Proximal-type epithelioid sarcoma - Case report

Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.     

Ectopic cutaneous schistosomiasis - Case report

Schistosomiasis mansoni is a systemic disease caused by a helminth of the schistosomagenus. The disease is generally asymptomatic or gastrointestinal symptoms maypredominate. Skin lesions related to the disease are rarely diagnosed, even inendemic areas. The authors report the case of a young girl diagnosed with cutaneousschistosomiasis with involvement of the abdomen, back and scapular region.Schistosoma eggs were found in the lesions by histopathologic exam. There was noevidence of systemic involvement. Schistosomiasis must be included in the list ofdifferential diagnosis of skin damage, especially in endemic areas, due to thepotential consequences, in case of late diagnosis and treatment.     

Targetoid hemosiderotic hemangioma - Case report

Targetoid Hemosiderotic Hemangioma, also known as Hobnail Hemangioma, is a lesion ofvascular origin, probably lymphatic. The most common clinical feature is a solitaryviolaceous papule surrounded by a pale, thin area and a peripheral ecchymotic ring,simulating a target. Histopathologically, there is a biphasic pattern, with dilatedvessels in the superficial dermis and pseudoangiosarcomatous pattern in the deepdermis, and endothelial cells with hobnail morphology. A simple excision is curative.We report a rare case of Targetoid Hemosiderotic Hemangioma.     

Ectopic cutaneous schistosomiasis - Case report

Schistosomiasis is best known in its visceral form but it can attack the skin, its ectopic cutaneous manifestation being rare and clinically difficult to diagnose. It is characterized by isolated or coalescent papules, erythematous, pruritic or asymptomatic, with zosteriform distribution, often located on the trunk. The authors report a case of a 28-year-old female patient with lesions on the abdomen, with positive stool results for Schistosoma and absence of active symptoms of visceral disease. The case reveals rare exuberant cutaneous manifestation and the importance of the diagnosis of this entity in patients from endemic regions.     

Unilateral punctate porokeratosis - Case report

This case report involves a 20-year-old man with unilateral punctate porokeratosis. The patient presented an 8-year history of numerous asymptomatic keratotic papules and pits with linear distribution on his left pal-mar surface and fifth finger of the left hand. Histopathological examination of the keratotic plug revealed findings of distinct epidermal depressions containing cornoid lamellae. This report review draws attention to differential diagnoses of punctate porokeratosis.     

Primary essential cutis verticis gyrata - Case report

Cutis verticis gyrata is characterized by excessive formation of scalp skin. It may be primary (essential and nonessential) or secondary. In the primary essential form it presents only folding skin formation on the scalp, mimicking cerebral gyri, without associated comorbidities. We report a rare case of a 28 year-old male patient with primary essential cutis verticis gyrata.     

Alkaptonuria - Case report

Alkaptonuria, also called endogenous ochronosis, is a rare metabolic autosomalrecessive disorder. It occurs by complete inhibition of homogentisic acid oxidaseenzyme having its deposition in various tissues. Male patient, 52 years old, soughtmedical help complaining about progressive appearance of hyperchromic papules on thelateral edge of the second finger of both hands for 02 years. He also complainedabout darkening of urine, sperm and underwear. Incisional biopsy of second handfinger and test for homogentisic acid in the urine results were positive. Thefindings are compatible with the diagnosis of alkaptonuria. Given these findings,treatment was initiated, followed-up by other specialties and he was advised to avoidcertain foods.     

Argyria - Case report

A 70-year-old male rural worker was referred to our clinic with widespread grey pigmentation of the skin and nails. The condition had been asymptomatic for its entire duration (5 years). He reported past intranasal application of 10% Silver Vitellinate. A skin biopsy was performed and histology corroborated the clinical diagnosis of Argyria. This case represents a currently rare dermatological curiosity. Although silver colloids and salts have been withdrawn and/or banned by some drug surveillance agencies, they continue to be freely sold and unregulated as food supplements and as ingredients in alternative medicines, thereby risking the emergence of new cases of silver poisoning.     

Psoriasiform Keratosis - Case report

Psoriasiform Keratosis is a rare clinic entity. The etiopathogenesis remains unknownand the disease is characterized by a solitary, scaly or keratotic papule, or plaquemainly located on the extremities. Histopathological features closely resemble thoseof psoriasis. We report the case of a 70-year-old woman presenting a solitary andasymptomatic keratotic plaque, located on the back of the left leg, unresponsive totopical corticosteroids. We performed an excisional biopsy and histopathology wasconsistent with psoriasiform keratosis.     

Follicular mucinosis - Case report

Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.     

A case of primary cutaneous Ewing sarcoma in a neutropenic patient

Primary cutaneous Ewing sarcoma is a rare clinical presentation of Ewing sarcoma, usually occurring as a small, localized tumor on the extremities of young adults and associated with favorable prognosis. We report a case of primary cutaneous Ewing sarcoma, which presented on the sole of the foot of a 27‐year‐old patient with relapsed acute myeloid leukemia and neutropenia. Diagnosis was determined through histological features and staining, as well as fluorescence in situ hybridization and molecular testing. The patient underwent wide‐local excision with plan to begin targeted chemotherapy, but unfortunately died from adenovirus pneumonia while neutropenic before targeted chemotherapy was initiated.     

Case report of cutaneous histiocytic sarcoma: diagnostic and therapeutic dilemmas

Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.     

