Histoid leprosy presenting as a large tumor

Abstract Histoid leprosy is a rare form of multibacillary leprosy, characterized by the presence of papules, plaques, or nodules whose appearance is keloid-like, skin colored, or erythematous. Fusiform cells are the main histopathological feature. Due to the fact that it can simulate other dermatological lesions, for example, dermatofibroma and neurofibroma, it constitutes a diagnostic challenge for clinicians and pathologists. It is a bacilliferous form of leprosy, and it plays an important role in disease transmission. A case of a patient with histoid leprosy living in the Northeast Region of Brazil is reported.

Lepromatous leprosy, melanoma, and basal cell carcinoma: clinical-histopathologic association

Abstract Cutaneous neoplasms frequently occur in leprosy, but there are few reports of the coexistence of leprosy and basal cell carcinoma in the same lesion. This case reports a 49-year-old male with an ulcerated plaque on the right lateral nasal wall, bright papules on the sternal region, and a blackened plaque on the right temporal region. The nasal and temporal lesions were diagnosed by histopathology as basal cell carcinoma and melanoma, respectively. The sternal lesions were excised with the repair of the "dog ear" which histopathological examination showed macrophages in the dermis parasitized with acid-fast bacilli, confirming the diagnosis of lepromatous leprosy with Fite-Faraco staining. This case report highlights the importance of referring the dog-ear specimen for histopathologic analysis.

Células gigantes en lepra lepromatosa: dermatitis difusa con células gigantes exuberantes de tipo cuerpo extraño en lepra lepromatosa tratada / Giant cells lepromatous leprosy: Diffuse dermatitis with exuberant foreign body giant cells in treated lepromatous leprosy

Resumen Los pacientes con lepra lepromatosa que han recibido tratamiento durante años, usualmente requieren seguimiento con biopsias de piel para detectar lesiones persistentes o si la baciloscopia es positiva, incluso si los valores son menores que los iniciales. Se presenta el caso de una mujer de 48 años de edad con lepra lepromatosa de 15 años de evolución, índice bacilar de 4 en el extendido directo y en la biopsia, que recibió tratamiento con múltiples medicamentos durante 32 meses, aunque lo recomendado por la Organización Mundial de la Salud (OMS) es una duración de 12 meses. Se tomó una biopsia de piel para determinar si la enfermedad estaba activa. Se observó inflamación dérmica difusa con numerosas células gigantes de tipo cuerpo extraño y macrófagos vacuolados (células de Virchow). Estas células, CD68 positivas, contenían material granular ácido-alcohol resistente positivo con inmunohistoquímica para BCG. Se encontraron bacilos fragmentados y el índice bacilar fue de 2. Se interpretó como una forma residual de lepra lepromatosa y se concluyó que la paciente no requería prolongar el tratamiento con múltiples medicamentos. Este perfil histológico se ha observado en casos similares, pero sin datos clínicos estas biopsias representan un reto diagnóstico. La acumulación de lípidos en estas células gigantes se debe a la destrucción bacilar y a la fusión de macrófagos vacuolados. Se revisó el papel de los lípidos del bacilo y del huésped en la patogenia de la lepra lepromatosa. En estos casos, no es necesario extender los 12 meses de tratamiento con múltiples medicamentos recomendados por la OMS. En el seguimiento de los pacientes, se recomienda contar con los hallazgos clínicos, la baciloscopia, la biopsia anual de piel y los títulos IgM antiglucolípido fenólico.

Abstract Patients with lepromatous leprosy that have received treatment for many years usually get follow up biopsies for persistent skin lesions or positive bacilloscopy even if the values are lower than in the initial bacilloscopy. We report the case of a 48-year old woman with long-standing lepromatous leprosy of 15 years of evolution, with a bacterial index of 4 in the direct smear and the initial skin biopsy. The patient was treated with multidrug therapy for 32 months although the treatment recommended by the World Health Organization (WHO) is only for 12 months. A skin biopsy was taken to determine if there was an active disease. We observed a diffuse dermal inflammation with numerous foreign body giant cells and vacuolated macrophages (Virchow´s cells). These cells contained granular acid-fast material that was also positive with immunohistochemistry for BCG. There were fragmented bacilli and the BI was 2. These cells were also strongly positive for CD68. The biopsy was interpreted as a residual form of lepromatous leprosy that did not require further multidrug therapy. We have observed similar histological profiles in several cases. The lack of clinical data makes it a histological challenge. The accumulation of lipids in these giant cells is due to bacillary destruction and fusion of vacuolated macrophages. We discuss here the role of bacillary and host lipids in the pathogenesis of lepromatous leprosy. We concluded that there was no need to extend the 12-month multidrug therapy recommended by WHO. Clinical findings, bacilloscopy, annual skin biopsy, and anti-phenolic glycolipid-I IgM titers are recommended procedures for the follow-up of these patients.

