Scielo RSS <![CDATA[Anais Brasileiros de Dermatologia]]> http://www.scielo.br/rss.php?pid=0365-059620160011&lang=en vol. 91 num. 5 lang. en <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Acquired perforating dermatosis in a patient with chronic renal failure]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100010&lng=en&nrm=iso&tlng=en Abstract Perforating dermatoses are a group of skin diseases characterized by transepidermal elimination of dermal material. The disease is divided into two groups: the primary group and the secondary group. The classical or primary perforating dermatoses are subdivided into four types according to the eliminated dermal materials: Kyrle disease, perforating reactive collagenosis, elastosis perforans serpiginosa, and perforating folliculitis. The secondary form is known as acquired perforating dermatosis. The term was proposed in 1989 by Rapini to designate the perforating dermatoses affecting adult patients with systemic disease, regardless of the dermal materials eliminated. This report describes a case of the disease with elimination of collagen and elastic fibers in a patient with chronic renal failure. <![CDATA[Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100014&lng=en&nrm=iso&tlng=en Abstract Frontal fibrosing alopecia is a distinctive form of scarring alopecia considered to be a clinical variant of lichen planopilaris. It predominantly occurs in postmenopausal women and has a slowly progressive course. It was first described by Kossard in 1994. Since then the number of reported cases has increased significantly. Coexistence of frontal fibrosing alopecia and autoimmune disorders - such as discoid erythematosus lupus and Sjögren's syndrome - may suggest a common pathogenic background among the diseases. <![CDATA[Bromoderma in an infant]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100017&lng=en&nrm=iso&tlng=en Abstract Bromoderma is a cutaneous eruption caused by the absorption of bromide. Clinical manifestations include acneiform and vegetative lesions. We report the case of an infant with bromoderma caused by the use of syrup for abdominal colic containing calcium bromide. The lesions regressed after discontinuation of the drug. <![CDATA[Coexistence of nail lichen planus and lichen planus pigmentosus]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100020&lng=en&nrm=iso&tlng=en Abstract We describe a fifty-six-year old, Afro-descendent female patient showing dystrophy of her twenty nails and hyperchromic, asymptomatic macule on her face. Histopathological examination of the macule showed vacuolization of the basal layer, melanophages in the superficial dermis and lymphoplasmocytic inflammatory infiltrate. Nail biopsy revealed orthokeratotic hyperkeratosis and lichenoid inflammatory infiltrate. Lichen planus pigmentosus is an uncommon variety of lichen planus. It is characterized by typical hyperpigmented macules on the face and neck. Nail changes might be present in 10% of lichen planus cases, but no associations with lichen planus pigmentosus have been described. We report a case of lichen planus in twenty nails associated with lichen planus pigmentosus on the patient's face. <![CDATA[Congenital hemangioma in spondylocostal dysostosis: a novel association]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100023&lng=en&nrm=iso&tlng=en Abstract Congenital hemangioma is a benign tumor caused by dysfunction in embryogenesis and vasculogenesis, which progresses during fetal life to manifest as fully developed at birth. Although hemangiomas are the most common tumor of infancy, rapidly involuting congenital hemangioma has not been described in spondylocostal dysostosis. I report the novel association of congenital hemangioma and spondylocostal dysostosis in a Mexican newborn female patient with neural tube defects. Given the embryological relationship between skin and nervous system, I surmise that this association is not coincidental. I also propose that these morphologic alterations be incorporated to the spondylocostal dysostosis phenotype and specifically looked for in other affected children, in order to provide appropriate medical management and genetic counseling. <![CDATA[Chemical leukoderma induced by dimethyl sulfate]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100026&lng=en&nrm=iso&tlng=en Abstract Chemical leukoderma occurs due to the toxic effect of a variety of chemical agents. Mechanisms include either destruction or inhibition of melanocytes. We report two male patients (36 and 51 years old) who presented with multiple hypopigmented macules and patches on the neck, wrist, and legs after exposure to dimethyl sulfate in a chemical industry. Physical examination revealed irregular depigmentation macules with