Progressive symmetrical erythrokeratodermia -- case report.

Progressive symmetrical erythrokeratodermia is a rare autosomal dominant genodermatosis with variable penetrance described by Darier in 1911. It is characterized by erythematous and keratotic plaques, sharply defined and symmetrically distributed along the extremities, buttocks and, more rarely, on the face. We report a case of a 55-year-old patient with lesions on the dorsum of the hands, interphalangeal pads, wrists, groin and back feet. This case demonstrates a rare and late diagnosis, clinical profusion and presence of familiar involvement.

Umbilical endometriosis: report of a case and its dermoscopic features.

Cutaneous endometriosis is a rare manifestation of endometriosis, representing 0.5% to 1% of all endometriosis cases. It can be divided into primary and secondary, when appearing spontaneously or after a surgical procedure, when it is mostly found on surgical scar tissue. Some etiologies were proposed, but none of them could entirely explain the appearance of the tumor. Differential diagnosis includes melanoma, metastatic nodule, keloid and pyogenic granuloma. Dermoscopic features are not yet well established, but there are some characteristics that suggest the diagnosis. Treatment is surgical in larger sized lesions. Malignization can occur. The screening for endometriosis is mandatory by means of gynecologic, imaging and marked-tumor evaluation. We report a case of primary umbilical endometriosis and discuss its dermoscopic aspects.

Do you know this syndrome? / Você conhece esta síndrome?

Piloleiomyoma, a benign smooth-muscle tumor arising from the arrectores pilorum muscles of the skin, affects males and females in the third decade of life. It presents as asymmetrical, reddish-brown nodules or papules with a firm consistency, predominantly located on the limbs. When multiple lesions are present, they may be tender or painful. Their association with uterine fibroids, referred to as Reed syndrome or familial leiomyomatosis cutis et uteri, is rare and may be associated with renal cell carcinoma. The approach consists of surgical excision in cases presenting few lesions and pharmacological treatment if symptomatic. The present paper describes a case of Reed syndrome in which a decision was made to monitor the patient in view of the absence of symptoms.

Tumor benigno de tecido muscular, o piloleiomioma tem origem no músculo eretor do pelo, atingindo ambos os sexos geralmente na terceira década de vida. Apresenta-se como nódulo-pápulas assimétricas nas extremidades, de cor eritêmato-acastanhada e de consistência firme. As lesões, quando múltiplas, podem ser sensíveis ou dolorosas. Sua associação com miomas uterinos, denominada de síndrome de Reed ou leiomiomatose cutis et uteri, é apresentação rara, podendo estar associada a carcinoma de células renais. A abordagem é cirúrgica em casos isolados e medicamentosa se houver sintomas. Relatamos um caso de síndrome de Reed em que se optou por acompanhamento pela ausência de sintomatologia.

Disseminated hypertrophic lichen planus: relevant response to acitretin.

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.

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.

[Do you know this syndrome? Piloleiomyoma]. / Você conhece esta síndrome? Piloleiomioma.

Piloleiomyoma, a benign smooth-muscle tumor arising from the arrectores pilorum muscles of the skin, affects males and females in the third decade of life. It presents as asymmetrical, reddish-brown nodules or papules with a firm consistency, predominantly located on the limbs. When multiple lesions are present, they may be tender or painful. Their association with uterine fibroids, referred to as Reed syndrome or familial leiomyomatosis cutis et uteri, is rare and may be associated with renal cell carcinoma. The approach consists of surgical excision in cases presenting few lesions and pharmacological treatment if symptomatic. The present paper describes a case of Reed syndrome in which a decision was made to monitor the patient in view of the absence of symptoms.

[Psoriasis of von Zumbusch.

A disorder of unknown origin, psoriasis is characterized by erythemato-squamous eruption. Amongst its clinical and morphologic presentations there is acute generalized pustular psoriasis, termed the von Zumbusch variant, accompanied by generalized toxicity. It is often triggered when stopping steroids, in unstable psoriasis patients. Case report of atypical development that began during prednisone use; complete clearing of lesions and symptoms was achieved with 35 days of acitretin.