Onychocytic matricoma

Abstract Onychocytic matricoma is a newly described tumor of the nail matrix. Clinically, it presents with localized thickening of the nail plate and melanonychia. Histologically, it represents a benign acanthoma of onychocytes. There are 8 cases reported in the literature. A 12-year-old girl presented with localized melanonychia and concurrent thickening of the nail plate restricted to the area of pigmentation affecting the right thumb, with no history of trauma or pain. We report a case of this rare tumor occurring in late childhood and provide a comprehensive review of its clinical presentation and differential diagnosis. Both clinicians and dermatopathologists should be aware of the presentation of onychocytic matricoma and include it in their scope of diagnosis of longitudinal nail bands.

Melanoma associated with congenital intermediate common blue nevus of the scalp - Case report

Abstract: Melanomas can arise either de novo (70%) or from pre-existing melanocytic lesions (30%). Of the latter, most cases arise at the dermoepidermal junction from small congenital or acquired non-blue nevi while only a few arise from blue nevi, notably the cellular subtype and less commonly the common (dendritic) type. Melanomas that arise from blue nevi usually occur on the scalp with greater frequency, as in the case described. Although previous studies have discussed melanoma arising from giant congenital blue nevi, few have discussed those arising from intermediate blue nevi. We present a case of a 52-yearold man with melanoma on the scalp evolving from an intermediate congenital common blue nevus.

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Abstract Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

Cutaneous involvement by cytomegalovirus in a renal transplant recipient as an indicator of severe systemic infection

Abstract Cytomegalovirus is an opportunistic virus that commonly affects immunosuppressed patients. Cutaneous involvement by this virus is rare and occurs in significantly immunocompromised hosts, with a poor prognosis. Skin ulcers may represent the first sign of systemic infection by cytomegalovirus in these patients. Herein, a case of a systemic infection by Cytomegalovirus presenting as genital and oral ulcers in a kidney-transplant recipient is reported.

Milker's nodule - Case report

Abstract Milker's nodule is an occupational viral skin disease of universal distribution, caused by the Paravaccinia virus and that occurs in individuals who deal with dairy cattle herds. We describe a case acquired due to lack of use of PPE (Personal Protective Equipment) and perform a literature review.

Alopecia mucinosa: relato de um caso com revisão de literatura e proposta dos achados dermatocópicos / Mucinous alopecia (follicular mucinosis): case report, review of the literature and dermatoscopic consideration

Doença rara, descrita por Pinkus em 1957, a alopecia mucinosa pertence às alopecias cicatriciais. É assim denominada pelo acúmulo de mucina e linfócitos na porção infundibular do aparelho pilossebáceo, promovendo destruição total e irreversível do local afetado. De etiologia desconhecida, clinicamente se manifesta em placas, geralmente normocrômicas, bem demarcadas, composta por pápulas com evidente proeminência folicular. A ausência de sintomas é a regra. Sua classificação como micose fungoide incipiente ainda não é consenso. Com pico bimodal de incidência costuma manifestar-se em crianças ou idosos, através de diferentes quadros clínicos, prognósticos e associações. Não existem, até o presente momento, estudos de dermatoscopia nessa rara entidade. Descrevemos uma apresentação atípica da doença e propomos seus achados dermatoscópicos.

Pityriasis amiantacea: clinical-dermatoscopic features and microscopy of hair tufts / Pitiríase amiantácea: aspectos clínicos e dermatoscópicos e microscopia dos tufos capilares

Pityriasis Amiantacea, also known as pseudotinea amiantacea, is a clinical syndrome which affects the scalp, having a reaction pattern reported to occur in several inflammatory dermatoses. The authors seek to establish the correlation between its clinical, dermoscopic and light microscopy features through the analysis of scale- encrusted hair tufts submitted to histological processing.

A pitiríase amiantácea, também chamada de pseudotínea amiantácea, é compreendida como uma síndrome clínica que acomete o couro cabeludo. Representa um padrão de reação a diversas dermatoses inflamatórias. Os autores procuram estabelecer a correlação entre características clínicas, dermatoscópicas e de microscopia ótica, através da análise do tufo de cabelos agregados pelas escamocrostas, submetido a processamento histológico.

Hemangioma glomeruloide e a síndrome POEMS: relato de dois casos e revisão da literatura / Glomeruloid hemangioma in POEMS syndrome: a report on two cases and a review of the literature

O hemangioma glomeruloide caracteriza-se por enovelados capilares contidos em espaços vasculares dilatados reminiscentes de glomérulos renais, sendo fortemente associado à síndrome POEMS (polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações cutâneas). Relatamse dois casos da síndrome associados a hemangiomas glomeruloides e faz-se uma revisão da literatura. O primeiro é uma paciente feminina, 63 anos, internada para investigação de ascite, hepatoesplenomegalia, dificuldade de deambulação e hemangiomas cutâneos. A histopatologia de uma dessas lesões estabeleceu o diagnóstico de hemangioma glomeruloide e direcionou a investigação, que revelou polineuropatia sensitivo-motora, plasmocitoma kappa-positivo em L4 e Diabetes mellitus, permitindo o diagnóstico da síndrome. O segundo caso é de uma paciente feminina, 39 anos, com edema, ascite, derrame pleural, hemangiomas glomeruloides e linfonodomegalias (doença de Castleman). Havia um componente monoclonal de IgG-lambda e lesões blásticas no ilíaco direito e em L4, assim como lesão desmielinizante sensitivo-motora nos quatro membros, compondo o diagnóstico de síndrome POEMS.

Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.

Líquen plano hipertrófico disseminado: relevante resposta à acitretina / Disseminated hypertrophic lichen planus: relevant response to acitretin

O líquen plano hipertrófico é uma variante do líquen plano, com pronunciada hiperplasia epidérmica em resposta à coçadura persistente. Clinicamente, caracterizam-se por placas hiperceratósicas, simétricas, de coloração cinza-violácea, com predileção pela região pré-tibial. O prurido intenso, a refratariedade aos tratamentos convencionais e a possibilidade de associação de um carcinoma epidermoide às lesões de longa duração impõem um tratamento eficaz. Os corticoides são considerados o tratamento de primeira linha e podem ser aplicados topicamente ou empregados de forma sistêmica. Outras modalidades terapêuticas propostas são a fototerapia com UVB-NB ou PUVA, imunossupressores e retinoides sistêmicos, com destaque para a acitretina. Relatamos um caso com apresentação clínica exuberante e excelente resposta à acitretin, totalizando um seguimento de nove meses.

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

Hidradenite supurativa tratada com infliximabe / Hidradenitis suppurativa treated with infliximab

A hidradenite supurativa é doença inflamatória de difícil tratamento. Recentemente, a terapia anti-TNF alfa, com anticorpos monoclonais ("terapia biológica"), tem sido apontada como uma alternativa. No entanto, ensaios clínicos avaliando a eficácia destas drogas na hidradenite supurativa, ainda não foram publicados. Apresentamos um caso no qual o uso do infliximabe não resultou em melhora clínica expressiva.

Hidradenitis suppurativa is an inflammatory disease with difficult treatment. Currently, the anti-TNF alfa therapy, with monoclonal antibodies ("biological therapy"), has been shown as a new alternative. However, clinical trials assessing the efficacy of these drugs to treat hidradenitis suppurativa have not yet been published. A case in which the use of infliximab did not result in significant clinical improvement is described.