sharp edges and clear hyperpigmentation around the lesions. History of repeated exposure to a chemical agent can help the clinical diagnosis of chemical leukoderma. This diagnosis is very important for prognosis and therapeutic management of the disease. <![CDATA[Disseminated cryptococcosis manifested as a single tumor in an immunocompetent patient, similar to the cutaneous primary forms]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100029&lng=en&nrm=iso&tlng=en Abstract Cryptococcosis is a fungal infection caused by Cryptococcus neoformans that tends to affect immunocompromised individuals. The fungi are mostly acquired by inhalation, which leads to an initial pulmonary infection. Later, other organs - such as the central nervous system and the skin - can be affected by hematogenous spread. In addition, cutaneous contamination can occur by primary inoculation after injuries (primary cutaneous cryptococcosis), whose diagnosis is defined based on the absence of systemic involvement. The clinical presentation of cutaneous forms typically vary according to the infection mode. We report an unusual case of disseminated cryptococcosis in an immunocompetent patient with cutaneous lesions similar to those caused by primary inoculation. This clinical picture leads us to question the definition of primary cutaneous cryptococcosis established in the literature. <![CDATA[Linear IgA and IgG bullous dermatosis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100032&lng=en&nrm=iso&tlng=en Abstract Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant. <![CDATA[Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100035&lng=en&nrm=iso&tlng=en Abstract Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation. <![CDATA[Elastoma: clinical and histopathological aspects of a rare disease]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100039&lng=en&nrm=iso&tlng=en Abstract Elastoma is a connective tissue nevus characterized by changes in elastic fibers. It can be congenital or acquired, and is usually diagnosed before puberty. Associated with osteopoikilosis, it is known as Buschke-Ollendorff syndrome. Histopathology with specific staining for elastic fibers is critical for a diagnostic conclusion. This report describes the case of a 7-year-old male patient with lesions diagnosed as elastoma, with absence of bone changes in the radiological imaging. This study aims to report the clinical presentation and histological examination of such unusual disease. <![CDATA[Erythroplasia of Queyrat treated with topical 5-fluorouracil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100042&lng=en&nrm=iso&tlng=en Abstract We report a 33-year-old male patient diagnosed with erythroplasia of Queyrat. The patient had an erythematous and eroded lesion affecting more than 50% of the glans associated with bleeding and local pain. Despite previous indication of penectomy, he was successfully treated with topical 5-fluorouracil. <![CDATA[Lichen planopilaris-like eruption during treatment with tyrosine kinase inhibitor nilotinib]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100045&lng=en&nrm=iso&tlng=en Abstract Tyrosine kinase inhibitors are effective as a target therapy for malignant neoplasms. Imatinib was the first tyrosine kinase inhibitor used. After its introduction, several other drugs have appeared with a similar mechanism of action, but less prone to causing resistance. Even though these drugs are selective, their toxicity does not exclusively target cancer cells, and skin toxicity is the most common non-hematologic adverse effect. We report an eruption similar to lichen planopilaris that developed during therapy with nilotinib, a second generation tyrosine kinase inhibitor, in a patient with chronic myeloid leukemia resistant to imatinib. In a literature review, we found only one report of non-scarring alopecia due to the use of nilotinib. <![CDATA[Scleromyxedema: clinical diagnosis and autopsy findings]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100048&lng=en&nrm=iso&tlng=en Abstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide. <![CDATA[Steatocystoma multiplex suppurativa: case report of a rare condition]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100051&lng=en&nrm=iso&tlng=en Abstract 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. <![CDATA[Phacomatosis pigmentovascularis of cesioflammea type]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100054&lng=en&nrm=iso&tlng=en Abstract Phacomatosis pigmentovascularis is a rare syndrome, defined as the simultaneous presence of vascular nevus and melanocytic nevus in the same patient. We report the case of a 53-year-old woman presented with dermal melanosis and extensive vascular nevus, which match the typical