Gloria Aparicio

Eruptive juvenile xanthogranuloma associated with relapsing acute lymphoblastic leukemia.

Abstract:  Juvenile xanthogranuloma is a benign, self‐healing disorder with characteristic lesions mainly involving the skin. Although most patients with juvenile xanthogranuloma have only cutaneous symptoms, recent articles have documented extracutaneous manifestations: systemic involvement of many organs has been reported and there is a known association between juvenile xanthogranuloma and childhood leukemia, most commonly juvenile chronic myelogenous leukemia. This case provides further corroboration, that in rare instances, juvenile xanthogranuloma may be associated with hematologic malignancies.

Vegetant Bromoderma in an Infant

Bromoderma is a cutaneous reaction caused by the use of products containing bromide. In this report we describe a 2‐month‐old girl who was admitted to our institution with vegetative lesions on the face and scalp owing to the administration of a syrup containing sodium bromide.

Is there any association between Sarcoidosis and infectious agents?: a systematic review and meta-analysis

BackgroundDuring the last few years, investigators have debated the role that infectious agents may have in sarcoidosis pathogenesis. With the emergence of new molecular biology techniques, several studies have been conducted; therefore, we performed a meta-analysis in order to better explain this possible association.MethodsThis review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement from the Cochrane collaboration guidelines. Four different databases (Medline, Scopus, Web of Science, and Cochrane Collaboration) were searched for all original articles published from 1980 to 2015. The present meta-analysis included case–control studies that reported the presence of microorganisms in samples of patients with sarcoidosis using culture methods or molecular biology techniques. We used a random effects or a fixed-effect model to calculate the odds ratio (OR) and 95% confidence intervals (CI). Sensitivity and subgroup analyses were performed in order to explore the heterogeneity among studies.ResultsFifty-eight studies qualified for the purpose of this analysis. The present meta-analysis, the first, to our knowledge, in evaluation of all infectious agents proposed to be associated with sarcoidosis and involving more than 6000 patients in several countries, suggests an etiological link between Propionibacterium acnes and sarcoidosis, with an OR of 18.80 (95% CI 12.62, 28.01). We also found a significant association between sarcoidosis and mycobacteria, with an OR of 6.8 (95% CI 3.73, 12.39). Borrelia (OR 4.82; 95% CI 0.98, 23.81), HHV-8 (OR 1.47; 95% CI 0.02, 110.06) as well as Rickettsia helvetica, Chlamydia pneumoniae, Epstein-barr virus and Retrovirus, although suggested by previous investigations, were not associated with sarcoidosis.ConclusionThis meta-analysis suggests that some infectious agents can be associated with sarcoidosis. What seems clear is that more than one infectious agent might be implicated in the pathogenesis of sarcoidosis; probably the patient’s geographical location might dictate which microorganisms are more involved. Future investigations and more clinical trials are need to bring these evidences to a more global level.

Evaluation of Cytokine Profile and HLA Association in Benznidazole Related Cutaneous Reactions in Patients With Chagas Disease

BACKGROUND Benznidazole is the drug of choice for Chagas disease. The major drawback of this drug is the high adverse events rate, being cutaneous reactions the most frequent one, leading to definitive withdrawal of treatment in 15%-30% of patients. METHODS Prospective observational study where adult Chagas disease patients accepting to receive benznidazole (100 mg/8 hours for 60 days) were included. The objective was to characterize the skin toxicity of benznidazole in patients with Chagas disease, determine the serum cytokine profile, and evaluate the potential association with specific HLA alleles and benznidazole concentration. Serum cytokine levels were measured at day 0, 15, and 60 of treatment. Class I and II HLA alleles were determined. When cutaneous reaction was detected, a skin biopsy was performed. Serum benznidazole concentration was determined at the time of cutaneous reaction, or at day 15 of treatment. RESULTS Fifty-two patients were included, 20(38.5%) had cutaneous reaction, and median time of appearance was 9 days. Skin biopsies showed histopathological findings consistent with drug eruption. Patients with cutaneous drug-reaction had higher proportion of eosinophilia during treatment, and higher interleukin (IL)-5 and IL-10 serum concentrations at day 15 of treatment than those without cutaneous reaction. Treatment interruption (that included moderate-severe cutaneous reactions) was more frequent in patients carrying HLA-B*3505 allele (45.5% vs 15.4%, P = .033). No differences in benznidazole serum concentration were found. CONCLUSIONS Benznidazole related cutaneous reaction rate is high, and it was produced by a delayed hypersensitivity reaction with a Th2 response. Carrying HLA-B*3505 allele could be associated with moderate-severe cutaneous reaction.

