Use of Intravenous Immunoglobulins and Systemic Corticosteroids in Patients with Toxic Epidermal Necrolysis: Experience of a Hospital in Mexico City. / Tratamiento con inmunoglobulina intravenosa y esteroides sistémicos en pacientes con necrólisis epidérmica tóxica: Experiencia en un hospital en Ciudad de México.

Toxic epidermal necrolysis is the most serious mucocutaneous adverse drug reaction. Multidisciplinary treatment and withdrawal of the causative drug are key to reducing mortality. Few studies have analyzed the use of systemic corticosteroids and intravenous immunoglobulins (IVIG) in patients with toxic epidermal necrolysis in Latin America. We describe our experience with 6 cases treated at a dermatology referral hospital in Mexico City. None of the patients died or developed complications in the short or medium term. The most widely used regimen was a combination of IVIG 1 g/kg for 3 to 5 days and methylprednisolone 1 g for 3 to 5 days. Mean hospital stay was 14.8 days. The combined use of systemic corticosteroids and IVIG seems to be a safe treatment option for patients with toxic epidermal necrolysis.

Interleukin-17A enhances the production of CD147/extracellular matrix metalloproteinase inducer by monocytes from patients with psoriasis.

OBJECTIVE: CD147 is the main inducer of extracellular matrix metalloproteinases, which are critically involved in different inflammatory diseases. Our objective was to assess whether in vitro stimulation with Th1 and Th17 cytokines modulate CD147 production in monocytes from psoriasis patients. PATIENTS AND METHODS: Serum CD147 levels were measured in 60 psoriasis patients and 60 healthy controls. Furthermore, CD14+ monocytes were cultured and stimulated with TNF, IFN-g or IL-17A, and CD147 production was measured. RESULTS: Serum CD147 levels were higher in psoriasis patients (median 1866, IQR 1517-2355 pg/mL vs. 1686, 1382-1947 pg/mL; p=0.023), allowing to distinguish between patients and controls (AUC-ROC 0.632 ± 0.0509). Baseline CD147 production was similar in monocytes from patients and controls (1298, 769-1645 pg/mL vs. 1290, 1048-1976 pg/ml, respectively). Stimulation with IL-17A (1638, 1426-2027 pg/mL; p<0.001), but no other cytokine, was associated with increased production of CD147 in monocytes from psoriatic patients. In contrast, none of the cytokines increased CD147 production in monocytes from healthy controls. CONCLUSIONS: CD147 production by activated monocytes is a cytokine-dependent process, specifically by cytokines of the Th17 phenotype instead of those belonging to the Th1 phenotype. CD147 is a novel inflammatory mediator that could be a therapeutic target in psoriasis.

An impressive case of chromoblastomycosis due to Fonsecaea pedrosoi in a patient with a long history of fungal infection.

Chromoblastomycosis is defined as a chronic cutaneous and subcutaneous fungal infection caused by melanized or brown-pigmented fungi. A 63-year-old man farmer showed on external and internal part of the right arm, a well-delimited verrucous and hyperkeratotic plaque, with atrophic and cicatricial areas. Direct examination of skin scrapings samples showed the presence of muriform cells, a classic feature of chromoblastomycosis. Fungal isolation was performed in Sabouraud dextrose agar, and dark olivaceous colonies were isolated. Skin biopsy samples were obtained for histopathological and molecular diagnosis. DNA extracted from both, paraffin-embedded skin biopsy samples and fungal colonies, was used for molecular identification by 18S-ITS1-5.8S-ITS2-28S rRNA amplification and sequencing. Fonsecaea pedrosoi was identified from paraffin-embedded skin samples and fungal colonies. A combined therapy with terbinafine and itraconazole, plus cryotherapy was applied with an important improvement. Herein, we report an impressive case of chromoblastomycosis due to Fonsecaea pedrosoi with a successful outcome.

