Scielo RSS <![CDATA[Anais Brasileiros de Dermatologia]]> http://www.scielo.br/rss.php?pid=0365-059620170011&lang=es vol. 92 num. 5 lang. es <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Acne fulminans]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100008&lng=es&nrm=iso&tlng=es Abstract: Acne fulminans is a rare and severe variant of acne. In Brazilian medical journals, cases are infrequently reported, confirming its rarity. We followed five young male patients with this severe variant of cutaneous lesions, accompanied by also severe systemic symptoms: fever, anorexia, weight loss, and arthralgia. All had a good response to corticosteroids (prednisone), but had significant scarring. <![CDATA[Acquired lymphangiomas mimicking multiple hallux warts]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100011&lng=es&nrm=iso&tlng=es Abstract: Lymphangioma is an uncommon benign vascular tumour that involves lymphatic vessels. It can be acquired or, most frequently, congenital. The acquired form presents with dilated lymphatic channels due to an obstruction. These lesions have no risk of malignant transformation, but they have a high rate of recurrence whether removed. We present a case of a 52-year-old woman with acquired lymphangiomas mimicking warts. She came to our observation for some keratotic lesions on her feet. Clinically, we found three warts on the sole of her left foot, but we also noticed the presence of swelling and papillomatous wart-like papules on both halluces. The hallux papules were studied by performing an excisional biopsy and were found to be lymphangiomas. <![CDATA[Acute renal failure in a patient with epidermolysis bullosa acquisita]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100014&lng=es&nrm=iso&tlng=es Abstract: Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure’s pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may be another cause of prerenal kidney failure. Further studies are needed to verify our hypothesis. <![CDATA[Ashy dermatosis with involvement of mucous membranes]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100017&lng=es&nrm=iso&tlng=es Abstract: Ashy dermatosis is a rare condition, of unknown aetiology, in which mucous membranes are typically spared. The authors report the case of a 57-year-old female with a history of asymptomatic gray-bluish macules located on the trunk and oral mucosa. There were no relief changes on examination. Skin biopsies from the oral mucosa and trunk were performed and both were compatible with ashy dermatosis. The patient started treatment with oral clofazimine but due to the absence of clinical improvement the drug was discontinued three months later. This case report illustrates an atypical case of ashy dermatosis owing to the involvement of mucous membranes, which is rarely described in the literature. <![CDATA[Association of amyloidosis cutis dyschromica and familial Mediterranean fever]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100021&lng=es&nrm=iso&tlng=es Abstract: Amyloidosis cutis dyschromica is a rare type of primary cutaneous amyloidosis characterized by reticulate hyper-pigmentation with discrete hypopigmented macules. Up to date, about 50 cases of amyloidosis cutis dyschromica have been reported and the majority are familial cases of Asian ethnicity. Various diseases, particularly autoimmune diseases such as systemic sclerosis and systemic lupus erythematosus, have been associated with amyloidosis cutis dyschromica. Herein, we report a case of amyloidosis cutis dyschromica accompanying familial Mediterranean fever with a delayed diagnosis of 40 years. To the best of our knowledge, this is the first report of the association of amyloidosis cutis dyschromica and familial mediterranean fever. <![CDATA[Atypical Kaposi's sarcoma in young inmunocompetent patient]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100024&lng=es&nrm=iso&tlng=es Abstract: Kaposi´s sarcoma is a rare tumor associated with human herpes virus 8 (HHV-8) infection. Four main clinical subtypes have been described. This study reports on a form of KS in an HIV negative and immunocompetent middle-aged man. The only remarkable factor is that he has sex with other men. This form of Kaposi´s sarcoma is rare. It occurs more in younger patients than in the classic form, is limited to the skin, and is associated with a good prognosis. The means of transmission of the virus is through saliva in oroanal or orogenital sexual practices. Mechanisms of tumor development are still not well known. Given the possible increased number of this variant, it would be interesting to extend this study. <![CDATA[Bilateral clear cell acanthoma of the areola and nipple: good response to topical