Lupus miliaris disseminatus faciei: when a rare skin disease involves the eyelid / Lupus miliaris disseminatus faciei: quando uma rara dermatose acomete a pálpebra

ABSTRACT The authors present a case of lupus miliaris disseminatus faciei , a rare skin disease of unknown etiology, which may cause unaesthetic scarring due to its difficult treatment. The histopathological examination of epithelioid granulomas with caseating necrosis, together with the clinical features, are important for diagnosis and early treatment with better results. Despite difficult and unsatisfactory treatment, there are ongoing studies on therapy to improve aesthetic and social impairment. This case report describes an initial misdiagnosis delaying appropriate treatment, and highlights the value of physical examination and clinical judgment for another pathological examination, whenever necessary, aiming at better treatment outcomes in daily practice.

RESUMO Os autores apresentam um caso de lupus miliaris disseminatus faciei , uma dermatose rara, de etiologia desconhecida, que pode deixar cicatrizes não estéticas, pela dificuldade de tratamento. O exame histopatológico de granulomas compostos por células epitelioides, com necrose caseosa, e as características clínicas, são importantes para o diagnóstico e tratamento precoce, com melhores resultados. Apesar do tratamento difícil e insatisfatório, há estudos em andamento sobre terapias para melhorar o comprometimento estético e social. Este relato de caso descreve um diagnóstico inicial errôneo, que atrasou o tratamento adequado, e destaca o valor do exame físico e raciocínio clínico para solicitar outro exame anatomopatológico, quando necessário, de forma a obter melhores desfechos com o tratamento, na prática diária.

Primary pituitary tuberculosis

Tuberculosis is an infectious disease that involves any organ. However, the primary pituitary tuberculosis is an extremely rare disease. Intracranial tuberculomas account for 0.15-5% of intracranial space-occupying lesions, of which, pituitary as the primary site is unusual, and easily misdiagnosed as pituitary adenoma. In this setting, the late diagnosis can result in permanent endocrine dysfunction. We report the case of a 50-year-old woman who presented to the neurosurgery outpatient department with complaints of progressively increasing headache and diminished vision over the last year. On the clinical examination, the patient was conscious and oriented. The routine hematological and biochemical workup showed an increased erythrocyte sedimentation rate (ESR) and increased prolactin levels. The radiological working diagnosis was consistent with pituitary macroadenoma. No other radiological and/or clinical clue that could elicit the suspicion of pulmonary or extrapulmonary lesions of tuberculosis was found. The transsphenoidal endonasal tumor excision was done. The histopathology showed numerous epithelioid cell granulomas, Langhans giant cells along with scant necrosis. Ziehl Neelsen staining demonstrated acid-fast bacilli, and the final diagnosis of pituitary tuberculoma was made. We report this rare case of pituitary lesion that may be included in the differential diagnosis of sellar lesions to avoid unnecessary surgical interventions, especially in regions where the disease is endemic.

Giant cell reparative granuloma of the nasal cavity: a rare site of presentation with review of literature

Giant cell reparative granuloma (GCRG) was first described in the 1950s. It is an uncommon and benign reactive tumor that is believed to occur after trauma or inflammation. It most commonly occurs in the maxilla and mandible and rarely affects the nasal cavity. It is often seen in children and during the second to third decades of life, predominantly seen among females. Histopathologically, GCRG shows many osteoclast-like multinucleated giant cells scattered in a background of mononuclear stromal cells and spindle-shaped fibroblasts also associated with areas of hemorrhage. The distinction between GCRG and giant cell tumors (GCT)is crucial since both have a similar clinical and histological presentation, but both have different management. GCTs have malignant potential, may metastasize, and have a high rate of recurrence. Surgical excision is the mainstay therapy of GCRG to ensure a low rate of recurrence. Here we discuss two cases GCRG, both presenting as nasal mass.

A case of facial atrophic sarcoidosis in an adolescent, successfully treated with the combination of prednisone and hydroxychloroquine

Abstract Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Cutaneous involvement occurs in up to 30% of patients and skin findings are often the initial presenting symptom. The facial atrophic form of sarcoidosis without associated ulceration in adolescents has rarely been described in the literature. We report a case of 13-year-old male patient with a facial atrophic sarcoidosis who was successfully treated with the combination of prednisone and hydroxychloroquine.