Erythroderma as a manifestation of leprosy

Abstract: Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.

Leprosy and American cutaneous leishmaniasis coinfection

Abstract: Brazil is a country with a high prevalence of infectious diseases such as leprosy and leishmaniasis. However, coinfection of these diseases is still poorly understood. We report a case of a patient who presented with lepromatous leprosy and cutaneous-mucosal leishmaniasis at the same period. After clinical, laboratory, and histopathological diagnosis, the treatment was introduced and the patient showed important clinical improvement. He was followed in our outpatient clinic. Both pathologies play an important role in the immune system. Depending on the immune response profile of the host, diseases may present themselves in different ways. In this case, the patient showed a divergent immune response for each disease. We hypothesized that this response is specific for each pathogen.

Lepromatous leprosy simulating rheumatoid arthritis - Report of a neglected disease

Abstract Brazil has the second largest number of leprosy cases in the world; nevertheless, late diagnosis is common. We report the case of a male patient with pain and numbness in both hands and feet for six years with positive rheumatoid factor and anticardiolipin under rheumatoid arthritis treatment for five years. Examination revealed diffuse cutaneous infiltration and leonine facies, characteristic features of lepromatous leprosy. Autoantibodies such as rheumatoid factor and anticardiolipin are markers of rheumatic autoimmune diseases, but their presence is also described in leprosy. We report the present case in order to alert health professionals to remember leprosy, even in areas where the disease is considered eliminated as a public health problem, avoiding misinterpretations of serologic findings and misdiagnosis.

Lucio's phenomenon: exuberant case report and review of Brazilian cases

Abstract Lucio’s phenomenon is an uncommon reaction characterized by severe necrotizing cutaneous lesions that occurs in patients with Lucio’s leprosy and lepromatous leprosy. It is considered by some authors as a variant of type 2 or 3 reaction. Death can occur because of blood dyscrasia or sepsis. Precipitating factors include infections, drugs and pregnancy. We report a 31-year-old female patient exhibiting both clinical and histopathological features of lepromatous leprosy and Lucio’s phenomenon presenting favorable response to treatment. We complement our report with a literature review of the Brazilian cases of Lucio’s phenomenon published in Portuguese and English.

Case for diagnosis

Hansen's disease is a chronic infecto-contagious disease caused by Mycobacterium leprae. The bacillus prefers low-temperature areas and the nose is usually the initial site of lesions. Transmission of the bacilli occurs by nasal and oropharyngeal secretions, and through solutions of continuity of the skin and/or mucosae. Nasal manifestations are found in the later stages of the disease.

Verrucous lepromatous leprosy: a rare form of presentation - Report on two cases

Leprosy skin lesions are described as hypochromic or erythematous macules, pale erythematous or reddish-brown plaques, papules, nodules, and diffuse cutaneous infiltration, depending on the clinical form of the disease. They may be accompanied by hypo or anesthesia, alopecia, and hypo or anhidrosis. Verrucous lesions are now quite uncommon in leprosy. The literature is sparse, with only 25 reported cases of this association, especially in the lepromatous pole of the disease. This work is a report on two cases of lepromatous leprosy of long evolution, coursing with vegetant verrucous lesions.

Tufted angioma in ear auricle: importance of the differential diagnosis / Hemangioma em penacho no pavilhao auricular: importancia do diagnostico diferencial

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.