corticosteroids]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100027&lng=es&nrm=iso&tlng=es Abstract: Clear cell acanthoma is a rare, epidermal tumor not common in the area of the nipples; indeed, the literature describes only 8 cases, all showing unilateral presentation. We here report the first case of bilateral clear cell acanthoma with good response to topical corticosteroids. Case report: A sixteen-year old girl presented with 2 excrescent, fleshy, and exudative tumor masses in both nipples and areola mammae. A biopsy was conducted and confirmed clear cell acanthoma histopathologically. Treatment with strong corticosteroids resulted in rapid improvement and resolution. After one year of follow-up, the patient developed atopic dermatitis. Discussion: We describe the first case of bilateral clear cell acanthoma localized in the nipple/areola that resolved with powerful corticosteroids, suggesting a reactive etiology of the lesion. <![CDATA[Blue nevus with satellitosis: case report and literature review]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100030&lng=es&nrm=iso&tlng=es Abstract: Blue nevus is a benign melanocytic lesion, typically asymptomatic and of unknown etiology. Many histological subtypes are recognized, the most commons being: common blue nevus, cellular blue nevus, and combined blue nevus. New rare variants have been described in the literature, with emphasis on eruptive blue nevus, plaque, agminate, linear, with satellitosis, disseminated, familial and targetoid. The diagnosis of blue nevus usually presents no difficulties, however, the presence of structures such as irregular edges or satellitosis, are highly suggestive of malignancy, and the differential diagnosis with malignant blue nevus and melanoma with peripheral spread should be considered. We report a case of blue nevus with satellitosis in a 15-year-old female patient. <![CDATA[Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100034&lng=es&nrm=iso&tlng=es Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia. <![CDATA[Bullous systemic lupus erythematosus in a 10-year-old child]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100037&lng=es&nrm=iso&tlng=es Abstract: Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal blistering disease, with few cases described in childhood. It has different clinical-pathological features. We report a case of BSLE in a 10-year-old child with systemic lupus erythematosus, treated with prednisone and hydroxychloroquine. There was complete remission with dapsone, with no recurrence of skin lesions throughout one year of follow-up. We highlight the rarity and early age of occurrence. <![CDATA[Congenital Langerhans cell histiocytosis: a good prognosis disease?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100040&lng=es&nrm=iso&tlng=es Abstract: Langerhans cell histiocytosis is rare and more frequent in children. The skin is affected in 50% of the cases and is the only site in 10%. Its course varies from self-limited and localized forms to severe multisystemic forms. Congenital cases are usually exclusively cutaneous and self-limited, with spontaneous remission in months. This study presents a rare congenital case, initially restricted to the skin, with subsequent dissemination and fatal outcome. A male newborn presented congenital disseminated erythematous scaly lesions. The biopsy was conclusive for Langerhans cell histiocytosis. The patient evolved into the multisystemic form in weeks, when chemotherapy was started, according to the LCH-2009 protocol; however, the patient was refractory to treatment and died. <![CDATA[Congenital plaque-like glomangioma: report of two cases]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100043&lng=es&nrm=iso&tlng=es Abstract: Glomus tumors are rare hamartomas that originate from the glomus body. These tumors can be divided into solitary and multiple, the latter also known as glomangioma. We report the case of two patients with a rare variety of glomangioma called congenital plaque-like glomangioma. It presents as numerous red to bluish compressible papules, that increase in size in proportion with the weight and height growth of the child. Diagnostic confirmation is with histopathology and the treatment is surgical. <![CDATA[Cutaneous metastases in a patient with no previous diagnosis of cancer: diagnostic challenge]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100047&lng=es&nrm=iso&tlng=es Abstract: On rare occasions, skin lesions are the first local of metastatic manifestation of internal malignancies. In case of no previous diagnosis of these tumors, the approach of suspicious skin lesions becomes a challenge, especially in differentiating cutaneous metastases and adnexal primary neoplasms. Currently, besides epidemiologic, dermoscopic