João Avancini

Drug reaction with Eosinophilia and Systemic Symptoms (DRESS) / Drug-induced Hypersensitivity Syndrome (DIHS): a review of current concepts

The Drug Reaction with Eosinophilia and Systemic Symptoms syndrome, also known as Drug Induced Hypersensitivity Syndrome presents clinically as an extensive mucocutaneous rash, accompanied by fever, lymphadenopathy, hepatitis, hematologic abnormalities with eosinophilia and atypical lymphocytes, and may involve other organs with eosinophilic infiltration, causing damage to several systems, especially to the kidneys, heart, lungs, and pancreas. Recognition of this syndrome is of paramount importance, since the mortality rate is about 10% to 20%, and a specific therapy may be necessary. The pathogenesis is related to specific drugs, especially the aromatic anticonvulsants, altered immune response, sequential reactivation of herpes virus and association with HLA alleles. Early recognition of the syndrome and withdrawal of the offending drug are the most important and essential steps in the treatment of affected patients. Corticosteroids are the basis of the treatment of the syndrome, which may be associated with intravenous immunoglobulin and, in selected cases, Ganciclovir. The article reviews the current concepts involving this important manifestation of adverse drug reaction.

Drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome: clinical features of 27 patients

Drug reaction with eosinophilia and systemic symptoms (DRESS) [also called drug‐induced hypersensitivity syndrome (DIHS)] includes severe reactions to drugs that need to be promptly recognized by physicians.

Development of Type 2, But Not Type 1, Leprosy Reactions is Associated with a Severe Reduction of Circulating and In situ Regulatory T-Cells

Leprosy is frequently complicated by the appearance of reactions that are difficult to treat and are the main cause of sequelae. We speculated that disturbances in regulatory T-cells (Tregs) could play a role in leprosy reactions. We determined the frequency of circulating Tregs in patients with type 1 reaction (T1R) and type 2 reaction (T2R). The in situ frequency of Tregs and interleukin (IL)-17, IL-6, and transforming growth factor beta (TGF)-β-expressing cells was also determined. T2R patients showed markedly lower number of circulating and in situ Tregs than T1R patients and controls. This decrease was paralleled by increased in situ IL-17 expression but decreased TGF-β expression. Biopsies from T1R and T2R patients before the reaction episodes showed similar number of forkhead box protein P3+ (FoxP3+) and IL-17+ cells. However, in biopsies taken during the reaction, T2R patients showed a decrease in Tregs and increase in IL-17+ cells, whereas T1R patients showed the opposite: Tregs increased but IL-17+ cells decreased. We also found decreased expansion of Tregs upon in vitro stimulation with Mycobacterium leprae and a trend for lower expression of FoxP3 and the immunosuppressive molecule cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) in T2R Tregs. Our results provide some evidence to the hypothesis that, in T2R, downmodulation of Tregs may favor the development of T-helper-17 responses that characterize this reaction.

Nail Sarcoidosis with and without Systemic Involvement: Report of Two Cases.

The most commonly seen nail change in sarcoidosis, often associated with chronic systemic disease, is onychodystrophy. Imaging of hands, feet and chest is of paramount importance when nail sarcoidosis is suspected, for evaluation of bone cysts and pulmonary involvement, respectively. We report 2 cases of sarcoidosis that developed nail involvement - one with pulmonary disease, and the other without any evidence of systemic involvement, which is very rare.

Primary cutaneous histoplasmosis developed in the penis of an immunocompetent patient

A 70-year-old male presenting a 3-month history of genital painless erythematous nodules in the balanopreputial sulcus was referred to our service. Histopathological exam presented a chronic dermatitis with epithelioid granulomas and Grocott staining revealed numerous fungal structures with a suggestive morphology of Histoplasma sp. Cultures evidenced Histoplasma capsulatum var. capsulatum. Treatment with oral itraconazole led to complete remission of lesions.