manifestations of phakomatosis pigmetovascularis of cesioflammea type, according to Happle's classification. The rare occurrence of this genodermatosis and the clinical exuberance of the skin lesions motivated this case report. <![CDATA[Eosinophilic fasciitis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100057&lng=en&nrm=iso&tlng=en Abstract Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Clinical features begin acutely, with local edema and a painful and symmetrical stiffening of the limbs, progressing rapidly to fibrosis, which can limit joint movements. Some cases have a history of strenuous physical exercise or trauma. The diagnosis is confirmed by a deep skin biopsy. Glucocorticoids in high doses is the treatment of choice. We report a typical eosinophilic fasciitis case with peripheral eosinophilia and dramatic response to pulse therapy with methylprednisolone. <![CDATA[Lucio's phenomenon: exuberant case report and review of Brazilian cases]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100060&lng=en&nrm=iso&tlng=en Abstract Lucio’s phenomenon is an uncommon reaction characterized by severe necrotizing cutaneous lesions that occurs in patients with Lucio’s leprosy and lepromatous leprosy. It is considered by some authors as a variant of type 2 or 3 reaction. Death can occur because of blood dyscrasia or sepsis. Precipitating factors include infections, drugs and pregnancy. We report a 31-year-old female patient exhibiting both clinical and histopathological features of lepromatous leprosy and Lucio’s phenomenon presenting favorable response to treatment. We complement our report with a literature review of the Brazilian cases of Lucio’s phenomenon published in Portuguese and English. <![CDATA[Follicular traction urticaria]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100064&lng=en&nrm=iso&tlng=en Abstract Inducible urticaria is a heterogeneous subgroup of chronic urticarias caused by a wide variety of environmental stimuli, such as exercise, cold, heat, pressure, sunlight, vibration, and water. A new term, follicular traction urticaria, was suggested as an unusual form of inducible urticarias. We report a patient who was diagnosed with follicular traction urticaria. <![CDATA[Pruritic folliculitis of pregnancy]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100066&lng=en&nrm=iso&tlng=en Abstract Pruritic folliculitis of pregnancy is a rare disease of unknown etiology. It occcurs primarily during pregnancy, usually with spontaneous resolution postpartum. It is characterized by a benign dermatosis, with papular and pustular follicular lesions that first appear on the torso and occasionally spread throughout the body. We report the case of a patient in the 27th week of pregnancy, with a two-month evolution of pruritic and papular erythematous lesions on her lower back. Differential diagnosis includes other pregnancy-specific dermatoses: gestational pemphigoid, pruritic urticarial papules and plaques of pregnancy (PUPPP), prurigo of pregnancy, and (PUPPP) and prurigo of pregancy. Histopathological tests showed changes consistent with pruritic folliculitis of pregnancy. This case is relevant due to its rare nature and its clinical and histopathological characteristics. <![CDATA[Leprosy on the scalp]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100069&lng=en&nrm=iso&tlng=en Abstract Leprosy is a chronic infectious disease caused by Mycobacterium leprae. This bacillus has a high predilection for skin and peripheral nerves. The scalp’s anatomical properties do not favor the development of such mycobacterium. We report a case of leprosy with scalp involvement, a rare occurrence in our literature. <![CDATA[Cutaneous leukemic infiltration following varicella - a case of Wolf's isotopic response]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100072&lng=en&nrm=iso&tlng=en Abstract Wolf's isotopic response designates the appearance of two subsequent unrelated dermatoses in the same anatomic location. We report the case of a 51-year-old man with a medical history of chronic lymphocytic leukemia without known extra-hematopoietic involvement. The patient developed a disseminated papulo-vesiculous eruption, diagnosed as varicella. Few days after recovering, an erythematous and violaceous papular dermatosis with histopathological examination compatible with leukemic infiltration appeared on the scars of previous herpetic lesions. Complete remission was obtained under systemic corticotherapy, without cutaneous recurrence or blastic transformation. Wolf's isotopic response is attributed to a localized immunologic imbalance following a certain stimulus. In this patient, herpetic infection acted as a local spur for inaugural cutaneous leukemic infiltration, with no impact on