Primer informe clínico de Balamuthia mandrillaris en el distrito de Camaná, Arequipa, Perú / First case report of the Balamuthia mandrillaris in the Camaná district of Arequipa, Peru

Resumen Las infecciones por amebas de vida libre causan compromiso cutáneo y neurológico. Estas afecciones presentan una baja frecuencia pero una alta letalidad, mayor al 98%. Gene ralmente el cuadro clínico es inespecífico y los exámenes de laboratorio no son de gran ayuda, por lo que representa un reto diagnóstico y terapéutico. En este informe presentamos el caso de un paciente de 21 años que fue hospitalizado por un síndrome convulsivo con tumoraciones cerebrales, además de una lesión cutánea en el muslo derecho. El análisis histopatológico, PCR y el cuadro clínico permitieron el diagnóstico de encefalitis amebiana granulomatosa. A pesar del tratamiento, el paciente tuvo un desenlace fatal.

Abstract Infections by free-living amoebas cause cutaneous and neurological compromise. These conditions have a low frequency, but a high lethality more than 98%. Generally, the clinical picture is nonspecific; the laboratory tests dont help, so it represents a diagnostic and therapeutic challenge. In this report, we present the case of a 21-year-old patient, who was hospitalized for a convulsive syndrome with brain tumors, in addition to a cutaneous lesion on the right thigh. Histopathological analysis, PCR and the clinical picture allowed the diagnosis of granulomatous amebic encephalitis. Despite the treatment, the patient had a fatal outcome.

Amiloidosis sistémica con compromiso cutáneo asociada a mieloma múltiple: presentación de un caso / Systemic amyloidosis with cutaneous involvement associated with multiple myeloma: a case report

Las amiloidosis son un grupo de enfermedades que se caracterizan por el depósito extracelular de material amiloide en órganos y tejidos, afectando su función normal. Se clasifican según la proteína fibrilar involucrada y, de acuerdo a su distribución, en localizada o sistémica.Presentamos una paciente con amiloidosis sistémica asociada a mieloma múltiple con compromiso esofágico y cutáneo. El interés de la presentación radica en la importancia del reconocimiento de las lesiones cutáneas características, para realizar diagnóstico precoz de esta patología.

Amyloidosis is a group of diseases characterized by the extracellular deposition of amyloid in organs and tissues affecting their normal function. They are classified by the fibrillar protein involved and, according distribution, in localized or systemic. A female patient with systemic amyloidosis associated with multiple myeloma and esophageal and cutaneous involvement is reported. Interest of this presentation is the importance in recognizing the characteristic skin lesions to make an early diagnosis of this disease.

Cutaneous Involvement of T-cell Lymphoblastic Lymphoma / 대한피부과학회지

Lymphoblastic lymphoma is classified as non-Hodgkin's lymphoma with high tendency of rapid progression to acute leukemia. Cutaneous involvement of this condition is rare. The clinical feature of skin lesions usually present single or multiple papular or nodular lesions preferentially located on the head and neck. Herein, we report a case of an 18-year-old man presenting skin involvement of T cell lymphoblastic lymphoma. The lesions manifested as asymptomatic, multiple, round, slightly pinkish nodules on the scalp and trunk. Histological examination showed dense, diffuse infiltration of medium-sized lymphoid cells throughout the entire dermis. The neoplastic cells had scant cytoplasm and nuclei characterized as round or slightly irregular with expression of CD3, CD5, CD45, TdT and Ki-67.

A Case of Kikuchi's Disease Presenting with Unique Facial Involvement

Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. When KD is involved in a skin lesion, it presents with various shapes, but rarely manifests with symmetrically distributed, erythematous, firm nodules only on the face. In this report, we describe a patient with KD and unique skin manifestations.

Púrpura de Henoch-Schõnlein com acometimento incomum de face / Unusual face involvement in Henoch-Schõnlein purpura

A Púrpura de Henoch-Shõnlein é uma vasculite de causa idiopática, que se caracteriza pelo depósito predominante de IgA na parede dos pequenos vasos, envolvendo tipicamente pele, intestino, articulações e glomérulo renal. O acometimento cutâneo ocorre principalmente em membros inferiores e região glútea, sendo raramente encontrado em face e membros superiores. Relatamos o caso de uma paciente do sexo feminino, com seis anos de idade, que apresentava lesões púrpuricas em região auricular, periorbital, malar, mentoniana e membros superiores, além de artralgia e volvo intestinal. Após tratamento cirúrgico e pulsoterapia com glicocorticoide, houve regressão dos sintomas, sem maiores complicações.