and histopathologic aspects, the evaluation also integrates immunohistochemical exams and cell markers such as p40 and p63, highly specific for skin metastases. This article describes the case of cutaneous metastases as the sole obvious sign of breast cancer in a previously asymptomatic woman. The diagnosis was made by the finding of neoplastic cells in the dermis and immunohistochemistry compatible with ductal carcinoma. <![CDATA[Cutaneous myeloid sarcoma associated with chronic myeloid leukemia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100050&lng=es&nrm=iso&tlng=es Abstract: Myeloid sarcoma is an extramedullary tumor of malignant myeloid cells often associated with acute myeloid leukemia, chronic myeloproliferative disorders and myelodysplastic syndromes. The skin is one of the most commonly affected sites. We report a rare case of cutaneous myeloid sarcoma associated with chronic myeloid leukemia. <![CDATA[Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100053&lng=es&nrm=iso&tlng=es Abstract: A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity. <![CDATA[Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100056&lng=es&nrm=iso&tlng=es Abstract Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. <![CDATA[Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100059&lng=es&nrm=iso&tlng=es Abstract Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement. <![CDATA[Diffuse cutaneous melanosis: rare complication of metastatic melanoma]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100062&lng=es&nrm=iso&tlng=es Abstract Diffuse cutaneous melanosis is a rare complication of metastatic melanoma related to a worse prognosis. There are few cases reported in the literature. Its pathogenesis has not been completely elucidated, although studies have suggested certain mechanisms for its occurrence. It is clinically manifested as a blue-gray discoloration of the skin and mucous membranes in a cephalo caudal progression and usually associated with melanuria. Skin and mucosa histopathology reveals only the presence of melanophages in the dermis, mainly perivascular, and free interstitial melanin. We report the case of a 68-year-old male with metastatic melanoma, diffuse hyperpigmentation of the skin and melanuria. <![CDATA[Eosinophilic annular erythema in adults: report of two cases and review of the literature]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100065&lng=es&nrm=iso&tlng=es Abstract We herein report two cases of eosinophilic annular erythema in adults, which is rare. In both patients, lesions developed rapidly in few days and were located mainly on the trunk, buttocks, and extremities. Diagnosis was histopathological, with typical features including acute dermal inflammatory infiltrate with abundant eosinophils. One of the patients recurred after treatment on three occasions and finally cured spontaneously. The second patient recurred once and was then successfully treated with topical clobetasol. Clinical and histopathological features of eosinophilic annular erythema in adults have rarely been reported. A review of the literature and discussion of relationship with Wells syndrome are also included. <![CDATA[Disseminated cryptococcosis with skin lesions: report of a case series]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100069&lng=es&nrm=iso&tlng=es Abstract Cryptococcosis is a common fungal infection in immunocompromised patients, caused by genus Cryptococcus, presenting with meningitis, pneumonia, and skin lesions. Cutaneous presentation can be varied, but specifically in solid organ transplant recipients (iatrogenically immunocompromised), cryptococcosis should always be considered in the differential diagnosis of cellulitis-like lesions, since the delay in diagnosis leads to worse prognosis and fatal outcome. We report four cases of cryptococcosis with cutaneous manifestation not only for its rarity, but also to emphasize the important role of the dermatologist in the diagnosis of this disease. <![CDATA[Folliculotropic mycosis fungoides: challenging clinical, histopathological and immunohistochemical diagnosis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100073&lng=es&nrm=iso&tlng=es Abstract Mycosis fungoides is a cutaneous T-cell lymphoma with various clinical and pathological presentations. Early lesions are nonspecific, which hinders early diagnosis. The folliculotropic subtype is manifested as acneiform lesions, follicular papules or erythematous plaques mainly on the face, neck and upper trunk. Histopathology shows dense lymphocytic infiltrate surrounding and infiltrating the hair follicles. A case of difficult histopathological diagnosis with florid and unusual skin lesions mainly on the face is reported. <![CDATA[Frontal