the prognosis for the underlying disease. <![CDATA[Interferon-gamma-induced local leukocytoclastic vasculitis at the subcutaneous injection site]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100076&lng=en&nrm=iso&tlng=en Abstract Cutaneous reactions associated with interferons (IFNs) treatment are either localized or generalized. The most common presentation of localized reactions at IFNs injection site is usually an erythematous patch or plaque. Local leukocytoclastic vasculitis presenting with cutaneous necrosis is extremely rare. We report a 19-year-old man with hepatitis B who had local leukocytoclastic vasculitis induced by interferon-gama injection at the injection site. After changing the injection sites and using the combined treatment of prednisone and colchicine, the previous lesion healed and no other cutaneous lesion occurred. We also made a mini review of such cases. <![CDATA[Laptop computer induced erythema ab igne: a new presentation of an old disease]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100079&lng=en&nrm=iso&tlng=en Abstract Erythema ab igne is a condition characterized by skin changes due to chronic exposure to moderate temperature. We describe a female patient with continuous use of a laptop computer on exposed legs for 6 months and consequent development of reticulated hyperpigmentation at the area. Histopathological examination revealed epidermal atrophy, collagen fragmentation, and vacuolar changes in the basal layer, among other signs. We consider this case to be a modern cause of erythema ab igne. <![CDATA[Extensive bullous lichen sclerosus et atrophicus]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100081&lng=en&nrm=iso&tlng=en Abstract Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. The patient was unsuccessfully treated with standard topical corticosteroid therapy, doxycycline and chloroquine. According to the literature, there is little evidence of the efficacy of doxycycline and hydroxychloroquine in the treatment of BLSA. We report a rare case of extensive BLSA that is unresponsive to these drugs. <![CDATA[Giant oral lipoma: a rare entity]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100084&lng=en&nrm=iso&tlng=en Abstract Lipomas are very common benign slow-growing soft tissue neoplasms composed of mature adipose tissue mostly diagnosed in the fifth decade of life. These tumors rarely present in the oral cavity, representing less than approximately 5% of all benign mouth tumors. They are usually less than 2cm in size and etiology remains unclear. We report a young male patient presenting with a giant lipoma in the buccal mucosa. Histopathology revealed a large area of mature fat cells consistent with conventional lipoma and an area of the mucosal lining of the lesion suggestive of morsicatio buccarum. In the present article, we emphasize the clinicopathological features and differential diagnosis of the disease. <![CDATA[Lupus tumidus: a report of two cases]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100087&lng=en&nrm=iso&tlng=en Abstract Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Treatment is based on photoprotection, topical corticosteroids and antimalarials. We report two cases of lupus tumidus, which deserve attention for their low frequency in the literature, in addition to their relevance as a differential diagnosis among dermatologic disorders. <![CDATA[Bier’s spots with onset in childhood]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100090&lng=en&nrm=iso&tlng=en Abstract Bier spots are small, irregular, hypopigmented macules that are usually found on the arms and legs. The macules disappear when the limb is raised. Bier spots have been reported in association with a number of conditions but there is no consistent association to specific desease. Although they usually affect young adults, we report a case of Bier spots that began in childhood. As an asymptomatic and possibly transitional condition, the disease does not require treatment. <![CDATA[Dermatologic manifestations of infective endocarditis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100092&lng=en&nrm=iso&tlng=en Abstract Despite advances in diagnosis and treatment, infective endocarditis still shows considerable morbidity and mortality rates. The dermatological examination in patients with suspected infective endocarditis may prove very useful, as it might reveal suggestive abnormalities of this disease, such as Osler’s nodes and Janeway lesions. Osler’s nodes are painful, purple nodular lesions, usually found on the tips of fingers and toes. Janeway lesions, in turn, are painless erythematous macules that usually affect palms and soles. We report a case of infective endocarditis and highlight the importance of skin examination