Skin disorders in overweight and obese patients and their relationship with insulin.

INTRODUCTION: The prevalence of obesity has increased worldwide in recent years. Some authors have described skin conditions associated with obesity, but there is little evidence on the association between insulin levels and such disorders. OBJECTIVE: To describe the skin disorders present in overweight and obese patients and analyze their association with insulin levels. MATERIAL AND METHODS: The study included nondiabetic male and female patients over 6 years of age who were seen at our hospital between January and April 2011. All the patients were evaluated by a dermatologist, who performed a physical examination, including anthropometry, and reviewed their medical history and medication record; fasting blood glucose and insulin were also measured. The patients were grouped according to degree of overweight or obesity and the data were compared using analysis of variance or the χ(2) test depending on the type of variable. The independence of the associations was assessed using regression analysis. RESULTS: In total, 109 patients (95 adults and 13 children, 83.5% female) were studied. The mean (SD) age was 38 (14) years and the mean body mass index was 39.6±8 kg/m(2). The skin conditions observed were acanthosis nigricans (AN) (in 97% of patients), skin tags (77%), keratosis pilaris (42%), and plantar hyperkeratosis (38%). Statistically significant associations were found between degree of obesity and AN (P=.003), skin tags (P=.001), and plantar hyperkeratosis. Number of skin tags, AN neck severity score, and AN distribution were significantly and independently associated with insulin levels. CONCLUSIONS: AN and skin tags should be considered clinical markers of hyperinsulinemia in nondiabetic, obese patients.

Analysis of genetic polymorphism of the HLA-B and HLA-DR loci in patients with dermatophytic onychomycosis and in their first-degree relatives.

Onychomycosis is known to have predisposing factors and a high prevalence within families that cannot be explained by within-family transmission. We determined the frequency of HLA-B and HLA-DR haplotypes in 25 families of Mexican patients with onychomycosis in order to define the role of the class II major histocompatibility complex (MHC) in genetic susceptibility to this infection. Seventy-eight subjects participated in the study, 47 with onychomycosis and 31 healthy individuals. The frequencies of the HLA-B and HLA-DR haplotypes were compared with those found in first-degree relatives without onychomycosis and in a historic control group of healthy individuals. The frequencies in the controls were similar to those of the healthy relatives of the patients. However, on comparison of the patients with historic controls, we detected a higher frequency of the HLA-DR8 haplotype (P=.03; odds ratio, 1.89; 95% confidence interval, 0.98-36). These findings suggest that there are polymorphisms in genes of the MHC that increase susceptibility to onychomycosis, particularly haplotype HLA-DR8.

[Analysis of genetic polymorphism of the HLA-B and HLA-DR loci in patients with dermatophytic onychomycosis and in their first-degree relatives]. / Análisis del polimorfismo genético de los loci HLA-B y HLA-DR en pacientes con onicomicosis dermatofítica y familiares en primer grado.

Onychomycosis is known to have predisposing factors and a high prevalence within families that cannot be explained by within-family transmission. We determined the frequency of HLA-B and HLA-DR haplotypes in 25 families of Mexican patients with onychomycosis in order to define the role of the class II major histocompatibility complex (MHC) in genetic susceptibility to this infection. Seventy-eight subjects participated in the study, 47 with onychomycosis and 31 healthy individuals. The frequencies of the HLA-B and HLA-DR haplotypes were compared with those found in first-degree relatives without onychomycosis and in a historic control group of healthy individuals. The frequencies in the controls were similar to those of the healthy relatives of the patients. However, on comparison of the patients with historic controls, we detected a higher frequency of the HLA-DR8 haplotype (P=.03; odds ratio, 1.89; 95% confidence interval, 0.98-36). These findings suggest that there are polymorphisms in genes of the MHC that increase susceptibility to onychomycosis, particularly haplotype HLA-DR8.

Lichen sclerosus of the oral mucosa: clinicopathological features of six cases.

