Necrotic erythema nodosum reaction associated with histological alterations of Lucio's phenomenon

Abstract Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio's phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Necrotic erythema nodosum reaction associated with histological alterations of Lucio's phenomenon.

Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio's phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Case for diagnosis. Erythematous and infiltrated plaques in the infrahyoid region

Abstract Leprosy is a chronic infectious disease caused by Mycobacterium leprae and, depending on the host immune status, presents different clinical forms. This report describes the case of a 46-year-old man who had hypoesthetic lesions in the infrahyoid region for 30 days. The bacilloscopy was negative. The anatomopathological examination showed alterations corresponding to the tuberculoid pole (epithelioid histiocytes) and virchowian pole (foamy histiocytes), compatible with borderline-virchowian leprosy (Ridley and Jopling classification). Rapid tests for HIV I, II, and syphilis were positive, with a CD4 count of 223. The patient started treatment with multibacillary multidrug therapy, antiretroviral therapy, and benzathine penicillin, with marked clinical improvement in two months.

Case for diagnosis. Erythematous and infiltrated plaques in the infrahyoid region.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae and, depending on the host immune status, presents different clinical forms. This report describes the case of a 46-year-old man who had hypoesthetic lesions in the infrahyoid region for 30 days. The bacilloscopy was negative. The anatomopathological examination showed alterations corresponding to the tuberculoid pole (epithelioid histiocytes) and virchowian pole (foamy histiocytes), compatible with borderline-virchowian leprosy (Ridley and Jopling classification). Rapid tests for HIV I, II, and syphilis were positive, with a CD4 count of 223. The patient started treatment with multibacillary multidrug therapy, antiretroviral therapy, and benzathine penicillin, with marked clinical improvement in two months.

Atypical crusted scabies in a patient with chronic liver disease caused by hepatitis B and D viruses.

Crusted scabies is a less common variant of scabies that is highly contagious, difficult to treat and involves infestation by Sarcoptes scabiei var. hominis. The classical clinical presentation includes crusted, scaly and generally non-pruritic lesions usually located on the head, neck, palmar, plantar and periungual region. It was first described in Norway in 1848 in patients with leprosy who presented with crusted lesions. In this study, we report the case of a patient with crusted scabies with florid clinical manifestations and chronic liver disease due to hepatitis B and delta virus infection.

Atypical crusted scabies in a patient with chronic liver disease caused by hepatitis B and D viruses

Abstract: Crusted scabies is a less common variant of scabies that is highly contagious, difficult to treat and involves infestation by Sarcoptes scabiei var. hominis. The classical clinical presentation includes crusted, scaly and generally non-pruritic lesions usually located on the head, neck, palmar, plantar and periungual region. It was first described in Norway in 1848 in patients with leprosy who presented with crusted lesions. In this study, we report the case of a patient with crusted scabies with florid clinical manifestations and chronic liver disease due to hepatitis B and delta virus infection.

Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area.

Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area

Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

Lacaziosis - unusual clinical presentation.

Lacaziosis or Jorge Lobo's disease is a fungal, granulomatous, chronic infectious disease caused by Lacazia loboi, which usually affects the skin and subcutaneous tissue. It is characterized by slow evolution and a variety of cutaneous manifestations with the most common clinical expression being nodular keloid lesions that predominate in exposed areas. We report the case of a patient who had an unusual clinical presentation, with a single-plated lesion on the back. Histopathological examination confirmed the diagnosis of Lacaziosis.

T regulatory cells (TREG)(TCD4+CD25+FOXP3+) distribution in the different clinical forms of leprosy and reactional states.

BACKGROUND: Leprosy is characterized histologically by a spectrum of different granulomatous skin lesions, reflecting patients' immune responses to Mycobacterium leprae. Although CD4+CD25+ FoxP3+ T regulatory cells are pivotal in the immuneregulation, presence, frequency, and distribution of Tregs in leprosy, its reactional states have been investigated in few studies. OBJECTIVES: This study aimed to verify the frequency and distribution of regulatory T cells in different clinical forms and reactional states of leprosy. METHODS: We performed an immunohistochemical study on 96 leprosy cases [Indeterminate (I): 9 patients; tuberculoid tuberculoid: 13 patients; borderline tuberculoid: 26 patients; borderline borderline: 3 patients; borderline lepromatous: 8 patients; lepromatous lepromatous: 27 patients; reversal reaction: 8 patients; and erythema nodosum leprosum: 2 patients]. RESULTS: FoxP3-positive cells were present in 100% of the cases with an average density of 2.82% of the infiltrate. Their distribution was not related to granulomatous structures or special locations. There was a statistically significant increment of FoxP3 expression in patients with leprosy reversal reactions when compared with patients presenting with type I leprosy (P= 0.0228); borderline tuberculoid leprosy (P = 0.0351) and lepromatous leprosy (P = 0.0344). CONCLUSIONS: These findings suggest that Tregs play a relevant role in the etiopathogenesis of leprosy, mainly in type I leprosy reaction.

