress syndrome: Report of nine cases

Background: DRESS Syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is an uncommon disease caused by drugs. It is characterized by a polymorphic disseminated eruption with fever and multiple organ dysfunction. Aim: To report the etiology, characteristics, treatment, prognosis, and follow up of patients with DRESS Syndrome admitted to a clinical hospital. Material and Methods: Review of medical records of patients admitted for drug reactions, selecting those patients complying with clinical criteria for DRESS Syndrome. Drugs used during three months prior to the onset of symptoms were evaluated as possible causes of the disease. Results: Nine patients aged 16 to 68 years (six males) complied with the clinical criteria for the disease. The causative medications were carbamazepine in three patients, phenytoin in three, antituberculous drugs in two and amoxicillin in one. All were treated with systemic steroids with a complete clinical resolution. Conclusions: DRESS syndrome is usually underdiagnosed and has a good response to systemic steroids.