Daniel Lago Obadia

Metástase cutânea rara de provável carcinoma basaloide de cólon simulando granuloma piogênico

Acrometastasis is a rare occurrence, especially when affecting the hands. It represents around 0.007-0.2% of all metastatic lesions. The most common site of origin is the lung, accounting for 40-50% of all cases reported in the literature. Kidneys and breasts are other sites also associated with neoplastic lesions that disseminate to the hands. More rarely, the site of origin may be the gastrointestinal tract or other systemic tumors or sarcomas. Early diagnosis is difficult, since the condition may be asymptomatic or may mimic tenosynovitis, arthritis, paronychia, pyogenic granuloma or a local infection. In the present paper, the authors report on a patient with the diagnosis of acrometastasis on both hands originating from a basaloid carcinoma of the anal canal. Response to radiotherapy was poor.

Síndrome de Sweet associada a linfoma de Hodgkin: relato de caso

Sweets syndrome is a rare cutaneous disease of unknown etiology. About 20% of the cases are associated with hematological neoplasms, and cases related with Hodgkins disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Agminated blue nevus - Case report

Blue nevi are benign melanocytic lesions located in the deeper reticular dermis, consequence of failure of melanocytic migration into the dermal-epidermal junction from the neural crest. Lesions are usually asymptomatic and solitary, but may present in a multiple or agminated (grouped) pattern. The agminated subtype is formed when bluish-pigmented lesions cluster together in a well-defined area. Lesions can be flat or raised. We report the case of a patient who presented multiple bluish macules (1-3 mm in diameter) grouped on the left upper back. Dermoscopy and anatomic pathological examination were consistent with blue nevus.

Granulomatous mycosis fungoides--a diagnostic challenge.

Granulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

Relato de Caso: Peeling Skin Syndrome

Peeling Skin Syndrome (PSS) e uma genodermatose rara, de provavel heranca autossomica recessiva, caracterizada por peeling superficial da pele durante a vida, localizada ou generalizada, com ou sem eritema e prurido. A etiologia ainda nao foi completamente elucidada. Traupe propos distincao entre tipo A, nao inflamatoria, e tipo B, associada a eritrodermia congenita (inflamatoria). O tipo generalizado nao inflamatorio e caracterizado por areas de eliminacao espontânea continua do estrato corneo, com inicio no nascimento ou durante a infância. Demonstramos o caso de um paciente masculino, 19 anos, com surgimento das lesoes na infância e historia familiar positiva.

Lentigo maligna on the face: a challenging conduct

Lentigo maligna is a melanoma in situ, of slow radial growth, which affects sun-exposed areas, especially in the elderly. When it affects the eyelid, due to the proximity to a noble organ, the conduct is controversial, but surgery is the method most commonly used, with with margins varying according to the reference used. Conservative treatments are described, such as imiquimod 5% and radiotherapy. This report aims to demonstrate the lack of studies on the surgical margin, and to name nonsurgical treatment options for lentigo maligna of the face.

Interstitial granulomatous dermatitis with arthritis

tion is in fact a new subtype of primary cutaneous amyloidosis or a variant of lichen amyloidosis.1,5 The histopathological analysis consists of a nodular deposit of amorphous, eosinophilic, and homogenous material in the papillary dermis. The overlaying epidermis is atrophic and can partially encompass the amyloid material, in thin collarettes, and can present hyperkeratosis. There is some conflicting evidence in the literature regarding the nature of the deposits. The first case reports suggest a collagenous nature of the material, as they were stained with Verhoeff-van Gieson and Periodic acid–Schiff (PAS) and not with Congo red, and presented electronic microscopy consistent with collagen deposit, and the lesions were called collagen papules of the auricular concha.4 However, the majority of the reported cases demonstrated that the material was stained with Crystal Violet and became orange-colored with Congo red, generally presenting positive birefringence when submitted to polarized light, thus suggesting the amyloid origin of such deposits.1,2,3,4 According to the author of the largest case study on this issue, they could represent two distinct entities, though clinically similar, which could have been clarified if immunohistochemistry had been used in the first cases.4 The material deposited in our patient presented a negative birefringence, which does not exclude the diagnosis of primary cutaneous amyloidosis, based on the clinical and histopathological findings. It is suggested that the amyloid material has an epidermal origin, since the immunohistochemical profile is positive for CK 34beta32, which corresponds to cytokeratins of high molecular weight, such as CK 1, 5, 10, and 14, which react with the squamous epithelium, including the epidermis.4 There is no specific treatment capable of removing the amyloid deposits. As the papules of the auricular concha are mostly asymptomatic, localized, and superficial, then electrocoagulation, curettage, and excision seem to be sufficient for a good aesthetic result.2,4q

Morbihan disease: a therapeutic challenge

Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Steatocystoma multiplex suppurativa: case report of a rare condition

Steatocystoma multiplex is a rare genetic disorder characterized by the presence of hamartomatous malformations at the junction of the pilosebaceous duct. It consists of encapsulated cystic lesions in the dermis, with adjacent sebaceous gland. When associated with inflammation, resembling hidradenitis, it is called steatocystoma multiplex suppurativa, a condition rarely reported. This is the first case of steatocystoma multiplex suppurativa reported in the Brazilian literature. Female patient, 23 years old, with papular and nodular cystic lesions that started in the armpits and groin, later spreading to the trunk, lower limbs, anticubital fossa, face and scalp. The presence of papular-nodular lesions associated with disseminated hidradenitis-like lesions in flexural areas and the histopathological diagnosis of steatocystoma defined the diagnosis of steatocystoma multiplex suppurativa.

Syndrome in question. Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome is an autosomal dominant disorder with variable penetrance and expression. It is characterized by a genetic predisposition to develop multiple adnexal neoplasias: cylindromas, trichoepitheliomas, and trichoblastomas. We describe a 54-year-old male patient with cylindromas, trichoepitheliomas, and trichoblastoma.