T regulatory cells (TREG)(TCD4+CD25+FOXP3+) distribution in the different clinical forms of leprosy and reactional states

BACKGROUND: Leprosy is characterized histologically by a spectrum of different granulomatous skin lesions, reflecting patients' immune responses to Mycobacterium leprae. Although CD4+CD25+ FoxP3+ T regulatory cells are pivotal in the immuneregulation, presence, frequency, and distribution of Tregs in leprosy, its reactional states have been investigated in few studies. OBJECTIVES: This study aimed to verify the frequency and distribution of regulatory T cells in different clinical forms and reactional states of leprosy. METHODS: We performed an immunohistochemical study on 96 leprosy cases [Indeterminate (I): 9 patients; tuberculoid tuberculoid: 13 patients; borderline tuberculoid: 26 patients; borderline borderline: 3 patients; borderline lepromatous: 8 patients; lepromatous lepromatous: 27 patients; reversal reaction: 8 patients; and erythema nodosum leprosum: 2 patients]. RESULTS: FoxP3-positive cells were present in 100% of the cases with an average density of 2.82% of the infiltrate. Their distribution was not related to granulomatous structures or special locations. There was a statistically significant increment of FoxP3 expression in patients with leprosy reversal reactions when compared with patients presenting with type I leprosy (P= 0.0228); borderline tuberculoid leprosy (P = 0.0351) and lepromatous leprosy (P = 0.0344). CONCLUSIONS: These findings suggest that Tregs play a relevant role in the etiopathogenesis of leprosy, mainly in type I leprosy reaction. .

Nosological profile in a dermatology referral center in the state of Amazonas -Brazil.

BACKGROUNDS: Fundaments: Skin diseases are associated wih high morbidity, low mortality and low rate of hospitalization. However, they can cause considerable interference in physical and emotional well-being of the individual. Several of them reach large population, requiring specific interventions for their control. OBJECTIVE: To describe the frequency of skin disease diagnosed in the dermatology service in Manaus, capital of Amazonas State. METHODS: We collected data on registered sex, age, origin and diagnostics for the first consultation of patients attended between January 2000 and December 2007. RESULTS: Of the 56.024 recorded visits, we obtained 56.720 cases of dermatological diagnoses, being the most common sexually transmitted diseases (25,12%), allergic skin disesases (14,03%), unspecified dermatoses (13,01%), leprosy (6,34%) and acne, seborrhea and related diseases (5,05%). The frequency was similar for both sexes, aged 20-29 years predominated and Manaus the origin most reported. CONCLUSIONS: The pattern of skin diseases identified in this study may serve as a baseline to managers of health system in the region develop strategies for prevention and control of dermatoses, with emphasis on sexually transmitted diseases, allergic skin diseases, leprosy and acne.

Perfil nosológico de centro de referência em dermatologia no estado do Amazonas - Brasil / Nosological profile in a dermatology referral center in the state of Amazonas -Brazil

FUNDAMENTOS: As doenças de pele estão associadas a alta morbidade, baixa mortalidade e baixa proporção de hospitalização. Entretanto, podem causar considerável interferência no bem-estar físico e emocional do indivíduo. Várias delas atingem grandes contingentes populacionais, havendo necessidade de intervenções específicas para seu controle. OBJETIVO: Descrever a frequência das dermatoses diagnosticadas em serviço de dermatologia na cidade de Manaus, capital do estado do Amazonas. MÉTODOS: Coletaram-se dados registrados sobre sexo, idade, procedência e diagnósticos referentes à primeira consulta dos pacientes atendidos entre janeiro de 2000 e dezembro de 2007. RESULTADOS: Das 56.024 consultas registradas, obtiveram-se 56.720 diagnósticos dermatológicos, sendo mais comuns as doenças sexualmente transmissíveis (25,12 por cento), as dermatoses alérgicas (14,03 por cento), as dermatoses não especificadas (13,01 por cento), a hanseníase (6,34 por cento) e acne, seborreia e afins (5,05 por cento). A frequência foi semelhante para ambos os sexos, a faixa etária de 20-29 anos foi predominante e Manaus foi a procedência mais referida. CONCLUSÕES: O padrão das doenças cutâneas identificadas neste estudo pode servir como linha de base para que gestores do sistema de saúde da região desenvolvam estratégias de prevenção e controle das dermatoses mais comuns, com ênfase nas doenças sexualmente transmissíveis, doenças cutâneas alérgicas, hanseníase e acne.

