Case Series of Frontal Fibrosing Alopecia and Fibrosing Alopecia in a Pattern Distribution: Is There a Familial Correlation?

Introduction: Frontal Fibrosing Alopecia (FFA) and Fibrosing Alopecia in a Pattern Distribution (FAPD) are two distinct entities of cicatricial pattern hair loss that share histological features of perifollicular lichenoid inflammation associated with concentric fibrosis. Although the pathophysiology of FFA and FAPD are still unknown, recently published reports of familial cases indicate a possible genetic correlation. Case Presentation: We report 6 cases of familial alopecia composed of mothers and daughters: five with FFA and one with FAPD. We describe clinical, trichoscopy and histological correlation in cases of familial alopecia. Conclusions: These cases of mother and daughter disease association suggest a potential benefit of and role for performing systematic scalp examinations of all first-degree relatives of patients with pattern cicatricial alopecia.

Comparison of clinical and histologic findings in daylight photodynamic therapy for skin field cancerization: A randomized controlled four-arm study on physical methods-assisted delivery of methyl aminolevulinate.

BACKGROUND: Daylight photodynamic therapy (DL-PDT) has similar efficacy to conventional photodynamic therapy in treating actinic keratosis (AKs). Good clinical outcomes have been reported when associated with physical methods such as microneedles, but a comparison of different methods and histologic studies is lacking. OBJECTIVES: To evaluate clinical and histologic modifications induced by standard DL-PDT and compare with DL-PDT associated with physical methods in treating skin field cancerization of the face. METHODS: Forty patients with photodamaged skin and at least one AK lesion on the face were randomly distributed into four groups, ten patients in each (I: Standard DL-PDT; II: DL-PDT + microneedles; III: DL-PDT + CO2 laser; IV: DL-PDT + microdermabrasion) and underwent two DL-PDT sessions with methyl aminolevulinate cream and 2-hour daylight exposure. Skin biopsies were performed on all patients before and 3 months after. All fragments were stained using the hematoxylin-eosin, orcein, and picrosirius. RESULTS: All 40 patients completed the study. Group III had a higher AK-clearance after 1 (p = 0,002) and 3 (p = 0,034) months, but it was similar in every group at 6 months (p = 0,441). Group III and IV had better clinical global improvement on texture, pigmentation and fine lines. In the groups associated with physical methods, the improvement of the keratinocytes' atypia and solar elastosis were remarkable. Only group III showed a significant reduction in solar elastosis (p = 0.034) and increased collagen type I (p = 0.028) after treatment. CONCLUSION: DL-PDT-associated with physical methods had better clinical and histologic results. AK-clearance were significantly higher after 1 and 3 months with pretreatment-CO2 laser. Photorejuvenation were more evident with pretreatment-CO2 laser and microdermabrasion. Pretreatment-CO2 laser showed a significant reduction in solar elastosis and increase of collagen type 1. These results pointed to the pretreatment with laser as a potentially better option for skin field cancerization of the face.

Fibrosing Alopecia in a Pattern Distribution (FAPD) in 16 African-Descent and Hispanic Female Patients: A Challenging Diagnosis.

BACKGROUND: Fibrosing alopecia in a pattern distribution (FAPD) has only been described in Caucasian patients, and it is not clear whether it can develop in dark-skin ethnicities. MATERIALS AND METHODS: Sixteen Brazilian female patients, 12 of African descent and 4 Hispanic, with progressive scarring alopecia in a pattern distribution were analyzed. RESULTS: Dermatoscopic features showed perifollicular erythema and scaling (14/16), hair fiber diameter diversity (16/16), loss of follicular ostia (16/16), and follicular keratosis (3/16). Late stages showed a honeycomb pigmented network (12/16), a hyperpigmented perifollicular halo (12/16), and small white patches (12/16). Histopathological features showed lichenoid perifollicular infiltrate (14/16), follicular miniaturization (16/16), concentric fibrosis (16/16), perifollicular lymphocytic infiltrate (16/16), and vellus hair involvement (10/16). Premature desquamation of the inner root sheath was found in 11 patients. CONCLUSIONS: The concomitant findings of cicatricial pattern hair loss (with or without the recess of the front hair line), hair fiber diversity, perifollicular erythema and scaling, a whitish perifollicular halo, and histological findings of androgenetic alopecia, with vacuolar interface alteration of the upper portion of the follicular epithelium, are the main key features to suggest the diagnosis of FAPD. FAPD is a possible diagnosis in patients of color with cicatricial pattern hair loss. Clinical, dermatoscopic, and histopathological examination allow a proper final differential diagnosis.