as a very important element in the presumptive diagnosis of infective endocarditis. <![CDATA[Melanoma as a surprising solution to the puzzle of intestinal obstruction]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100095&lng=en&nrm=iso&tlng=en Abstract We present a case of a 71-year-old man with an advanced melanoma of the right colon. The final diagnosis was determined based on histopathological examination of the material collected during urgent laparotomy performed due to ileus. Although we considered the tumor to be a disseminated primary melanoma of the colon, the possibility of unknown primary origin could not be excluded. Palliative chemotherapy and radiotherapy reduced symptoms associated with the disease and prolonged patient's survival. <![CDATA[Multiple cutaneous melanomas associated with gastric and brain metastases]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100098&lng=en&nrm=iso&tlng=en Abstract The occurrence of multiple primary melanomas in a single individual is rare. Most commonly, malignant melanocytic lesions subsequent to the initial diagnosis of melanoma are secondary cutaneous metastases. We report a patient with gastrointestinal bleeding from gastric metastasis of cutaneous melanoma. During clinical evaluation and staging, we discovered a brain metastasis associated with 3 synchronous primary cutaneous melanomas. We suggest the research on the mutation in the cyclin-dependent kinase inhibitor 2A (CDKN2A) (INK4a) in such cases. We also emphasize the importance of clinical examination and dermoscopy of the entire tegument, even after a malignant melanocytic lesion is identified. <![CDATA[Ovarian carcinoma presenting as cutaneous nasal metastasis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100101&lng=en&nrm=iso&tlng=en Abstract Metastatic ovarian cancer uncommonly presents with skin metastasis. When present, skin metastases of ovarian cancer are usually localized in the vicinity of the primary tumor. We report a case of a 58-year-old woman with a rapid growing erythematous, well-defined nodule localized on the left nasal ala. A skin biopsy was performed and histopathological and immunohistochemical findings were compatible with a cutaneous metastasis of adenocarcinoma. A systematic investigation revealed a bilateral ovarian cystadenocarcinoma associated with visceral dissemination, likely associated with nose cutaneous metastasis. We report a very uncommon case because of the presentation of ovarian carcinoma as cutaneous metastasis. To our knowledge, this atypical localization on the nose has not been described yet in the literature. <![CDATA[Cutaneous metastasis as the first manifestation of occult malignant breast neoplasia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100105&lng=en&nrm=iso&tlng=en Abstract Cutaneous metastases from primary internal malignancies represent 0.7-9% of patients with cancer. We report a 65-year-old female patient referred for evaluation of normochromic papules on the trunk and upper limbs that had been present for three months. A skin biopsy revealed diffuse cutaneous infiltration by small round cell tumors. Immunohistochemistry was positive for AE1/AE3, CK7, estrogen receptor and mammaglobin. The final diagnosis was cutaneous metastasis of occult breast cancer, since the solid primary tumor was not identified. The location of the primary tumor can not be determined in 5-10% of cases. In these cases, 27% are identified before the patient’s death, 57% at autopsy, and the remaining 16% can not be located. <![CDATA[Mycosis fungoides and Kaposi’s sarcoma association in an HIV-negative patient]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100108&lng=en&nrm=iso&tlng=en Abstract The association of mycosis fungoides and kaposi’s sarcoma in HIV-negative patients is a rare phenomenon. The presence of human herpesvirus 8 (HHV-8) – associated with all forms of Kaposi’s sarcoma – has also been recently identified in mycosis fungoides lesions. However, a causal association between HHV-8 and the onset of mycosis fungoides has not been established yet. The present case reports a patient who developed Kaposi’s sarcoma lesions after a two-year UVB phototherapy to treat a mycosis fungoides. Negative immunohistochemistry staining for Kaposi’s sarcoma-associated herpesvirus in the initial mycosis fungoides lesions strengthens the absence of a link between Kaposi’s sarcoma-associated herpesvirus and mycosis fungoides. Immunosuppression caused by the lymphoma and prolonged phototherapy were probably the contribut ing factors for the onset of Kaposi’s sarcoma. <![CDATA[Acral papular mucinosis: a new case of this rare entity]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100111&lng=en&nrm=iso&tlng=en