fibrosing alopecia and extrafacial lichen planus pigmentosum in a caucasian woman]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100076&lng=es&nrm=iso&tlng=es Abstract We present the case of a 72-year-old Caucasian woman with frontal fibrosing alopecia of one year’s duration. Eighteen months later, she presented with sudden hyperpigmentation on the submental area, neck and upper chest. Histopathological examination was consistent with lichen planus pigmentosus. This is the report of a unique case of a FFA and extrafacial lichen planus pigmentosus. <![CDATA[Frontal fibrosing alopecia and lichen planus pigmentosus: diagnosis and therapeutic challenge]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100079&lng=es&nrm=iso&tlng=es Abstract Frontal fibrosing alopecia is a variant of lichen planopilaris with marginal progressive hair loss on the scalp, eyebrows and axillae. We report a case of frontal fibrosing alopecia and lichen planus pigmentosus in a postmenopausal woman, that started with alopecia on the eyebrows and then on the frontoparietal region, with periocular and cervical hyperpigmentation of difficult management. The condition was controlled with systemic corticosteroid therapy and finasteride. Lichen planus pigmentosus is an uncommon variant of lichen planus frequently associated with frontal fibrosing alopecia in darker phototipes. It should be considered in patients affected by scarring alopecia with a pattern of lichen planopilaris and areas of skin hyperpigmentation revealing perifollicular hyperpigmentation refractory to multiple treatments. This case illustrates diagnostic and therapeutic challenge in face of scarring alopecia and perifollicular hyperpigmentation. <![CDATA[Granuloma annulare treated with narrowband UVB phototherapy]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100082&lng=es&nrm=iso&tlng=es Abstract Granuloma annulare is a benign cutaneous inflammatory disease, whose lesions have spontaneous improvement in two years in 50% of cases, but there is recurrence in 40% of patients. Treatment may be topical, intralesional or systemic. The use of phototherapy with narrowband UVB is highlighted, whose mechanism of action in this disease is still unclear, probably related to the inhibition of T lymphocytes. Herein, a case of a disseminated granuloma annulare of difficult therapeutic management is described. It was treated with narrowband UVB phototherapy twice a week for six months, with good clinical improvement, being a good low-risk therapeutic option and that, in this case, provided quick and satisfactory response. <![CDATA[Guillain-Barré syndrome in a patient on adalimumab for the treatment of psoriasis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100085&lng=es&nrm=iso&tlng=es Abstract The use of TNF-α inhibitors for the treatment of moderate to severe psoriasis and psoriatic arthritis is increasingly more frequent. The authors report a case of Guillain-Barré syndrome as a late manifestation of the treatment with adalimumab. Although unusual, this is relevant for professionals who prescribe biologic drugs. We also stress the importance of investigating the past and family medical history regarding demyelinating diseases before starting treatment. <![CDATA[Hereditary leiomyomatosis and renal cell carcinoma syndrome: a case report and implications of early onset]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100088&lng=es&nrm=iso&tlng=es Abstract Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is an autosomal dominant manifestation of cutaneous and uterine leiomyomas together with renal cancer due to autosomal dominant germline mutations of fumarate hydratase gene. A twenty-year-old female patient presented with type-II segmental piloleiomyoma and increased menstruation due to uterine leiomyomas, with a history of bilateral nephrectomy performed at 13 and 16 years of age for type 2 papillary renal cell carcinoma. This case represents one of the very early onsets of hereditary leiomyomatosis and renal cell carcinoma syndrome. As genetic anticipation for renal cancer is a well-documented entity for HLRCC syndrome, early recognition is crucial for both the patient and her family in order to provide appropriate counseling and initiation of surveillance. <![CDATA[Hyperpigmentation as a peculiar presentation of mycosis fungoides]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100092&lng=es&nrm=iso&tlng=es Abstract Hyperpigmented mycosis fungoides is an extremely rare subtype of mycosis fungoides. It presents as multiple pigmented macules and patches without poikilodermatous changes and characterized by a CD8+ phenotype on immunohistochemistry. This report describes a typical case of hyperpigmented mycosis fungoides in a 