Nevus lipomatosus cutaneous superficialis.

Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Nevus lipomatosus cutaneous superficialis

Abstract: Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Sweet's syndrome associated with Crohn's disease.

Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Sweet's syndrome associated with Crohn's disease

Abstract: Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus.

Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus / Escleredema de Buschke associado a diabetes mellitus tipo 2 de difícil controle

SUMMARY Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

RESUMO Escleredema de Buschke (EB) é doença rara do tecido conjuntivo caracterizada por endurecimento difuso e não depressível da pele, principalmente nas regiões cervical, deltoideanas e dorso. Enquadrado no grupo das mucinoses cutâneas, tem etiologia desconhecida e associação com: infecções bacterianas ou virais, alterações hematológicas e diabetes mellitus. O exame histopatológico evidencia derme espessada com fibras colágenas calibrosas separadas por fendas que correspondem a depósito de mucopolissacárides, observados por colorações especiais. Diversos tratamentos são relatados na literatura sem resultados bem definidos. Descrevemos caso de EB em paciente com diabetes mellitus tipo 2.

PARACOCCIDIOIDOMYCOSIS IN A RENAL TRANSPLANT RECIPIENT.

Paracoccidioidomycosis (PCM) is the most common endemic mycosis in Latin America. The etiological agents, which comprise two species, Paracoccidioides brasiliensis and P. lutzii, are thermodimorphic fungi that usually affect previously healthy adults. They primarily involve the lungs and then disseminate to other organs. Such mycosis is rare in organ transplant recipients; there have been only three cases reported in literature, until now. We report a case of PCM in a renal transplant recipient with an unusual dermatological presentation.

PARACOCCIDIOIDOMYCOSIS IN A RENAL TRANSPLANT RECIPIENT

Paracoccidioidomycosis (PCM) is the most common endemic mycosis in Latin America. The etiological agents, which comprise two species, Paracoccidioides brasiliensis and P. lutzii, are thermodimorphic fungi that usually affect previously healthy adults. They primarily involve the lungs and then disseminate to other organs. Such mycosis is rare in organ transplant recipients; there have been only three cases reported in literature, until now. We report a case of PCM in a renal transplant recipient with an unusual dermatological presentation.

Lesão palpebral de lúpus discoide como manifestação única da doença / Eyelid?s discoid lupus lesion as the only manifestation of the disease

Lúpus eritematoso discoide é uma desordem autoimune benigna da pele e representa a forma clínica mais comum de lúpus eritematoso cutâneo. A apresentação do lúpus eritematoso discoide com acometimento palpebral exclusivo, como neste caso, é extremamente rara, o que dificulta o diagnóstico precoce da doença. O desconhecimento dessa apresentação de lúpus e o consequente retardo do seu diagnóstico podem contribuir com a progressão da doença e resultar em cicatrizes inestéticas, o que pode prejudicar a função palpebral.

Comedo-like openings in melanoma.

We describe a case of melanoma with the presence of comedo-like openings at dermoscopy. These structures, typical of seborrheic keratosis, represent an uncommon finding in melanoma. We emphasize the importance of searching for specific dermoscopic criteria for melanocytic lesions during the examination of a pigmented lesion, despite possible observations of characteristic structures of non-melanocytic lesions, in order to increase the accuracy in the diagnosis of melanoma.