Diagnóstico diferencial en pacientes con granulomas en biopsias de pulmón, pleura y/o ganglios mediastínicos del Instituto Nacional del Tórax / Differential diagnosis in patients with granulomas in the biopsy of the lung, pleura and/or mediastinal nodes

Las enfermedades granulomatosas incluyen una amplia gama de enfermedades. Sin embargo, en la práctica clínica, muchos casos de enfermedad granulomatosa permanecen sin etiología después del examen histológico. Nuestro objetivo fue determinar, a partir de las biopsias de pulmón, pleura y ganglios linfáticos mediastínicos, en los que se informaron granulomas, las características clínicas y los diagnósticos de estos pacientes. Así también la mortalidad a un año de seguimiento. Metodología: Analizamos retrospectivamente biopsias de pulmón, pleura y/o ganglios linfáticos mediastínicos con granulomas de 75 pacientes del Instituto Nacional del Tórax (2012-2016), sus características clínicas y de laboratorio. La información se obtuvo de los registros médicos. Los datos de mortalidad se obtuvieron del registro civil. Resultados: Se determinó una etiología en todos los casos, excepto en 3 (4%). Los diagnósticos más frecuentes fueron tuberculosis (n = 37; 49%) y sarcoidosis (n = 18; 24%). Otras causas fueron silicosis (5,3%), vasculitis (4%) y neumonitis por hipersensibilidad (2,7%). Los pacientes con tuberculosis (TB) tenían parámetros inflamatorios más altos, como velocidad de eritrosedimentación y proteína C reactiva. Además, sólo se encontraron granulomas con necrosis caseosa en pacientes con tuberculosis. En cambio, los pacientes con sarcoidosis tenían lesiones cutáneas y una mayor frecuencia de linfadenopatías. Cuatro (5.3%) pacientes fallecieron a un año de seguimiento: dos debido a neumonía, uno por hipersensibilidad crónica y uno por TB. Conclusión: La tuberculosis y la sarcoidosis fueron las causas más frecuentes de granulomas respiratorios en este estudio retrospectivo. Se logró determinar una etiología en el 96% de los casos, considerando variables clínicas, de laboratorio e histopatológicas para un diagnóstico diferencial correcto.

Granulomatous diseases comprise a wide range of pathologies. However, in clinical practice, many pulmonary granulomas remain without etiology after the histologic examination. Our aim was to determine from the biopsies of the lung, pleura and mediastinal lymph nodes in which granulomas were reported, the clinical characteristics and diagnoses of the patients. Methodology: We analyzed retrospectively biopsies of the lung, pleura and mediastinal lymph nodes with granulomas from 75 patients handled at our institution (2012-2016), as well as their clinical and laboratory data. The information was obtained from medical records. A one-year mortality date was obtained from the civil registry. Main results: A cause was determined in all the cases, except in three of them (4%). The most frequent diagnoses were tuberculosis (n =37; 49%) and sarcoidosis (n =18; 24%). Other causes were silicosis (5.3%), vasculitis (4%) and hypersensitivity pneumonitis (2.7%). Patients with tuberculosis (TB) had higher inflammatory parameters such as erythrocyte sedimentation rate and C-reactive protein. Besides granulomas with caseous necrosis were only found in TB patients. Instead, patients with sarcoidosis had skin lesions and a higher frequency of lymphadenopathy. Four patients (5.3%) died in a one-year of follow-up: two of them because of pneumonia and the other two patients because of chronic hypersensitivity and TB respectively. Conclusion: Tuberculosis and sarcoidosis were the most common causes of respiratory granulomas in this retrospective study. A specific cause was determined in 96% of cases, considering clinical, laboratory and histopathological variables to do a right differential diagnosis.

Schistosoma mansoni granulomas in the skeletal striated muscles in the murine model of neuroschistosomiasis: histological findings

Schistosomiasis mansoni presents many clinical manifestations during migration of schistosomes in their hosts, including diarrhea, hepatomegaly, splenomegaly, liver abscesses, skinlesions, brain tumors and myeloradiculopathy. No lesions have been reported in skeletal striated muscles due to schistosomiasis mansoni in the literature. This short communication reports the histopathological findings on skeletal musculature in a murine model of neuroeschistosomiasis mansoni. Lesions were found in the tongue, masseter muscle, buccinator muscle, digastric muscle and temporalis muscle. Worm recovery was carried out to confirm the infection. We describe here, for the first time in the literature, injuries in the skeletal musculature due to Schistosoma mansoni nfection.