O Hemangioma em penacho é uma proliferação vascular adquirida de características histológicas peculiares. Sua característica clínica mais comum são máculas eritematosas. Ocorre predominantemente em crianças e adultos jovens, principalmente no tórax e pescoço. Relata-se o caso de paciente do sexo masculino, 12 anos, com aumento do pavilhão auricular direito associado a máculas eritematosas e aumento da temperatura local, encaminhado com diagnóstico de Hanseníase Virchowiana, entretanto, tal diagnóstico foi questionado. Histopatologia compatível com Hemangioma em penacho.

Lucio's Phenomenon. Case study of an exceptional response to treatment exclusively with multibacillary multidrug therapy / Fenomeno de Lucio. Relato de um caso exuberante com excelente resposta ao tratamento com Poliquimioterapia Multibacilar

Lucio's phenomenon represents a serious cutaneous necrotizing reaction, which can occur with Lucio's leprosy and also in other forms of lepromatous leprosy. The authors discuss the case of a 63-year-old male patient presenting with ulcers and necrotic cutaneous lesions on the limbs, torso and face, associated with recurring sinusitis, hoarseness and a weight loss of 25 kilos over the last year. Bacilloscopy of intradermal scrapings showed a bacilloscopic index of 5 and the histopathological findings were compatible with the diagnosis of Lucio's phenomenon. The chosen treatment was exclusively multibacillary multidrug therapy, resulting in important dermatological improvement after one month of therapy. The authors present an impressive case of Lucio's phenomenon with an exceptional response to treatment exclusively with multibacillary multidrug therapy.

O fenômeno de Lúcio representa uma reação cutânea necrosante grave que pode ocorrer na hanseníase de Lúcio e em outras formas de hanseníase virchowiana. Os autores relatam o caso de um paciente masculino de 63 anos de idade apresentando há um ano lesões ulceronecróticas nos membros, lóbulos auriculares e tronco, associadas a sinusite de repetição, rouquidão e perda ponderal de 25 kg. A baciloscopia de raspado intradérmico mostrou índice baciloscópico de 5 e o exame histopatologico foi compatível com fenômeno de Lúcio. O tratamento instituído foi exclusivamente com poliquimioterapia multibacilar (PQT-MB), havendo melhora importante do quadro cutâneo após um mês de tratamento. Os autores apresentam um caso exuberante de fenômeno de Lúcio que obteve excelente resposta ao tratamento somente com PQT-MB.

Indeterminate leprosy and lepromatous index case: four cases in the same family / Hanseniase indeterminada e caso indice virchowiano: 4 casos na mesma familia

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, which primarily affects the skin and peripheral nerves. Brazil remains as the country with the second largest number of cases in the world. We report the case of three patients diagnosed with indeterminate leprosy in the same family. Two patients were HIV positive. An active search led to the discovery of the index case. It was crucial to persist in the search of the index case. This report shows how important it is to teach physicians and the general population about the signs and symptoms of leprosy. Early diagnosis and treatment are necessary to prevent sequelae and to eliminate the disease as a public health problem.

A hanseníase é uma doença infecciosa crônica causada pelo Mycobacterium leprae, que afeta principalmente a pele e nervos periféricos. O Brasil continua sendo o segundo país do mundo com maior número de casos. Aqui relatamos três pacientes diagnosticados com hanseníase indeterminada, pertencentes à mesma família, cuja busca ativa levou à descoberta do caso índice. Dois pacientes eram HIV positivos. Foi de imensa importância a insistência na procura pelo caso índice. Este relato ilustra a importância do ensino sobre sinais e sintomas de hanseníase na formação médica e à população. O pronto reconhecimento e tratamento são necessários para prevenir sequelas e eliminar a doença como problema de saúde pública.

Non Hodgkin T cell lymphoma: an atypical clinical presentation / Linfoma não Hodgkin de células T citotóxico: uma apresentação clínica atípica

Cytotoxic lymphomas comprise a spectrum of peripheral T-cell lymphomas that can have a initial or late cutaneous presentation. We describe a 46-year-old man from Cape Verde, with a dermatosis involving his face and trunk, consisting of monomorphic papules with a smooth surface and both motor and sensory polyneuropathy.The hypothesis of leprosy was supported by the clinical and initial hystopathological findings and the patient was referred to our hospital with suspected Hansen's disease. In the new skin and lymph node biopsies a lymphocyte population was identified whose immunohystochemistry study allowed the diagnosis of T-cell lymphoma with expression of cytotoxic markers. The patient was started on chemotherapy with initial remission of the skin lesions but, subsequently, progression of systemic disease.