62-year-old woman, who presented with a 7-year history of multiple hyperpigmented macules and patches on the trunk and right leg with progression over this half a year. Histology and immunohistochemical staining of skin samples confirmed the diagnosis of mycosis fungoides. She received psoralen plus ultraviolet A (PUVA) therapy. After an 8-week treatment, the erythematous changes cleared without recurrence during a 6-month follow-up period. An intractable hyperpigmented patch should raise the clinical suspicion of mycosis fungoides with sequential skin biopsy. <![CDATA[Kissing nevus of the penis: a case report and dermatoscopic findings]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100095&lng=es&nrm=iso&tlng=es Abstract Divided nevus, also known as kissing nevus, is a rare variant of congenital melanocytic nevi in which there are two adjacent nevi in areas of the body that undergo embryonic cleavage. The original description of this type of lesion was on the eyelid. The location on the penis is even rarer, with only 17 case reports in the literature so far, and only one of them described the dermoscopic findings. We report the case of a patient with divided nevus of the penis and its clinical, dermoscopic and histopathological features. <![CDATA[Lymphocytoma cutis on the inguinal region: report of a rare case of benign lymphoproliferative disorder]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100098&lng=es&nrm=iso&tlng=es Abstract Lymphocytoma cutis, or benign reactive lymphoid hyperplasia, is an inflammatory skin lesion that mimics clinically and histologically malignant lymphoma. Most cases are idiopathic, but they may also be triggered by multiple factors, such as insect bites, tattoos, injections and herpes zoster. Clinically, the lesions are erythematous, soft papules, plaques or nodules, usually located on the upper limbs and face. The diagnosis is mainly based on histopathology and immunohistochemistry. Corticosteroid injections, cryosurgery, PUVA therapy, radiotherapy and surgery can be therapeutic options in cases requiring immediate treatment. To demonstrate an atypical presentation of this tumor, a case lymphocytoma skin on the groin will be reported, describing its diagnosis and treatment. <![CDATA[Melanoma <em>in situ</em> and syringoma: a rare collision tumor. Clinical-pathological report of a case]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100101&lng=es&nrm=iso&tlng=es Abstract Collision or contiguous tumors, defined as two or more distinct tumors occurring at one site, are often an unexpected finding and may represent a diagnostic challenge, as clinical and histological presentations do not always coincide. Various combinations of collision tumors have been described with respect to melanocytic lesions, with the most frequently reported being the combination of nevus and basal cell carcinoma. We present an unusual case on the nose involving a melanoma in situ and a clinically-inapparent syringoma, which, to the best of our knowledge, is the first report of this combination. <![CDATA[Metastatic Crohn’s disease despite infliximab therapy]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100104&lng=es&nrm=iso&tlng=es Abstract Metastatic Crohn’s disease is a rare extraintestinal manifestation of Crohn’s disease. It is characterized by polymorphic skin lesions formed by non-caseating granulomas located on anatomical sites distant from the gastrointestinal tract. We report a rare case of metastatic Crohn’s disease, simultaneously displaying multiple clinically heterogeneous cutaneous lesions, in a patient with previously diagnosed Crohn’s disease in remission due to anti-TNF-α use. This case highlights the need for high clinical suspicion and early biopsy in the setting of a patient with Crohn’s disease and persistent skin lesions, even under biologic therapy. Furthermore, it reinforces the need of monitoring of the serum level of infliximab, increasing the dose in case it is low or undetectable. <![CDATA[Oral manifestation of histoplasmosis on the palate]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100107&lng=es&nrm=iso&tlng=es Abstract This case report describes an uncommon manifestation of histoplasmosis on the soft palate. The importance of appropriate treatment and follow-up in complex cases is emphasized, especially in patients with chronic diseases. Oral lesions may occur as multiple, granular and painful ulcers, as well as verrucous growths. Lesions may also be deep, with infiltrative edges and erythematous or with white areas, accompanied by local lymphadenopathy, resembling a carcinoma on clinical examination. In this sense, a fast and accurate diagnosis is essential to the success of treatment of oral histoplasmosis. <![CDATA[Oral