Granulomatous pigmented purpuric dermatosis: report of a Latin-American case with blaschkoid distribution

Abstract Granulomatous pigmented purpuric dermatosis clinically manifests as hyperpigmented maculae and petechiae, predominantly on the lower extremities. Histopathologically, it is characterized by a lymphocytic infiltrate in the upper dermis, extravasated erythrocytes, and hemosiderin deposits. There is an infrequent variant called granulomatous pigmented purpuric dermatosis, which histologically is characterized by the presence of non-necrotizing granulomas associated with the classic findings of other pigmented purpuric dermatoses. It more frequently affects middle-aged women of Asian origin, and predominantly on the lower extremities. The authors present the case of a female patient with granulomatous pigmented purpuric dermatosis on the lower extremities with blaschkoid distribution.

Obstrucción duodenal y hemorragia digestiva alta como presentación inicial de una tuberculosis duodenal aislada / Duodenal obstruction and upper gastrointestinal bleeding as the initial presentation of an isolated duodenal tuberculosis

Resumen La tuberculosis duodenal primaria es muy infrecuente, incluso en regiones endémicas. El diagnóstico plantea un gran reto, y requiere un alto índice de sospecha, apoyado en estudios de imágenes, microbiología, e histopatología obtenida por biopsia endoscópica o quirúrgica. Presentamos el caso de un varón de 31 años, sin infección por VIH ni antecedente de tuberculosis, que debutó con una obstrucción duodenal. Posterior a una laparatomía exploradora presentó una estenosis duodenal y una hemorragia digestiva alta. Luego de varias biopsias no concluyentes, sólo la última, realizada con la técnica "biopsia sobre biopsia", demostró la presencia de granulomas con bacilos ácido-alcohol resistentes. El diagnóstico de tuberculosis fue confirmado por reacción de polimerasa en cadena de tejido duodenal. No se evidenció compromiso de otros órganos. La respuesta terapéutica fue excelente.

Primary duodenal tuberculosis is rare, even in endemic regions. The diagnosis poses a major challenge and requires a high index of suspicion, supported by imaging studies, microbiology, and histopatology obtained by endoscopic or surgical biopsy. We report the case of a 31-year-old man, without HIV infection or any previous history of tuberculosis, who presented with duodenal obstruction. After exploratory laparotomy, he presented a duodenal stenosis and upper gastrointestinal bleeding. A total of four diagnostic procedures (one laparotomy and three endoscopies) were performed, all of which included biopsies. Only the last endoscopy made with the technique "biopsy upon biopsy" showed the presence of acid fast bacilli and granulomas. The diagnosis of tuberculosis was confirmed by polymerase chain reaction in duodenal tissue. There was no evidence of involvement of other organs by tuberculosis. The patient had an excellent therapeutic response.

In-vivo assessment of a case of cutaneous sarcoidosis using reflectance confocal microscopy

Abstract: Reflectance confocal microscopy (RCM) is a noninvasive imaging technique that allows visualization of the epidermis and papillary dermis with cellular-level resolution. Granulomatous reactions such as sarcoidosis could be assessed using RCM. The identification of bright beaded-like structures that could correspond to reticulin fibers overlying granulomas, in association with dermoscopy, may be a very useful approach in the diagnosis of sarcoidosis and for the differentiation of this granulomatous entity with superficial cutaneous metastasis.

Reporte de caso de granuloma facial, una vasculitis cutánea infrecuente / A case report of granuloma faciale, an uncommon cutaneous vasculitis

El granuloma facial es una dermatosis benigna poco frecuente de etiología desconocida, generalmente asintomática, caracterizada por inflamación crónica y localizada habitualmente en zonas fotoexpuestas de la cara, con un patrón histológico característico. Si bien la respuesta al tratamiento es variable, existen múltiples opciones terapéuticas que han reportado ser efectivas en algunos pacientes. Entre estas alternativas se incluyen tratamientos sistémicos con corticoides y dapsona o tratamientos intralesionales con corticoides, crioterapia e inhibidores de calcineurina tópicos. Describimos el caso de un paciente adulto con una placa eritemato violácea asintomática en mejilla derecha, de crecimiento lentamente progresivo de dos años de evolución, clínica e histopatológicamente compatible con granuloma facial y respuesta favorable a corticoides intralesionales.