Lichen sclerosus is a chronic inflammatory mucocutaneous disease, rarely involving the mouth. There are only 20 well-documented cases of oral lichen sclerosus reported in the English-language literature. This report describes the clinicopathological features of 6 cases of oral lichen sclerosus; 5 in women. There were 12 lesions, mainly on the lips (50%) and buccal mucosa (25%). The affected areas appeared as irregular whitish patches, harder than the surrounding tissue. Half of the patients were symptomatic and presented with no associated skin and/or genital lesions. All cases were biopsied, and histopathological features were evaluated using hematoxylin-eosin and Verhoeff's stains, S-100 immunohistochemical reaction and transmission electron microscopy. Management of the oral lesions consisted of surgical excision, intralesional triamcinolone acetonide, oral colchicine, and regular follow-up. There is no effective curative treatment, but there are some options for patient management; and colchicine may be considered an additional choice.

[Skin metastasis of gastric adenocarcinoma. Case report]. / Metástasis cutáneas de adenocarcinoma gástrico; informe de un caso.

Incidence of cutaneous metastases of malignant tumors has been estimated between 0.7%-10.8%. These lesions generally occur in late stages of disease, being uncommon their presentation at the time of diagnosis. We inform the case of a male patient who presented a metastatic adenocarcinoma in the skin of the chest. The histopathological analysis showed signet ring carcinoma cells. Under the suspect of primary tumor in the gastrointestinal tract, an upper gastrointestinal endoscopy was made corroborating diagnosis of a primary gastric cancer. The case instructs the unusual morphology of cutaneous metastasis of gastric adenocarcinoma, as well appearing as the initial clinical data in an extended malignant disease. Key words: cutaneous metastases, metastatic adenocarcinoma, gastric carcinoma, Mexico.

Giant onychomatricoma: report of two cases with rare clinical presentation.

BACKGROUND: Onychomatricoma was reported for the first time by Baran and Kint as a rare nail matrix tumor with specific clinical and histologic features, including a macroscopic appearance of filiform digitations originating from the nail matrix which are inserted in the nail plate.(1) The appearance of the lesion may resemble that of an "anemone." All previous reports have been mostly from Europe, with only one case from North America. These are the first case reports from Mexico. They show rare clinical characteristics, in particular tumor involving the entire nail matrix. METHODS: Two cases are presented: Case 1, a 59-year-old man with a 2-year history of deformity of the entire nail apparatus of the right thumb, clinically suggestive of onychomatricoma; Case 2, a 45-year-old woman with a 3-year history of gradual nail dystrophy, initially misdiagnosed as onychomycosis and treated unsuccessfully for several months with antimycotics by a general physician. In both cases, the entire nail plate was affected and, because of the clinical appearance, nail exploration and excisional biopsy of the tumors at the nail matrix were performed. RESULTS: During nail matrix surgical exploration, including avulsion of the nail plate, characteristic digitiform projections that were firmly attached to the nail plate were observed. In both cases, the entire nail matrix was affected, producing a giant form of onychomatricoma. Hematoxylin and eosin stain showed characteristic filiform projections that corresponded to the clinical appearance, with elongated epithelium from the nail matrix and fibromyxoid stroma with multiple basophilic cells, and typical clefts in between. CONCLUSIONS: Onychomatricoma has a classical clinical appearance; however, it is difficult to identify, as it is not until surgery, when the typical filiform projections are more visible, that the diagnosis can be made. Onychomatricoma is a rare tumor. The involvement of the entire nail matrix, with secondary nail dystrophy, in both cases presented here makes them interesting case reports of this unusual form of tumor.

Solitary subungual neurofibroma: an uncommon finding and a review of the literature.

We report an uncommon case of solitary subungual neurofibroma on the right first finger of a middle-aged healthy woman. Surgical excision was used for the treatment. Common clinical findings, treatment modalities, and reported recurrence rates in previous studies are reviewed.