Phaeohyphomycosis of the ungual apparatus - Case report

Phaeohyphomycosis is a disease caused by dematiaceous fungi with a worldwidegeographic distribution and broad spectrum. It is most commonly found in adultindividuals of both genders and all races. We report the case of a 57-year-oldwoman with phaeohyphomycosis in the ungual apparatus.     

Vibrio vulnificus infection in Southern Brazil - Case report

The genus Vibrio is a member of the family Vibrionaceae, and among their disease-causing species, Vibrio vulnificus, a lactose-positive gram-negative bacillus, is one of the most virulent pathogen of the noncholerae vibrios. We describe the case of a 39-year-old male patient, who was using immunosuppressive therapy, admitted to the hospital for liver transplantation. Twelve hours later, the patient presented high fever, myalgia, anuria and erythematous plaques on lower limbs, of rapid growth and proximal progression. The patient was treated with ceftriaxone, meropenem and oxacillin, however he expired within 30 hours. Blood cultures showed growth of a gram-negative bacillus, which was later identified as Vibrio vulnificus.     

Musculoskeletal and cutaneous sarcoidosis: exuberant case report

Sarcoidosis is a multisystem granulomatous disease of unknown cause. The osteoarticular involvement in sarcoidosis is rare and is often associated with cutaneous and long-standing chronic multisystem disease. More common in black women, osseous sarcoidosis is difficult to diagnose, with an incidence of 3 to 13%. The most characteristic radiological clinical picture evidences rounded, well-defined cysts, with no periosteal reaction and without peripheral sclerosis. The small bones of hands and feet are the most frequently involved sites. This report aims to demonstrate a rare case of osteoarticular sarcoidosis with characteristic clinical presentation, and highlight the importance of detecting osteoarticular involvement in this pathology.     

Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report

A 24-year-old male patient, who underwent kidney transplant six years ago due toLupus nephritis, for the last two years presented asymptomatic erythematous scalyplaques on the abdomen and areas exposed to light. Post-transplantationimmunosuppressive medications included prednisone, mycophenolate sodium andsirolimus. The histopathologic features were typical for epidermodysplasiaverruciformis. Epidermodysplasia verruciformis is a rare autosomal recessivegenodermatosis with increased susceptibility to specific strains of cutaneous humanpapilloma virus. The term ''''acquired epidermodysplasia verruciformis'''' was recentlyintroduced to the literature and describes epidermodysplasia verruciformis occurringin patients with impaired cell-mediated immunity. We report an additional caseassociated to immunosuppression after kidney transplantation.     

Bannayan-Riley-Ruvalcaba syndrome with deforming lipomatous hamartomas in infant - Case report

Bannayan-Riley-Ruvalcaba Syndrome is a rare condition caused by mutations in the PTENgene. It displays association of multiple lipomas, macrocephaly, hemangiomas,hamartomatous intestinal polyposis, developmental delay and speckled pigmentedmaculae on the male genitalia. We report the case of a nine-month-old boy who hadfast growing and progressive tumors for three months, macrocephaly and lentigines onthe penis. Imaging tests showed extensive lipomatosis with invasion of paraspinalmuscles, enlargement of the spinal canal and spinal cord compression; after surgicalexcision of the mass, the pathology was consistent with lipoma. Adipocyte culturekaryotype demonstrated PTEN mutation. We present this case for its rarity andexuberance.     

Epidermolytic Hyperkeratosis - case report

Epidermolytic hipercetarose is a rare genodermatosis, with a prevalence of1:100.000 to 1:300.000, with autosomal dominant inheritance. We report thecase of a 5 year old girlwho presented an hypertrophic verrucous plaques inthe neck, under arm, buttocks, knees, pelvis, legs, dorsum of the rightfoot and elbows. Histological examination of the skin lesions showedtypical changes of epidermolytic hyperkeratosis. Because it is an autosomaldominant disorder with complete penetrance, the individual carrying themutation will necessarily develop the disease. However, in 50% of casespostzygotic mutation occur. The case report emphasizes early diagnosis anddifferential diagnoses with ichthyosis and other bullous diseases ofchildhood, as well as discussing the therapeutic possibilities.     

Multiple metastatic basal cell carcinoma with concurrent metastatic pleomorphic sarcoma in chronic lymphedema area - Case report

Chronic lymphedema presents as interstitial fluid retention due to a failure in thelymphatic system drainage. The affected region becomes more vulnerableimmunologically and predisposed to the onset of neoplasms. Basal Cell Carcinoma isthe most common sort of neoplasm, nevertheless it rarely metastisizes. Sarcomas aremalignant mesenchymal neoplasms, locally aggressive, which can spread. Here isreported an infrequent case of multiple basal cell carcinoma, synchronous to a poorlydifferentiated pleomorphic sarcoma, both spreading to lymph nodes and arising fromtissue compromised by chronic lymphedema.     

De Sanctis-Cacchione Syndrome in a female infant - Case report

The De Sanctis-Cacchione Syndrome is the rarest and most severe kind of xeroderma pigmentosum, characterized by microcephaly, hypogonadism, neurological disorders, mental and growth retardation, with very few cases published. The clinical findings compatible with De Sanctis-Cacchione Syndrome and the therapeutic approach used to treat a one year and nine months old child, with previous diagnosis of xeroderma pigmentosum, are reported.