plasmablastic lymphoma as the first manifestation of AIDS]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100110&lng=es&nrm=iso&tlng=es Abstract Plasmablastic lymphoma is a non-Hodgkin lymphoma characterized by its plasmacytic differentiation and predilection for the oral cavity. It is among the lymphomas most commonly associated with AIDS. This report details a case of a HIV-positive patient with a 1-month history of an exophytic mass in the gingival area of the upper left quadrant. The diagnosis of plasmablastic lymphoma was made based on its histopathological and immunophenotypical features. She was treated with chemotherapy followed by autologous hematopoietic stem cell transplantation. Despite complete resolution of the lesion, the patient died of cardiorespiratory arrest. This case illustrates plasmablastic lymphoma as the first clinical manifestation of AIDS, highlighting the importance of differentiating between a potentially malignant lesion and other pathologic processes. <![CDATA[Osteoma cutis: rare painful tumor in atypical location]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100113&lng=es&nrm=iso&tlng=es Abstract Osteoma cutis or cutaneous ossification is a rare entity characterized by the formation of bone in the skin. We present an isolated primary osteoma cutis located on the palm, an atypical location. <![CDATA[Pemphigus foliaceus with pustular presentation in a patient with psoriasis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100115&lng=es&nrm=iso&tlng=es Abstract Pemphigus foliaceus is a chronic autoimmune disease of the skin, clinically characterized by scaly and crusty cutaneous erosions involving the seborrheic areas. The patient can eventually become erythrodermic. There are reports of atypical cases of pemphigus foliaceus with pustules and neutrophils, and clinical differentiation from generalized pustular psoriasis of von Zumbusch is difficult. We report the case of a 55-year-old man with a history of psoriasis vulgaris that has developed pemphigus foliaceus with pustules, triggered by withdrawal of systemic corticosteroids. This is the first report associating this atypical form of pemphigus with psoriasis, suggesting that an overlap with generalized pustular psoriasis can occur. <![CDATA[Periorbital leiomyosarcoma treated by means of conventional surgery and frontal myocutaneous flap]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100118&lng=es&nrm=iso&tlng=es Abstract Cutaneous leiomyosarcoma is a rare neoplasia, and its periorbital presentation is rather uncommon. We present a case of a male patient who was surgically treated, with the reconstruction performed with a frontal myocutaneous flap. The patient has been followed up for one year with adequate oncologic control and good aesthetic outcome. <![CDATA[Porokeratotic eccrine and hair follicle nevus: a report of two cases and review of the literature]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100121&lng=es&nrm=iso&tlng=es Abstract: Porokeratotic eccrine and hair follicle nevus is a very rare non-hereditary disorder of keratinization with eccrine and hair follicle involvement with only 9 cases described in the literature. In 2009 the term porokeratotic anexial ostial nevus was proposed to comprehend porokeratotic eccrine and hair follicle nevus and a related and more common process without follicular involvement: porokeratotic eccrine ostial and dermal duct nevus Recent findings suggest that both entities may be produced by a mutation in GJB2 gene, which is associated to KID syndrome. Herein we report 2 cases of porokeratotic eccrine and hair follicle nevus and review the existing cases in the Spanish and English literature. <![CDATA[Pretibial dystrophic epidermolysis bullosa]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100126&lng=es&nrm=iso&tlng=es Abstract Epidermolysis bullosa is a group of mechano-bullous genetic disorders caused by mutations in the genes encoding structural proteins of the skin. Dystrophic epidermolysis bullosa is caused by mutations in the COL7A1 gene encoding collagen VII, the main constituent of anchoring fibrils. In this group, there are autosomal dominant and recessive inheritances. The pre-tibial form is characterized by the presence of blisters, milia, atrophic scars and lesions similar to lichen planus. The diagnosis is clinical and laboratory and subtypes are distinguished by means of immunohistochemical and ultrastructural studies, in addition to genetic differentiation. Electron microscopy and immunomapping are used in the diagnosis. <![CDATA[Pyoderma gangrenosum associated with left iliac vein compression syndrome: presentation of difficult diagnosis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100129&lng=es&nrm=iso&tlng=es