Histoid leprosy: clinical and histopathological analysis of patients in follow-up in University Clinical Hospital of endemic country

Histoid leprosy (HL) is a rare form of lepromatous leprosy, characterized by hyperchromic indurated nodules above normal skin. Its main histopathological aspect is spindle cells. Because it may simulate other aspects, such as dermatofibroma and neurofibroma, histoid leprosy poses itself as a diagnostic challenge.

Prevalence of dermatoses in patients referred for evaluation in an outpatient clinic of specialties

BACKGROUND Brazilian Unified Health System presents a long waiting period for a dermatology appointment, varying from 34 to 239 days. OBJECTIVES Analyze the prevalence of cutaneous diseases evaluated by dermatologists in a specialized center, in patients referred from the primary care, and to present possible interventions for the primary units. METHODS Retrospective analyses of the International Codes of Diseases (ICD-10) described in every dermatology appointment in a specialty center in Sao Paulo from January 2014 to August 2015. RESULTS A total of 7.350 consultations were included. Superficial mycosis corresponded to 1,058 (14.4%) of the main complaints and dermatophytosis was the most frequently used ICD, corresponding to 481 individual consultations (6.5%), followed by onychomycosis, responsible for 464 consultations (6.3%), acne in 347 (4,7%). and contact dermatitis in 311 consultations (4,2%). STUDY LIMITATIONS The study was based on retrospective analysis of ICD described and no previous orientation for a solid use of the codes was performed to the dermatology team; consultations in which the ICD was not informed or a non-especific ICD was used were excluded; different dermatologists were responsible for the consultations. CONCLUSION Superficial mycosis corresponded to 14.4% of the chief complaints in the studied period and was the most frequent cause of reference from primary care doctors to dermatologists. Prevalence data obtained in the present study could assist the capacitation policies in the primary care system, focusing the dermatology teaching in the most prevalent dermatological disorders.Background Brazilian Unified Health System presents a long waiting period for a dermatology appointment, varying from 34 to 239 days. Objectives Analyze the prevalence of cutaneous diseases evaluated by dermatologists in a specialized center, in patients referred from the primary care, and to present possible interventions for the primary units. Methods Retrospective analyses of the International Codes of Diseases (ICD-10) described in every dermatology appointment in a specialty center in Sao Paulo from January 2014 to August 2015. Results A total of 7.350 consultations were included. Superficial mycosis corresponded to 1,058 (14.4%) of the main complaints and dermatophytosis was the most frequently used ICD, corresponding to 481 individual consultations (6.5%), followed by onychomycosis, responsible for 464 consultations (6.3%), acne in 347 (4,7%). and contact dermatitis in 311 consultations (4,2%). Study limitations The study was based on retrospective analysis of ICD described and no previous orientation for a solid use of the codes was performed to the dermatology team; consultations in which the ICD was not informed or a non-especific ICD was used were excluded; different dermatologists were responsible for the consultations. Conclusion Superficial mycosis corresponded to 14.4% of the chief complaints in the studied period and was the most frequent cause of reference from primary care doctors to dermatologists. Prevalence data obtained in the present study could assist the capacitation policies in the primary care system, focusing the dermatology teaching in the most prevalent dermatological disorders.

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

BackgroundErythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation.Case presentationWe report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO’s multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ + over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma.ConclusionsThis case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.

Angiosarcoma in HIV-negative patients is not associated with HHV-8.

BACKGROUND Angiosarcoma is an aggressive, malignant neoplasm of vascular or lymphatic origin. Herpes virus 8 (HHV-8) is a member of the herpes family with a tropism for endothelial cells and it has been proven to induce vascular neoplasms, such as Kaposis sarcoma. The role of HHV-8 in the pathogenesis of angiosarcoma has not been well defined. OBJECTIVE To investigate the relationship between the presence of HHV-8 and angiosarcoma. METHODS In this study, the team investigated the relationship between the presence of HHV-8, as determined by polymerase chain reaction, and angiosarcoma, using samples from patients with epidemic Kaposis sarcoma as controls. RESULTS While all control cases with epidemic Kaposis sarcoma were positive for HHV-8, none of the angiosarcoma cases was. CONCLUSION These findings support most previous studies that found no association between HHV-8 and angiosarcoma.