Abstract Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis. <![CDATA[Nevus lipomatosus cutaneous superficialis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100114&lng=en&nrm=iso&tlng=en Abstract We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle (NCLS), with multiple lesions, in a ten-year-old child. The NLCS is considered rare. The classical clinical presentation is characterized by multiple skin-colored or yellowish papules and nodules, which can have a linear distribution. Histologically, it is characterized by the presence of mature ectopic adipocytes in the dermis. The main therapeutic option is surgical excision. The classical Nevus lipomatosus cutaneous superficialis is reported in this case. <![CDATA[Onychogryphosis in tuberous sclerosis complex: an unusual feature]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100116&lng=en&nrm=iso&tlng=en Abstract Onychogryphosis is an acquired nail plate change. It often affects the toenail and is characterized by an opaque, yellow-brownish nail plate that is distorted, grossly thickened, elongated, and partly curved resembling a ram's horn. Tuberous sclerosis complex is a multisystem disorder associated with high rates of mental retardation, autism, cognitive impairment, behavioral problems, or seizures. Nail disease can also be associated, which is a concern to patients due to pain and nail distortion. We reported a typical tuberous sclerosis complex patient with distinctive clinical features of a ram's horn nails, which presented a great challenge to surgical treatment and nail restoration. <![CDATA[Porokeratosis simulating Bowen’s disease on dermoscopy]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100119&lng=en&nrm=iso&tlng=en Abstract Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled “volcanic crater contour” – the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen’s disease due to the many glomerular vessels seen on clinical examination and dermoscopy. <![CDATA[Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100122&lng=en&nrm=iso&tlng=en Abstract A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area.. <![CDATA[Pili canaliculi as manifestation of giant axonal neuropathy]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100125&lng=en&nrm=iso&tlng=en Abstract Giant axonal neuropathy is a rare autosomal recessive neurodegenerative disease. The condition is characterized by neurons with abnormally large axons due to intracellular filament accumulation. The swollen axons affect both the peripheral and central nervous system. A 6-year old female patient had been referred to a geneticist reporting problems with walking and hypotonia. At the age of 10, she became wheelchair dependent. Scanning electron microscopy of a curly hair classified it as pili canaliculi. GAN gene sequencing demonstrated mutation c.1456G&gt;A (p.GLU486LYS). At the age of 12, the patient died due to respiratory complications. Dermatologists should be aware of this entity since hair changes are considered suggestive of GAN. <![CDATA[Plasmoacanthoma]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100128&lng=en&nrm=iso&tlng=en Abstract Plasmoacanthoma is an extremely rare verrucous tumor located on periorificial regions characterized by dense dermal plasmacytic infiltrates. Some authors classify it as a form of reactive plasma cell proliferation which represents a heterogeneous spectrum of mucocutaneous disorders. These plasma cell proliferations have been considered to be a benign immunologic inflammatory reaction to known or unknown stimuli. However, the etiology of plasmoacanthoma remains highly speculative. We report the case of a 40-year-old woman who presented with a lobulated warty lesion affecting the lower lip. Biopsy from the lesion was compatible with plasmoacanthoma, which remains an underreported disease in the dermatology literature. <![CDATA[Porokeratosis of Mibelli in an HIV-positive patient]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100131&lng=en&nrm=iso&tlng=en Abstract Porokeratosis represents a group of disorders of epidermal keratinization that are characterized by one or more annular plaques surrounded by a histologically distinctive hyperkeratotic ridge-like border called the cornoid lamella. Many studies showed that organ transplantation and immunosuppression were associated in a significant number of cases. Furthermore, an association with squamous cell carcinoma and basal cell carcinoma has been noted in all variants of porokeratosis. The rarity of this disorder and its atypical clinical presentation – a single lesion on the thumb of an HIV-positive male patient – motivated this report. <![CDATA[Porokeratosis ptychotropica]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100134&lng=en&nrm=iso&tlng=en