Henoch-Schõnlein Purpura is an idiopathic vasculitis characterized by deposits of immunoglobulin, mainly IgA, on the walls of small vessels, typically involving the skin, gut, joints, and renal glomeruli. Cutaneous involvement affects specially the lower limbs and buttocks, and it is seldom found on the face and upper limbs. We report the case of a 6-year old girl with purpuric lesions over the auricular, periorbital, malar, and mentonian regions and the upper limbs, arthralgia, and intestinal torsion. After surgical treatment and pulse therapy with glucocorticoids, her symptoms subsided without further complications.

A Case of Cutaneous Involvement of T-cell Lymphoblastic Lymphoma / 대한피부과학회지

Lymphoblastic lymphoma is a malignant neoplasm of precursor lymphocytes with a B- or T cell phenotype. T cell lymphoblastic lymphoma is a clinically aggressive disease with frequent involvement of extranodal sites, but the involvement of the skin is rare. The clinical appearance of skin lesions usually includes single or multiple papular or nodular lesions preferentially located on the head and neck. Herein, we report the case of a 31-year-old man presenting with skin involvement of T cell lymphoblastic lymphoma. The lesions manifested as asymptomatic, multiple, round, reddish brown macules, and patches on both extremities. Histology examination showed dense, diffuse infiltration of medium sized lymphoid cells into the entire dermis. The tumor cells had irregular nuclei, finely dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and expressed TdT, CD3 and CD5.

Two Cases of Cutaneous Rosai-Dorfman Disease / 대한피부과학회지

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, idiopathic histiocytic proliferative disorder affecting lymph nodes as well as extranodal sites. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. Although cutaneous involvement in RDD is common, a purely cutaneous disease is very rare. Histologic findings show characteristic large, pale, histiocytic cells exhibiting cytophagocytosis (emperipolesis). Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but negative for CD1a. The etiology is unknown, although it is thought to be a reactive disorder rather than neoplastic. We report two cases of Rosai-Dorfman disease showing involvement limited to the skin.

A Case of Primary Nasal CD56+ NK/T cell Lymphoma with Cutaneous Involvement / 대한피부과학회지

Recently a distinct subtype of lymphoma was reported, the natural killer(NK)/T-cell lymphoma, which can involve the skin in a primary or secondary event. NK/T-cell lymphoma is characterized by the expression of the NK-cell antigen CD56. CD56+ lymphoma are further subdivided into nasal and non-nasal NK/T-cell lymphoma. We report a case of primary nasal NK/T-cell lymphoma with secondary cutaneous involvement.

A Case of Kikuchi's Disease (Histiocytic Necrotizing Lymphadenitis) with Cutaneous Involvement Presenting As Aseptic Meningitis / 대한피부과학회지

Histiocytic necrotizing lymphadenitis, or Kikuchi's disease, is a benign self-limiting febrile illness usually affecting young women, which is manifested clinically by fever and cervical lymphadenopathy. Skin involvement in Kikuchi's disease is not frequent and Kikushi's disease presenting as aseptic meningitis is rare, with only three previous reports in English literature to date. We present a 16-year-old girl with cervical lymphadenopathy and fever who was diagnosed with Kikuchi's disease, suffering from multiple transient erythematous maculopapular skin lesions and presenting as aseptic meningitis.

Primary Nasal CD56+ NK/T-cell Lymphoma with Cutaneous Involvement / 대한피부과학회지

BACKGROUNDS: There are recently increasing reported cases of patients with malignant lymphoma with unique characteristics, designated nasal-type T/natural killer (NK) cell lymphoma (NKTL), which usually expresses the NK cell marker and shows frequent extra-nodal involvement including skin and poor prognosis. The CD56+ NKTL can be divided into nasal and non-nasal types. Nasal and non-nasal (nasal type) CD56+ NKTL share the same pathologic features such as angiocentricity and necrosis. OBJECTIVE: We have studied clinicopathologic features of 5 cases of nasal CD56+ NKTL with cutaneous involvement to further elucidate the behaviour of nasal CD56+ NKTL. RESULTS: Clinically, the cutaneous lesions were variable. Four of the five cases died with rapidly progressive disease within 25 months. Histologically, variable sized pleomorphic lymphoid cells with or without prominent angiodestruction were observed. Severe necrosis was consistent features. The results of immunophenotypes(both CD56+ and CD3+) and TCR gene rearrangement study showed these 5 cases are of NK cell origin(4 cases) or NK-like T-cell origin (1 case). A firm association with Epstein-Barr virus (EBV) infection was confirmed in our study. CONCLUSION: Although the prognosis of nasal CD56+ NKTL was not so bad, higher stage of nasal CD56+ NKTL with cutaneous metastasis seemed to be very fatal. The term NKTL has to be further specified according to immunophenotypes such as CD56+ or true T-cell, NK-like T cell lymphoma since biologic behaviour of each subgroup can be different.