[Analysis of HLA-DR in Mexican patients with pemphigus]. / Análisis de HLA-DR en pacientes mexicanos con pénfigo.

UNLABELLED: Pemphigus are a group of bullous skin disorders histologically characterized by intraepidermal acantholytic and circulating antibodies blisters due to directed against the cellular surface of keratinocytes. In Mexican patients with pemphigus, HLA antigens have not been studied as they have been for other populations; for this reason, a comparative, prospective, transversal and observational study has been done with 25 patients, 18 with pemphigus vulgaris and the other seven with pemphigus foliaceus. DNA was extracted by the salting-out method and HLA-DR was determined by amplification with PCR and allele-specific oligonucleotides (ASO). RESULTS: HLA-DR14 (DR6) is more common in patients with pemphigus vulgaris than in the healthy population, which corroborates with previous reports. On the other hand, as reported we also found that HLA-DR1 in Mexican population represents a higher risk for pemphigus foliaceus.

Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report.

Mastocytosis encompasses a range of disorders characterized by overproliferation and accumulation of tissue mast cells. Mast cell disease is most commonly seen in the skin, but the skeleton, gastrointestinal tract, bone marrow, and central nervous system may also be involved. We present a 10-year-old boy with diffuse cutaneous mastocytosis characterized by disseminated papular, nodular, and infiltrated leathery lesions. The patient presented with chronic diarrhea and malnutrition. Laboratory studies were normal except for an elevated urinary 1-methylhistamine level. The bone marrow aspirate showed a dense mast cell infiltrate confirming systemic involvement.

A case of herpetic whitlow associated with erythema multiforme.

We report a 6-month-old girl with erythema multiforme minor occurring during the course of a herpetic whitlow. Erythema multiforme minor is unusual in infants, and in this population herpes simplex virus has not been reported as a significant etiologic factor. The clinical coexistence of erythema multiforme minor and the herpetic lesion is also atypical. To our knowledge, this is the first report of erythema multiforme minor associated with a concomitant HSV infection in an infant.

Lenticular acral keratosis in washerwomen.

BACKGROUND: In 1952, a Brazilian dermatologist, Oswaldo Costa, described a dermatosis characterized by accentuation of the cutaneous folds on the knuckles of both hands and small horny papules on the thenar eminences, posterior surface of the wrists, and the interdigital space between thumb and index finger; he called this entity acrokeratoelastoidosis. Other similar entities, such as focal acral hyperkeratosis and marginal keratoelastoidosis, have been described. The features of the different types of lenticular acral keratosis are discussed. MATERIALS AND METHODS: Fifteen patients with lenticular acral keratosis and five controls were studied clinically and pathologically. The skin biopsies were processed for light and transmission electron microscopy. The clinical data were reviewed, and the following variables were recorded: age, sex, distribution and morphology of the lesions, history of exposure to sunlight and objective evidence of photodamage, familial incidence, occupation and hobbies, time of evolution, and response to previous treatments. The results were compared with samples taken at autopsy from five women without dermatoses. RESULTS: All patients were women, with flat, keratotic papules located on the transition between the dorsal and volar surfaces of the fingers and hands. Histologically, there was an increased amount of elastic fibers, which were coarse and tortuous, and appeared to be interrupted in some areas. In contrast, there were sparse, thin fibers in the mid and deep dermis in the skin of controls. Transmission electron microscopy of these papules showed enlarged, thickened elastic fibers, with deposits of electron-dense, coarse clumps. CONCLUSIONS: Our cases do not seem to correspond to any of the three entities which are manifested clinically by acral keratotic plaques. All of these women washed clothes by hand on a stone washboard for many hours every day. As there is no clinical or histologic evidence of actinic damage, chronic trauma seems to be the cause of the dermatosis in this type of patient. We propose the term "occupational lenticular acral keratosis" for our cases.