Os linfomas citotóxicos compreendem um espectro de linfomas de células T periféricos e linfomas Natural Killer que podem ter expressão cutânea primária ou secundária. Descrevemos o caso de um homem com 46 anos de idade, natural de Cabo Verde,com dermatose envolvendo a face e tronco constituída por pápulas monomorfas superfície lisa e polineuropatia sensitivo motora.A hipótese de Hanseníase foi colocada suportada por achados histopatológicos sugestivos sendo o doente referenciado à consulta de Doença de Hansen do nosso hospital. Em biopsia de pele e de gânglio identificou-se proliferação linfocitária cujo estudo imunohistoquímico permitiu o diagnóstico de linfoma T com expressão de marcadores citotóxicos. Iniciou quimioterapia verificando-se inicialmente remissão parcial das lesões cutâneas mas posteriormente a progressão da doença sistémica.

Mast Cells in Non-neoplastic Skin Lesions.

Background: Mast cells are found in tissues throughout body, particularly in proximity to surfaces that interface with the external environment. They are found at all levels of dermis, where they are grouped around blood vessels, nerves and appendages. Increase in mast cell number have been reported in various cutaneous diseases. Aims: We aim to identify and analyse the morphological aspects of distribution of mast cells and their value in non-neoplastic skin lesions. Material and Methods: Ninety seven biopsies of various skin lesions were studied for mast cells by Toluidine Blue at pH 3.9. Result: All the skin lesions showed significant increase in mast cell number. Psoriasis showed maximum number of mast cells (127/mm2) followed by Lichen planus (108/mm2). Among the cases of cutaneous bacterial infections, mast cells were maximum in leprosy cases (99/mm2) and lowest in cutaneous tuberculosis. A comparative decrease in number of mast cells was seen from lepromatous leprosy to tuberculoid leprosy. A wide range of morphological alterations of mast cells were observed in these skin lesions. Psoriasis and bullous pemphigoid cases showed maximum spindle cell morphology. Conclusion: Tissue mast cell number, distribution and morphology vary in different skin lesions depending on the degree of mast cell activation. This may have a bearing on diagnosis and management.

Case report of a histoid leprosy patient

Histoid leprosy is one of the rare kinds of lepromatous leprosy with specific clinical pathologic manifestations. It is most commonly observed in patients who have been under long-term single drug therapy with dapsone. Clinical manifestations mostly consist of red papules and nodules and pathologic examination there are spindle like histiocytes. Following acid-fast staining, abundant bacilli can be seen. Significance of these patients is due to their rarity, atypicality of dermal lesions, failure in early diagnosis, and high bacillus load which can be a barrier in the eradication of the disease and act as a potential source of infection in areas where the disease has been eradicated. Our case showed up with plentiful dermal papules and nodules from two years ago which caused no discomfort. He had a history of leprosy 25 years ago and had been treated only with dapsone for 3 years. Therefore, the diagnosis of histoid type lepromatous leprosy was made and confirmed based on clinical and histopathological findings.

Erythema multiforme in leprosy

The clinical course of leprosy is often interrupted by reactions, which are acute inflammatory episodes that can be classified as type I or type II. Type II reactions can present as cutaneous lesions that resemble erythema multiforme (EM). EM is classically associated with drug allergies or pre-existing viral infections. However, the differential diagnostic criteria of the diverse causative agents remain controversial. The aim of this study was to determine both the clinical relevance and the morphological and immunohistochemical characteristics of the EM-like lesions during the course of type II leprosy reactions. Twenty-seven skin biopsies were taken from typical EM-like lesions of multibacillary patients and reviewed; their histological features were correlated to their clinical aspects. Then, a computer-assisted morphometric analysis was performed to measure the extent of angiogenesis during these acute episodes. The histopathological and immunohistochemical analysis of the EM lesions revealed that they shared the same features that have been previously described for ENL, including immunopositivity in the identical cell-mediated immune markers. Our results point to leprosy as the cause of the EM-like lesions in our patients. Therefore, leprosy should be considered in the differential diagnosis of EM.