Comedo-like openings in melanoma

We describe a case of melanoma with the presence of comedo-like openings at dermoscopy. These structures, typical of seborrheic keratosis, represent an uncommon finding in melanoma. We emphasize the importance of searching for specific dermoscopic criteria for melanocytic lesions during the examination of a pigmented lesion, despite possible observations of characteristic structures of non-melanocytic lesions, in order to increase the accuracy in the diagnosis of melanoma.

Cutaneous leukocytoclastic vasculitis in the presence of methimazole therapy.

Treatment with antithyroid drugs may be accompanied by side effects. We present a patient diagnosed with Grave's Disease who developed extensive vasculitis in the lower limbs during methimazole use. After suspension of the methimazole and the introduction of prednisone in immunesupressor doses the cutaneous lesions started to involute.

Cutaneous leukocytoclastic vasculitis in the presence of methimazole therapy / Vasculite leucocitoclástica cutânea na vigência de terapêutica com metimazol

Treatment with antithyroid drugs may be accompanied by side effects. We present a patient diagnosed with Grave's Disease who developed extensive vasculitis in the lower limbs during methimazole use. After suspension of the methimazole and the introduction of prednisone in immunesupressor doses the cutaneous lesions started to involute.

A terapêutica com drogas antitireoidianas pode ser acompanhada de efeitos colaterais. Apresentamos um caso de paciente com diagnóstico de Doença de Graves que na vigência da terapêutica com metimazol desenvolveu um quadro extenso de vasculite de membros inferiores. A partir da suspensão do metimazol e da introdução de prednisona em doses imunossupressoras as lesões cutâneas entraram em involução.

Síndrome de Sweet associada à policitemia vera / Sweet’s syndrome associated withpolycythemiavera

A síndrome de Sweet pode estar associada a malignidades hematológicas, principalmente, à leucemia mieloide aguda, porém existem poucos relatos demonstrando a associação com a policitemia vera. Relata-se o caso de doente do sexo masculino, de 65 anos, portador de policitemia vera,que evoluiu com aparecimento de síndrome de Sweet na sua forma paraneoplásica.

Sweet’s syndrome may be associated with hematological malignancies, particularly with acute myelogenous leukemia, but there are few reports of its association with polycythemiavera. We describethe case of a 65-year-old male patient, diagnosed with polycythemiavera, which developedinto paraneoplastic Sweet's syndrome.

[Sweet's syndrome associated with polycythemia vera]. / Síndrome de Sweet associada à policitemia vera.

Sweet's syndrome may be associated with hematological malignancies, particularly with acute myelogenous leukemia, but there are few reports of its association with polycythemiavera. We describethe case of a 65-year-old male patient, diagnosed with polycythemiavera, which developedinto paraneoplastic Sweet's syndrome.

Alteraçöes pulmonares nas infecçöes por Pneumocytis carinii em pacientes aidéticos / Pulmonary changes caused by Pneumocytis carinii infections in patients with AIDS

Os autores discutem os aspectos clínicos, patológicos e padröes radiológicos das pneumonias por Pneumocystis carinii na SIDA. Foram estudados 68 casos de pacientes aidéticos com comprometimento pulmonar, encontrando-se lesäo por Pneumocystis carinii em 29 casos (42,7%). O padräo mais freqüentemente encontrado foi o de lesäo reticular bilateral em regiöes peri-hilares, por vezes com padräo acinar associado, sem linfonodomegalias ou comprometimento pleural

Padräo lesional da medula óssea na imunodeficiência adquirida (AIDS) / Lesional pattern of the bone marrow in acquired immunodeficiency syndrome

O estudo da medula óssea em 20 casos de necrópsia de pacientes portadores da Síndrome da Imunodeficiência Adquirida sugere que as alteraçöes medulares säo freqüentes e de valor diagnóstico no reconhecimento da Síndrome. É admitida a existência de um padräo lesional diagnóstico onde predominam os seguintes elementos: hipercelularidade, ausência ou reduçäo da granulopoiese, plasmocitose, histiocitose e displasia megacariocítica