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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.
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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.
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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.
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Nádegas , Lipomatose/patologia , Nevo/patologia , Biópsia , Feminino , Humanos , Lipomatose/cirurgia , Nevo/cirurgia , Adulto JovemRESUMO
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.
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Humanos , Feminino , Adulto Jovem , Nádegas , Lipomatose/patologia , Nevo/patologia , Biópsia , Lipomatose/cirurgia , Nevo/cirurgiaRESUMO
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.
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Doença de Crohn/complicações , Síndrome de Sweet/complicações , Adulto , Brasil , Doença de Crohn/patologia , Feminino , Humanos , Dermatopatias/patologia , Síndrome de Sweet/patologiaRESUMO
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.
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Humanos , Feminino , Adulto , Doença de Crohn/complicações , Síndrome de Sweet/complicações , Dermatopatias/patologia , Doença de Crohn/patologia , Síndrome de Sweet/patologiaRESUMO
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.
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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.
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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.
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Transplante de Rim/efeitos adversos , Paracoccidioides/isolamento & purificação , Paracoccidioidomicose/diagnóstico , Adulto , Evolução Fatal , Humanos , MasculinoRESUMO
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.