BACKGROUNDS: Fundaments: Skin diseases are associated wih high morbidity, low mortality and low rate of hospitalization. However, they can cause considerable interference in physical and emotional well-being of the individual. Several of them reach large population, requiring specific interventions for their control. OBJECTIVE: To describe the frequency of skin disease diagnosed in the dermatology service in Manaus, capital of Amazonas State. METHODS: We collected data on registered sex, age, origin and diagnostics for the first consultation of patients attended between January 2000 and December 2007. RESULTS: Of the 56.024 recorded visits, we obtained 56.720 cases of dermatological diagnoses, being the most common sexually transmitted diseases (25,12 percent), allergic skin disesases (14,03 percent), unspecified dermatoses (13,01 percent), leprosy (6,34 percent) and acne, seborrhea and related diseases (5,05 percent). The frequency was similar for both sexes, aged 20-29 years predominated and Manaus the origin most reported. CONCLUSIONS: The pattern of skin diseases identified in this study may serve as a baseline to managers of health system in the region develop strategies for prevention and control of dermatoses, with emphasis on sexually transmitted diseases, allergic skin diseases, leprosy and acne.

T regulatory cells and plasmocytoid dentritic cells in hansen disease: a new insight into pathogenesis?

Leprosy is characterized by spectrum of histologically different granulomatous skin lesions that reflects the patient's immune response to Mycobacterium leprae. Presence, frequency, and distribution of both CD4+ CD25+ FoxP3+ T regulatory cells (T-regs) and CD123+ plasmacytoid dendritic cells in leprosy have never been investigated. We performed a retrospective immunohistochemical study on 20 cases of leprosy [tuberculoid tuberculoid (TT): 1 patient; borderline tuberculoid (BT): 3 patients; borderline lepromatous (BL): 5 patients; lepromatous lepromatous (LL): 5 patients; borderline borderline in reversal reaction (BB-RR): 1 patient; BT-RR: 2 patients; and erythema nodosum leprosum (ENL): 3 patients]. FoxP3-positive cells were present in 95% of the cases with an average density of 2.9% of the infiltrate. Their distribution was not related to granulomatous structures or special locations. There was no statistical difference of FoxP3 expression between TT, BT, BL, and LL, whereas a statistical significant increment (P = 0.042) was observed in patients affected by reversal leprosy reactions (BT-RR and BB-RR) compared with patients affected by ENL and patients with nonreactional disease forms (BL, LL, BT, TT). CD123 expression was not observed in any of the biopsy specimens evaluated; with the exception of 2 cases of ENL, in which a focal positivity for CD123 was observed. Our results show that plasmacytoid dendritic cells are not involved in the immune response against M. leprae while T-regs are present in leprosy skin lesions. These data raise the question if T-regs have a pathogenetic role in HD as previously demonstrated in Leishmania major and Mycobacterium tuberculosis.

Hanseníase históide de localização restrita / Histoid leprosy of restricted location

A hanseníase permanece importante problema de saúde pública, e os técnicos de unidades básicas de saúde devem estar capacitados para reconhecer as diversas formas de apresentação clínica da doença. Relata-se caso de paciente com lesões pouco habituais, cujo diagnóstico foi importante por se tratar de doente multibacilar, principal elo da cadeia de transmissão da doença. O diagnóstico e tratamento oportuno desses casos contribuem para atingir as metas de eliminação da doença no país.

Leprosy remains a public health problem in Brazil. Every health professional at the primary health care level should be able to detect all presentation forms of leprosy. We report the case of a multibacillary patient with unusual lesions, whose diagnosis was important to interfere in the transmission chain. Diagnosis and adequate treatment contribute to reach the leprosy elimination goal in our country.

Lacaziose simulando hanseníase dimorfa tuberculóide / Lacaziosis mimicking borderline tuberculoid leprosy

Lacaziose é infecção crônica causada pelo fungo Lacazia loboi, originalmente descrita por Jorge Lobo (1931) em paciente oriundo da Amazônia brasileira. Manifesta-se, sobretudo, por lesões cutâneas queloidiformes. Relata-se o caso de paciente com placa eritêmato-violácea, cuja hipótese diagnóstica inicial foi de hanseníase dimorfa tuberculóide, doença também endêmica na região amazônica. Ressalta-se a importância da inclusão de formas paucibacilares de hanseníase no diagnóstico diferencial da lacaziose.

Lacaziosis is a chronic skin infectious disease caused by the fungus Lacazia loboi, which usually results in indolent cutaneous keloid-like lesions. It was first described by Jorge Lobo, in 1931, in a patient from the Amazon region. We report a case of lacaziosis, manifested by an erythematous infiltrated plaque, causing misdiagnosis with paucibacillary leprosy, since both diseases are endemic in Amazon basin.