Pitiriasis rubra pilaris aguda postinfecciosa: una dermatosis mediada por superantígenos

Resumen La pitiriasis rubra pilaris (PRP) aguda postinfecciosa es una variante de la forma juvenil de PRP (tipo III de Griffiths) caracterizada por la ausencia de antecedentes familiares, curso agudo relacionado con un episodio febril previo y buen pronostico. Clinicamente puede simular otras enfermedades mediadas por superantigenos, como los exantemas escarlatiniformes o el sindrome de la escaldadura estafilococica; sin embargo, su histologia y tratamiento son distintos. Presentamos 4 casos de PRP aguda postinfecciosa que ilustran las caracteristicas clinicas de este proceso infrecuente y revisamos los posibles mecanismos fisiopatogenicos subyacentes.

Prognostic value of skin lesions in sarcoidosis: clinical and histopathological clues

BackgroundAlthough tremendous advances have been made, a significant gap exists between the vast knowledge accumulated concerning sarcoidosis in recent years and our understanding of this disease.ObjectiveDescribe the main clinical and histopathological findings associated with cutaneous sarcoidosis and to investigate the relationship of these skin lesions with systemic involvement.Materials and MethodsAretrospective review of 41 patients who were diagnosed with cutaneous sarcoidosis was done.ResultsThe study included 34 females and 7 males. Systemic disease occurred frequently in patients with lupus pernio and nodulo-plaque type lesions. Systemic symptoms were observed more commonly in patients with raised serum ACE levels (84.6% vs. 40%; p<0.05). Our study also indicated that patients with skin lesions that were associated with systemic symptoms had a more chronic form of the disease than patients with only cutaneous lesions (91.6% vs. 29.4%; p<0.001). Additionally, complete resolution of cutaneous lesions was observed more frequently in patients with no associated systemic symptoms (66.6% vs. 23.5%; p<0.05). Interestingly, we found that patients with a moderate/severe granulomatous infiltrate in their biopsies had a more severe clinical presentation during the course of the disease, with a more generalized skin involvement (65.6% vs. 30%) as well as a more chronic course of the disease (56.3% vs. 30%). Another interesting histopathological finding observed was the presence of a grenz zone in 20 cases (47.6%).ConclusionsA correct and methodical clinicopathological correlation is important for our clinical practice because it can give us useful clues to the diagnosis and prognosis of this disease.

Acute Postinfectious Pityriasis Rubra Pilaris: A Superantigenmediated Dermatosis

Acute postinfectious pityriasis rubra pilaris (PRP) is a variant of juvenile PRP (Griffiths type III) characterized by no family history, an acute course associated with a prior fever, and good prognosis. Clinical features may resemble other superantigen-mediated diseases, such as scarlatiniform rash or staphylococcal scalded skin syndrome, but its histology and treatment are different. We present 4 cases of acute postinfectious PRP that illustrate the clinical features of this uncommon disease and we review possible underlying pathogenic mechanisms.

Cutaneous infections by dematiaceous opportunistic fungi: Diagnosis and management in 11 solid organ transplant recipients

The incidence of cutaneous infections by dematiaceous fungi is rising in our environment due to the high number of solid organ transplant recipients (SOTR).

Utility of Ultrasound in a Rapidly Growing Cutaneous Nodule in an Infant

A healthy 8-year-old girl consulted for a rapidly growing nodule on her left arm, which had appeared after a contusion 6 weeks earlier. Physical examination results revealed an indurated nodule, 2.5 cm in diameter, violaceous, with a pseudoblisterous surface (Figure 1; available at www.jpeds.com). Ultrasonography showed a well-defined subcutaneous nodule with central hyperechoic points, peripheral hypoechoic rim, and a posterior acoustic shadowing; echo-Doppler scanning revealed increased peripheral vascularity. All of this is compatible with pilomatrixoma (Figure 2). Surgical excision was performed and histopathologic examination showed a well-demarcated subcutaneous tumor, with a peripheral proliferation of basaloid cells, central eosinophilic shadow cells, and areas of calcification. The overlying dermis showed a lax tissue with dilated lymphatic vessels and without elastic fibers, consistent with anetodermic pilomatrixoma (Figure 3; available at www.jpeds.com). Pilomatrixoma, a benign appendage tumor derived from the matrix cells of the hair follicle, appears mainly in children or young adults and is normally located in the head, neck, and upper extremities. It is usually solitary, asymptomatic, with a slow-growing pattern and with a characteristic histology. Ultrasonography is an accessible, quick, and noninvasive imaging tool; it is gaining an important role in the diagnosis of cutaneous lesions, especially in pediatric patients. It has been seen that the combination of central internal heterogeneous echo texture by scattered pattern calcifications,peripheral hypoechoic rim,and subsequentposterior shadowing could be discriminative ultrasonographic criteria for differentiating pilomatrixoma from other subcutaneous tumours. Anetodermic pilomatrixoma is rare. Clinically, it is characterized by atrophic, wrinkled, pseudoblisterous, or bulla-like changes of the skin overlying the tumor. Preceding mechanical trauma at the site of occurrence of the tumor has been reported, as is the case in our patient. It would lead to the disruption of the dermal integrity and cutaneous microcirculation responsible of the clinical appearance of the tumor. ■