Granuloma faciale is an uncommon benign dermatosis, with unknown etiology, usually asymptomatic, characterized by chronic inflammation localized in sun-exposed areas of the face with a characteristic histological pattern. Although response to treatment is variable, there are multiple therapeutic alternatives that have been reported to be effective in some patients, including systemic treatments with steroids and dapsone or also topical treatments like intralesional corticosteroid, cryotherapy and calcineurin inhibitors, such as tacrolimus. We present the case of an adult patient with an asymptomatic erythematous-violet plaque on the right cheek, with progressive slow growth over two years, clinically and histologically and pathologically compatible with a facial granuloma. The patient responded well to intralesional corticosteroids.

Pólipo y/o granuloma aural en niños. Estudio prospectivo en 75 casos / Aural polyp and/or granuloma in children. A prospective study of 75 cases

En otorrinolaringología (ORL) infantil es habitual el hallazgo de pólipos y granulomas de oído en niños que consultan especialmente por otorrea de evolución prolongada. El pólipo y/o granuloma aural es una masa de carácter inflamatorio, que ocupa parte de la luz del conducto auditivo externo, generalmente pediculado, de aspecto congestivo, a veces friable y fácilmente sangrante, cuyo origen generalmente es a nivel de la mucosa del oído medio. Con el objetivo de describir las características clínicas, otológicas, bacteriológicas e histopatológicas de los pólipos y granulomas de oído diagnosticados en un servicio de ORL pediátrico se realizó un estudio prospectivo, descriptivo, observacional y longitudinal. Se estudió a la población pediátrica con diagnóstico de pólipo y/o granuloma aural en su primera consulta en el servicio de ORL del Hospital de Pediatría "Prof. Dr. Juan P. Garrahan". Se incluyeron 75 pacientes en el estudio, evaluados consecutivamente desde el 02 de diciembre 2013 y hasta 30 enero del 2015, con una edad media: 93 meses (rango 2­180). Se realizó otomicroscopía y, en los casos de granulomas y pólipos accesibles, se realizó toma de muestra para estudio bacteriológico e histopatológico y evaluaciones audiológicas y radiológicas con tomografía computarizada (TC) en los casos necesarios. Se indicó el tratamiento médico o quirúrgico adecuado a cada patología. El motivo de consulta principal fue la otorrea como único síntoma en el 81,33% de los casos y, en menor porcentaje, asociada a otros síntomas. Tiempo medio de evolución de los síntomas: 13,5 meses (rango 1-96). No se pudo extraer material en el 20% de los pacientes. Se tomaron muestras para estudio de 60/75 granulomas óticos accesibles. El informe anatomo-patológico fue: granuloma o pólipo inflamatorio en el 50%, tejido epidermoide compatible con colesteatoma en el 41,7%, tuberculosis (TBC) en 3,3%, granuloma por cuerpo extraño en 1,7%, histiocitosis de células de Langerhans (HCL) en 3,3% muestras de pólipos. Se realizó estudio bacteriológico en 57/75 casos. Se desarrollaron gérmenes en 52/57 cultivos. El 32,7% (17/52) fueron cultivos polimicrobianos. Dos casos desarrollaron Mycobacterium tuberculosis. Se observó velamiento de caja, ático o mastoides con erosión ósea en el 46,2% (24/52) de los casos evaluados con TC. Diagnóstico final: colesteatoma 39 pacientes, OMA con pólipo de Scheibe o complicada con mastoiditis 16, OMC simple granulomatosa 13, TBC 2, HCL 2, otitis externa y celulitis en conducto auditivo externo 2 y granuloma a cuerpo extraño 1. Conclusiones: es importante obtener el diagnóstico histológico y microbiológico de los pólipos aurales en niños precozmente para excluir neoplasia u otras enfermedades granulomatosas específicas y evitar cirugías que pueden provocar secuelas al no estar indicadas en el tratamiento adecuado de ciertos tumores e infecciones (AU)