Abstract Porokeratosis is a skin disorder clinically characterized by annular plaques with keratotic borders resembling the Great Wall of China and histopathologically by cornoid lamellae. The disease has several clinical variants. Porokeratosis ptychotropica, which has recently become part of these variants, is quite rare and little known. The entity is characterized by verrucous plaques – which may resemble a psoriasis plaque – that affect the regions of the buttocks, most commonly the gluteal cleft, with or without extremity involvement. Itching is often present. We report a rare case of porokeratosis ptychotropica and highlight its unusual manifestation (single plaque), the first case reported in the Brazilian literature. <![CDATA[Paradoxical psoriasis after the use of anti-TNF in a patient with rheumatoid arthritis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100137&lng=en&nrm=iso&tlng=en Abstract The use of tumor necrosis factor antagonists (anti-TNF) has become a usual practice to treat various inflammatory diseases. Although indicated for the treatment of psoriasis, anti-TNF may paradoxically trigger a psoriasiform condition. We present a case of a female patient who, during the use of infliximab for rheumatoid arthritis, developed psoriasis. In an attempt to switch anti-TNF class, we observed a cumulative worsening of the lesions requiring suspension of the immunobiological agent and the introduction of other drugs for clinical control. The therapeutic challenge of this paradoxical form of psoriasis is the focus of our discussion. The use of another anti-TNF in these patients is a matter of debate among experts. <![CDATA[Paramedian forehead flap combined with hinge flap for nasal tip reconstruction]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100140&lng=en&nrm=iso&tlng=en Abstract The paramedian forehead flap is a great option for restoration of complex nasal defects. For full-thickness defects, it may be used alone or in combination with other methods. We present a patient with a basal cell carcinoma on the distal nose treated by Mohs micrographic surgery, and a resulting full-thickness defect repaired with paramedian forehead flap combined with a hinge flap. For optimal results with the paramedian forehead flap, adequate surgical planning, patient orientation and meticulous surgical technique are imperative. <![CDATA[Staged retroauricular flap for helical reconstruction after Mohs micrographic surgery]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100144&lng=en&nrm=iso&tlng=en Abstract Staged retroauricular flap is a great option for full-thickness defects along the helical rim and antihelix. Donor site consists of the posterior ear, postauricular sulcus and mastoid area. The advantages of this flap include hidden donor scar, donor tissue similarity and rich vascularity. We present a case of collision tumor on the left helix treated with Mohs micrographic surgery and the resulting full-thickness defect repaired with a staged retroauricular flap. This flap is an effective technique for full-thickness helical defect repair with relatively little operative morbidity. High esthetic and functional results may be obtained restoring the ear size and shape. <![CDATA[Early malignant syphilis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100148&lng=en&nrm=iso&tlng=en Abstract Early malignant syphilis is a rare and severe variant of secondary syphilis. It is clinically characterized by lesions, which can suppurate and be accompanied by systemic symptoms such as high fever, asthenia, myalgia, and torpor state. We report a diabetic patient with characteristic features of the disease showing favorable evolution of the lesions after appropriate treatment. <![CDATA[Rosacea fulminans: unusual clinical presentation of rosacea]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100151&lng=en&nrm=iso&tlng=en Abstract Rosacea fulminans or pyoderma faciale is a rare cutaneous disorder that usually affects women usually between the ages of 15-46. The disease is characterized by sudden onset of papules, pustules, cysts, and painful coalescing nodules with red-cyanotic centrofacial erythema. Although its etiology remains unknown, hormonal, immunological, and vascular factors have been reported. Early diagnosis and prompt treatment should minimize unsightly scars. We report a case of a 33-year-old female patient treated with traditional doses of doxycycline, with improvement of the lesions and regression of the condition in two months. <![CDATA[Histiocytoid Sweet's syndrome presenting with annular erythematous plaques]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100154&lng=en&nrm=iso&tlng=en Abstract Histiocytoid Sweet's Syndrome is a rare inflammatory disease described in 