Abstract: Pyoderma gangrenosum is a rare neutrophilic dermatosis of unknown etiology, of which the most frequent clinical manifestations are ulcers. The diagnosis difficulty is, among other things, to rule out other causes of ulcers, since it is considered a diagnosis of exclusion. Skin ulcerations may also occur in the iliac vein compression syndrome, which, like pyoderma gangrenosum, mainly affects young women. Because they have such similar characteristics, the presence of vascular disease may hinder the diagnosis of concurrent pyoderma gangrenosum. Because of the clinical relevance of ulcerated lesions and scars, the early diagnosis and treatment of this condition is considered extremely important. We report a case in which the two diseases were associated, hampering the diagnosis of pyoderma gangrenosum. <![CDATA[Reconstruction of surgical defect of the dorsum of the hand]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100132&lng=es&nrm=iso&tlng=es Abstract In some cases, keratoacanthomas can be indistinguishable from squamous cell carcinoma, making the gold standard treatment surgical excision with histopathological evaluation. The surgical defects created on the back of the hand can be challenging to repair. We describe the use of bilateral rotation flap as an option in this area, and discuss the indications and contraindications of some of the possible surgical techniques for reconstruction of the dorsum of the hand. <![CDATA[Red nodule on the face with “spontaneous” regression]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100135&lng=es&nrm=iso&tlng=es Abstract Pseudolymphomatous folliculitis is a rare entity included among the cutaneous pseudolymphomas. A 32-year-old man, with an unremarkable medical history, presented with a two-month history of an asymptomatic solitary nodule on his left cheek. Histopathological examination demonstrated a dense nodular and diffuse dermal lymphocytic infiltrate with numerous histiocytes and dendritic cells that surrounded hypertrophic hair follicles. Pseudolymphomatous folliculitis commonly presents in the fourth decade of life, with no sex predominance, as an asymptomatic, rapidly growing and solitary red dome-shaped nodule on the face. It has a benign clinical course as the lesions usually resolve with surgical excision or regress spontaneously after incisional biopsy. Although there is no report of pseudolymphomatous folliculitis progressing into lymphoma in the literature, follow-up of these patients is recommended. <![CDATA[Sarcoidal granulomatous reaction due to tattoos: report of two cases]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100138&lng=es&nrm=iso&tlng=es Abstract Numerous infectious, inflammatory and neoplastic complications secondary to tattoo placement have been reported in the literature. Within inflammatory complications sarcoidal granulomatous reactions have been described. We report two cases, a 55-year-old woman with yellowish infiltrated plaques on bilateral ciliary region, 16 years after the placement of a permanent tattoo in the eyebrows, and a 20-year-old tattoo artist who developed orange papules on 3 of his tattoos. Histopathology in both cases confirmed diagnosis of sarcoidal granulomatous reaction due to tattoo pigment. <![CDATA[“String of pearls pattern”: report of three cases of non clear-cell acanthoma]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100142&lng=es&nrm=iso&tlng=es Abstract The coiled and dotted vessels in a serpiginous arrangement or “string of pearls” is considered a classical vascular pattern associated with clear cell acanthoma. We present three cases of epidermal tumors different from clear cell acanthoma that have the same “string of pearls” vascular pattern. Even though most authors keep considering the “string of pearls” vascular pattern an almost pathognomonic sign of clear-cell acanthoma, the cases presented here suggest that some other epidermal tumors can also show this pattern. <![CDATA[The importance of direct immunofluorescence in pemphigus herpetiformis diagnosis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100145&lng=es&nrm=iso&tlng=es Abstract Pemphigus herpetiformis is an autoimmune bullous disease, that combines clinical features of dermatitis herpetiformis and linear IgA bullous dermatosis and immunological characteristics of pemphigus, which makes this disease peculiar and this diagnosis rarely suspected in the first evaluation of the patient. The reported case is of a patient with clinically bullous disease similar to dermatitis herpetiformis, whose multiple biopsies were inconclusive, and only after direct immunofluorescence with a pemphigus pattern (intraepidermal intercellular pattern) the confirmation of the diagnosis was