In pediatric otolaryngology (ENT) ear polyps and granulomas are a common finding in children who consult especially for prolonged otorrhea. The aural polyp and/or granuloma is an inflammatory mass occupying part of the lumen of the external auditory canal. It is usually pedunculated, congestive, sometimes friable, and may bleed easily. Its origin is usually at the level of the mucosa of the middle ear. With the aim to describe the clinical, otological, bacteriological, and histopathological features of ear polyps and granulomas diagnosed in a Department of pediatric ENT, a longitudinal, prospective, descriptive, observational study was conducted. Pediatric patients diagnosed with an aural polyp and/or granuloma at the first visit at the Department of ENT of Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" were studied. Seventy-five patients were included in the study, evaluated consecutively from December 2, 2013 to January 30, 2015; Mean age was 93 months (range 2 ­ 180). Otomicroscopy was performed and, in cases of accessible granulomas and polyps, a sample was taken for bacteriological and histopathological study. Audiological and radiological evaluations with computed tomography (CT scan) were performed when necessary. Appropriate medical or surgical treatment was indicated accordingly. The main reason for the consultation was otorrhea as the only symptom in 81.33% of cases and, in a lesser percentage, associated with other symptoms. Mean time from symptom onset to diagnosis: 13.5 months (range 1-96). No sample could be harvested in 20% of patients. Samples were taken for study of 60/75 accessible ear granulomas. Pathology report was: Inflammatory granuloma or polyp in 50%, epidermoid tissue compatible with cholesteatoma in 41.7%, tuberculosis (TBC) in 3.3%, granuloma due to a foreign body in 1.7%, and Langerhans cell histiocytosis (LHC) in 3.3% of the samples of polyps. Bacterial cultures, performed in 57/75 cases, were positive in 52/57. Polymicrobial microorganisms were found in 32.7% (17/52). Mycobacterium tuberculosis was found in two cases. Opacification of the antrum, attic, and mastoid cavities with bone erosion was observed in 46.2% (24/52) of the cases evaluated with CT. Final diagnosis: Cholesteatoma in 39 patients, OMA with a Scheibe polyp or complicated with mastoiditis in 16, simple granulomatous OMC in 13, TBC in 2, LHC in 2, external otitis and cellulitis in the external ear canal in 2, and granuloma due to a foreign body in 1. Conclusions: Histological and microbiological diagnosis of aural polyps in children should be obtained early to rule out neoplasia other granulomatous diseases to avoid surgery that may cause sequelae and is not the adequate management of certain tumors and infections (AU)

Lupus miliaris disseminatus faciei

Abstract: Lupus miliaris disseminatus faciei is a rare inflammatory dermatosis of unknown etiology that primarily affects young adults. Clinically, it is characterized by an asymptomatic papular eruption mainly involving the central face, typically on and around the eyelids. Characteristic histopathological features include dermal epithelioid cell granulomas with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells. Lupus miliaris disseminatus faciei has a spontaneously resolving course, yet can be cosmetically debilitating given the location and potential for scarring. Treatment is difficult and there is a lack of controlled studies. We report a new case of lupus miliaris disseminatus faciei successfully treated with minocycline and systemic steroids, and briefly discuss its nosology and therapeutic options.

Do you know this syndrome? Heerfordt-Waldenström syndrome

Abstract: Heerfordt-Waldenström syndrome is a rare subacute variant of sarcoidosis, characterized by enlargement of the parotid or salivary glands, facial nerve paralysis and anterior uveitis. Granulomas with a peripheral lymphocyte deficit are found in the anatomic pathology of affected organs. It is normally self-limiting, with cure achieved between 12 and 36 months, but some prolonged cases have been reported. Diagnosis of the syndrome is clinical, and treatment depends on the degree of systemic impairment. Oral corticosteroids represent the first line treatment option. The mortality rate ranges between 1 and 5% of cases.

Sarcoidal granulomatous reaction due to tattoos: report of two cases

Abstract Numerous infectious, inflammatory and neoplastic complications secondary to tattoo placement have been reported in the literature. Within inflammatory complications sarcoidal granulomatous reactions have been described. We report two cases, a 55-year-old woman with yellowish infiltrated plaques on bilateral ciliary region, 16 years after the placement of a permanent tattoo in the eyebrows, and a 20-year-old tattoo artist who developed orange papules on 3 of his tattoos. Histopathology in both cases confirmed diagnosis of sarcoidal granulomatous reaction due to tattoo pigment.

Granuloma faciale: clinical, morphological and immunohistochemical aspects in a series of 10 patients

Abstract Granuloma faciale is a chronic, benign, cutaneous vasculitis with well-established clinical and morphological patterns, but with an unknown etiology. This study describes clinical and pathologic aspects of patients diagnosed with granuloma faciale. The authors analyzed demographic, clinical, morphological and immunohistochemical data from patients with a final diagnosis of granuloma faciale, confirmed between 1998 and 2012. There was a proportional and mixed inflammatory infiltrate, Grenz zones were present in almost all the samples. Immunophenotyping confirmed a higher intensity of T lymphocytes than B lymphocytes in thirteen samples, with a predominance of T CD8 lymphocytes in 64% of cases, in contrast to the literature, which indicates that the major component is T CD4 lymphocytes. All cases were positive for IgG4 but the majority (12/14) had less than 25% of stained cells. The pathogenesis of granuloma faciale remains poorly understood, making studies of morphological and immunohistochemical characterization important to better understand it.