2005 as a variant of the classical Sweet's Syndrome (SS). Histopathologically, the dermal inflammatory infiltrate is composed mainly of mononuclear cells that have a histiocytic appearance and represent immature myeloid cells. We describe a case of Histiocytoid Sweet's Syndrome in an 18-year-old man. Although this patient had clinical manifestations compatible with SS, the cutaneous lesions consisted of erythematous annular plaques, which are not typical for this entity and have not been described in histiocytic form so far. The histiocytic subtype was confirmed by histopathological analysis that showed positivity for myeloperoxidase in multiple cells with histiocytic appearance. <![CDATA[Morbihan syndrome: a case report and literature review]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100157&lng=en&nrm=iso&tlng=en Abstract Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known. It is clinically characterized by the slow appearance of erythema and solid edemas on the upper portion of the face, with accentuation in the periorbital region, forehead, glabella, nose, and cheeks. We report the case of a patient presented with edema on the upper eyelid for a year. These findings suggested the diagnosis of Morbihan syndrome. We aim to report a rare, particularly refractory and chronic form of rosacea, which has received little attention in the literature. <![CDATA[Oculoectodermal syndrome: twentieth described case with new manifestations]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100160&lng=en&nrm=iso&tlng=en Abstract Oculoectodermal syndrome is a rare disease characterized by the association of aplasia cutis congenita, epibulbar dermoids, and other abnormalities. This report describes the twentieth case of the disease. We report a 4-year-old female child who presented with the classical features of the syndrome: aplasia cutis congenita and epibulbar dermoids. Our case expands the clinical spectrum of the disease to include: diffuse hyperpigmentation (some following the Blaschko´s lines); hypopigmented skin areas on the trunk; arachnoid cyst on the right fronto-parietal border; rounded left side of the hippocampus; and dermoid cyst underlying the bulb-medullary transition. Our patient also reported infantile hemangioma on the right wrist and verrucous hemangioma on the left leg, the latter not previously described in the literature. <![CDATA[Gianotti-Crosti syndrome: a case report of a teenager]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100163&lng=en&nrm=iso&tlng=en Abstract Gianotti-Crosti syndrome is a rare disease characterized by acral papular eruption with symmetrical distribution. It is a benign and self-limited disease; the symptoms disappear after two to eight weeks, without recurrences or scars. Skin lesions are usually asymptomatic. Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. Diagnosis is eminently clinical, and this disease is associated with viral infections. Due to its rarity and low occurrence in adolescents and adults, we report a case of Gianotti-Crosti syndrome of a teenager. <![CDATA[Squamous cell carcinoma arising in a multiple verrucous epidermal nevus]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100166&lng=en&nrm=iso&tlng=en Abstract Verrucous epidermal nevi are hamartomatous lesions of the epidermis that, unlike other epidermal nevi (such as sebaceous nevus or nevus comedonicus), are rarely associated with malignant neoplasms. The majority of squamous cell carcinoma develop in linear or multiple epidermal nevus and rarely in solitary epidermal nevus. In general, the prognosis is favorable. We report a case of well-differentiated invasive squamous cell carcinoma arising from a multiple verrucous epidermal nevus. Although there is no consensus on prophylactic removal of epidermal nevus, its removal and biopsy should be considered if changes occur. <![CDATA[Symmetrical peripheral gangrene complicating ventricular pseudoaneurysm: a report of an unusual case and a brief review of the literature]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100169&lng=en&nrm=iso&tlng=en Abstract Symmetrical peripheral gangrene is an ischemic necrosis simultaneously involving the distal portions of two or more extremities without any proximal arterial obstruction or vasculitis. It may occur as a result of a large number of infectious and non-infectious causes. A few cases of symmetrical peripheral gangrene associated with cardiac disease have been described in the literature. We describe a case of symmetrical peripheral gangrene complicating ventricular pseudoaneurysm, probably a hitherto unreported occurrence. In this report, we sought to emphasize the importance of cardiac evaluation while dealing with a case of symmetrical peripheral gangrene.