possible. <![CDATA[Trigeminal trophic syndrome simulating rodent ulcer basal cell carcinoma: a new clinico-dermoscopic approach]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100148&lng=es&nrm=iso&tlng=es Abstract Trigeminal trophic syndrome is an uncommon cause of facial ulcers, that affects the sensitive area of the trigeminal nerve. We present the case of an 84-year-old patient with ulcerated facial trigeminal trophic syndrome, and report the development of a clinico-dermoscopic approach for his clinical examination. The value of this model for the diagnosis of facial ulcers suspected to be a rodent ulcer basal cell carcinoma is suggested. <![CDATA[Tunneled island flaps in facial defects reconstruction]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100151&lng=es&nrm=iso&tlng=es Abstract The reconstruction of facial surgical defects is usually challenging for the dermatologic surgeon. Three different cases of facial defects in which tunneled island flaps were used are reported. In 2 cases, wide defects involving the nasal dorsum and ala were repaired using a nasolabial island flap tunneled through the lateral side of the nose. A tunneled island glabellar flap was used for medial canthus reconstruction in the third case. Despite complex pedicle dissection and frequent trapdoor deformation, tunneled island flaps allow reconstruction of wide defects in a single-staged procedure, camouflaging the scar of the donor area in boundaries of cosmetic units and preserving the facial central symmetry. <![CDATA[Unique autosomal recessive variant of palmoplantar keratoderma associated with hearing loss not caused by known mutations]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100154&lng=es&nrm=iso&tlng=es Abstract Inherited Palmoplantar Keratodermas are rare disorders of genodermatosis that are conventionally regarded as autosomal dominant in inheritance with extensive clinical and genetic heterogeneity. This is the first report of a unique autosomal recessive Inherited Palmoplantar keratoderma -sensorineural hearing loss syndrome which has not been reported before in 3 siblings of a large consanguineous family. The patients presented unique clinical features that were different from other known Inherited Palmoplantar Keratodermas -hearing loss syndromes. Mutations in GJB2 or GJB6 and the mitochondrial A7445G mutation, known to be the major causes of diverse Inherited Palmoplantar Keratodermas -hearing loss syndromes were not detected by Sanger sequencing. Moreover, the pathogenic mutation could not be identified using whole exome sequencing. Other known Inherited Palmoplantar keratoderma syndromes were excluded based on both clinical criteria and genetic analysis. <![CDATA[Usefulness of the subunit principle in nasal reconstruction]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100159&lng=es&nrm=iso&tlng=es Abstract The subunit principle in nasal reconstruction proposed the concept of reconstructing the specific topographic subunits that were identified as dorsum, tip, columella, the paired alae, sidewalls, and soft triangles. In patients with more than 50% of subunit loss, removing the remaining portion of the subunit and reconstructing the entire subunit may yield better results. The placement of incisions along the borders of the subunits minimizes scar lines. Furthermore, as trapdoor contraction occurs, the entire restored subunit bulges in a way that mimics the normal contour of a nasal tip, dorsum or ala. Two cases of nasal reconstruction that followed this important principle are described. <![CDATA[Wooly hair nevus]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100163&lng=es&nrm=iso&tlng=es Abstract Woolly hair nevus is a rare condition characterized by a structural anomaly of the hair, restricted to certain areas of the scalp. The hair becomes coiled and slightly hypopigmented. The term woolly hair refers to changes that affect all the scalp and has a hereditary character. We present a case of woolly hair nevus, that developed at the age of 2 years, associated with dental diastema and verrucous epidermal nevus. <![CDATA[Zoon vulvitis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100166&lng=es&nrm=iso&tlng=es Abstract Zoon vulvitis or vulvitis chronica plasmacellularis is a rare, chronic benign inflammation of the vulvar mucosa, diagnosed histologically, with variable therapeutic responses. It is important to be diagnosed because it mimics the presentation of other genital conditions, such as lichen planus and squamous cell carcinoma, which require specific treatment. We report a case of a female patient with three asymptomatic shallow ulcers on the labia minora. Laboratory tests ruled out infectious diseases and the biopsy was consistent with Zoon Vulvitis.