Evaluation of diaphragmatic mobility following intra-abdominal sub-diaphragmatic fixation of a double-layered mesh in rats

PURPOSE: To evaluate the tissue integration of a double-sided mesh after fixation in diaphragm and to study the diaphragmatic mobility by ultrasound. METHODS: Twenty male Wistar rats were used. The animals were assigned into two equal groups according to the day of euthanasia. The animals were anesthetized and a 1.5 x 1.5 cm of double-layer mesh was inserted between the diaphragm and the liver. For the evaluation of the diaphragm mobility a sonographic method was used. Measurements on specific breathing parameters were taking place. Pathological evaluation took place after the animal's euthanasia. RESULTS: Extra-hepatic granuloma was not differentiated overtime, (χ2=0.04, p>0.05). Neither fibrosis was significantly differentiated, (χ2=0.04, p>0.05). Intra-hepatic granuloma was significantly differentiated overtime, (χ2=10.21, p<0.05). Concerning Te parameter, means were significantly differentiated over time, F (3, 30) = 5.12, (p<0.01). Ttot parameter, it was differentiated over time, F (3, 8)=4.79, (p<0.05). IR parameter was also longitudinally differentiated, F (3, 30)=3.73, (p<0.05). CONCLUSION: The measurements suggest a transient malfunction of diaphragmatic mobility despite the fact that inflammatory reaction, fibrosis and extra-hepatic granuloma were not significantly differentiated with the passage of time.

Dermatosis purpúrica pigmentaria, variante granulomatosa, a propósito de un caso / Granulomatous variant of chronic pigmented purpuric dermatosis: case report

La dermatosis purpúrica pigmentaria (DPP) corresponde a un grupo de enfermedades caracterizadas clínicamente por petequias y coloración bronce de la piel. Generalmente se localiza en las extremidades inferiores y se describen varios tipos, dentro de los cuales se encuentra la variante granulomatosa. Se trata de una dermatopatía benigna, poco frecuente, de etiología aún no precisada y de difícil manejo. Se presenta el caso de una paciente de 48 años, sexo femenino, sin antecedentes mórbidos, que es derivada a dermatología desde reumatología, con el diagnóstico clínico de vasculitis, por la aparición de lesiones maculares y papulares eritematovioláceas, confluentes y pruriginosas en extremidades. Presenta todos sus exámenes normales. Se planteó, entonces, una acroangiodermatitis o liquen plano. Sin embargo, fue necesario llegar al estudio histopatológico para llegar al diagnóstico de dermatosis purpúrica pigmentaria, variante granulomatosa. Si bien se considera que la DPP granulomatosa es una entidad poco frecuente, la literatura sugiere que es una entidad subdiagnosticada. Se debe plantear como diagnóstico diferencial de lesiones cutáneas en extremidades inferiores, lo que determina la importancia de realizar el estudio histopatológico. De esta forma, la DPP granulomatosa será cada vez más común y se plantearán alternativas de tratamiento mejores a las actuales.

The pigmented purpuric dermatoses are a group of diseases characterized clinically by petechiae and bronze skin color, usually affecting the lower extremities. Several types are described and one of them is the granulomatous variant. This is a still rare and benign skin disease of unknown etiology. We present the case of a 48 years old female, previously healthy, derived from rheumatology to dermatology, with the clinical diagnosis of vasculitis due to the presence of confluent erythematous macular and papular lesions, mildly pruritic in lower extremities. Her tests were all in normal ranges. Therefore, an acroangiodermatitis or lichen planus were considered. However, it was necessary to perform a histopathology study to reach the final diagnosis of granulomatous pigmented purpuric dermatosis. It is considered that Granulomatous DPP is a rare entity, but the literature suggests that it is underdiagnosed. It should always be considered as a differential diagnosis of cutaneous lesions located in lower extremities, which determines the importance of histopathology. Thus, the granulomatous DPP will become more common and will be posible to propose better treatment alternatives.