Deciphering the contribution of lipid droplets in leprosy: multifunctional organelles with roles in Mycobacterium leprae pathogenesis

Leprosy is an infectious disease caused by Mycobacterium leprae that affects the skin and nerves, presenting a singular clinical picture. Across the leprosy spectrum, lepromatous leprosy (LL) exhibits a classical hallmark: the presence of a collection of M. leprae-infected foamy macrophages/Schwann cells characterised by their high lipid content. The significance of this foamy aspect in mycobacterial infections has garnered renewed attention in leprosy due to the recent observation that the foamy aspect represents cells enriched in lipid droplets (LD) (also known as lipid bodies). Here, we discuss the contemporary view of LD as highly regulated organelles with key functions in M. leprae persistence in the LL end of the spectrum. The modern methods of studying this ancient disease have contributed to recent findings that describe M. leprae-triggered LD biogenesis and recruitment as effective mycobacterial intracellular strategies for acquiring lipids, sheltering and/or dampening the immune response and favouring bacterial survival, likely representing a fundamental aspect of M. leprae pathogenesis. The multifaceted functions attributed to the LD in leprosy may contribute to the development of new strategies for adjunctive anti-leprosy therapies.

La lepra y el testículo / Leprosy and the testis

Introducción. La afección testicular es frecuente en la lepra lepromatosa, daño que se incrementa cuando cursa con eritema nudoso leproso. Objetivo. Presentar un paciente con lepra lepromatosa y eritema nudoso leproso con grave compromiso testicular. Materiales y métodos. Se estudió un hombre de 28 años con lepra lepromatosa desde los 22, que durante la poliquimioterapia para la lepra presentó eritema nudoso leproso crónico que afectó ambos testículos y no respondió al manejo convencional. El dolor persistente obligó a practicar orquidectomía izquierda. Resultados. Este testículo presentaba atrofia tubular y fibrosis notorias, conglomerados de macrófagos espumosos, sin bacilos, hiperplasia focal de células de Leydig, endarteritis y arteritis linfocitaria y granulomatosa de vasos pequeños y medianos; estos cambios también estaban presentes en el epidídimo. Un estudio llevado a cabo dos años después de terminar su tratamiento y de la orquidectomía izquierda, demostró azoospermia, testosterona total normal, testosterona libre discretamente disminuida y hormonas lutropina (luteinizante) y folitropina (estimulante del folículo) elevadas. No había disminución de la libido ni de su actividad sexual. Se revisaron los conceptos generales sobre el eritema nudoso leproso y las alteraciones que la lepra produce en el testículo. Conclusión. La lepra lepromatosa puede conducir a hipogonadismo. Los programas de lepra deben contemplar esta complicación para corregir y evitar sus secuelas.

Introduction. Damage of testicles is frequent in lepromatous leprosy and worsened by the presence of erythema nodosum leprosum. Objective. A patient is presented who developed lepromatous leprosy and erythema nodosum leprosum with major testicular compromise. Material and methods. The 28-year-old male patient had lepromatous leprosy since age 22. During a polychemotherapy treatment for the lepromatous leprosy, he presented chronic erythema nodosum leprosum that affected both testicles; he did not respond to the conventional treatment. A left orchidectomy was performed to treat the persistent pain. Results. The extracted testis evidenced the following: tubular atrophy, extensive fibrosis, cumulus of foamy macrophages without rods, focal Leydig cell hyperplasia, linfocitary and granulomatous arteritis and endarteritis of small and medium size vessels. These changes were also observed in the epididymis. Two years after the polychemoterapy and the orchidectomy, the patient exhibited azoospermy, normal total testosterone, slightly diminished free testosterone and elevated levels of luteinizing hormone and follicle-stimulating hormone. No loss of libido or sexual activity was reported. General concepts of erythema nodosum leprosum were reviewed, as well as the pathologic changes produced by leprosy in the testis. Conclusion. Lepromatous leprosy may lead to hypogonadism. This condition is recommended for inclusion in leprosy diagnostic programs in order to detect and treat the consequences of the possible hypogonadism.