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Sclerodermatous graft-versus-host disease (GVHD) is one of the many clinicopathological variants of chronic GVHD. One of the rarest forms of this variant is GVHD-associated angiomatosis (GVHD-AA). We describe the case of a 62-year-old male with sclerodermatous GVHD who presented, in consecutive years, two different lesions that showed characteristics of GVHD-AA. The first lesion fitted perfectly with the previously known features of this rare entity. However, the second lesion, was more interesting as the angiomatoid lesion was surrounded by newly appeared adipocytes, something not previously described. The appearance of this peculiar adipose tissue may be explained as related to an important dermal atrophy, as a concomitant appearance of a lipomatous nevus and GVHD-AA or finally, as mature adipose tissue related to a previous inflammatory process, that is lipomatous metaplasia. Both lesions were diagnosed as GVHD-AA, and the second one was considered to be associated with dermal lipomatous metaplasia. We also considered that hypoxia could be related to both lesions. In the present report we review previously published cases of GVHD-AA and discuss the different hypothesis that could explain the appearance of metaplasia associated to the second lesion. This article is protected by copyright. All rights reserved.

Background:Interferon signaling pathway plays a relevant role in autoimmunity. Genetic variants in theinterferon regulatory factor (IRF) 5gene, that encodes the major regulator of the type I interferon induction [1], have been related to the development of several inflammatory diseases [2].Objectives:To determine the influence ofIRF5on Immunoglobulin-A vasculitis (IgAV), an inflammatory vascular disease.Methods:ThreeIRF5polymorphisms (rs2004640, rs2070197 and rs10954213) representative of 3 different haplotype blocks were genotyped in 372 Caucasian patients with IgAV and 876 sex and ethnically matched healthy controls.Results:No statistically significant differences between patients with IgAV and controls were observed when eachIRF5polymorphism was analyzed independently. Similarly, no statistically significant differences between patients with IgAV and controls were found whenIRF5polymorphisms were evaluated combined conforming haplotypes. Additionally, there were no statistically significant differences in genotype, allele and haplotype frequencies ofIRF5when patients with IgAV were stratified according to the age at disease onset or to the presence/absence of gastrointestinal or renal manifestations.Conclusion:Our results do not support an influence ofIRF5on the pathogenesis of IgAV.References:[1]Nat Immunol 2011; 12: 231-8;[2]Arthritis Res Ther 2014; 16: R146.Acknowledgments:This study was supported by European Union FEDER funds and “Fondo de Investigaciones Sanitarias” (grant PI18/00042) from ‘Instituto de Salud Carlos III’ (ISCIII, Health Ministry, Spain). RL-M is a recipient of a Miguel Servet type I programme fellowship from the ISCIII, co-funded by the European Social Fund (ESF, `Investing in your future´) (grant CP16/00033). SR-M is supported by funds of the RETICS Program (RD16/0012/0009) (ISCIII, co-funded by the European Regional Development Fund (ERDF)). VP-C is supported by a pre-doctoral grant from IDIVAL (PREVAL 18/01). LL-G is supported by funds of PI18/00042 (ISCIII, co-funded by ERDF).Disclosure of Interests:Sara Remuzgo Martinez: None declared, Fernanda Genre: None declared, Verónica Pulito-Cueto: None declared, D. Prieto-Peña: None declared, Belén Atienza-Mateo: None declared, Belén Sevilla: None declared, Javier Llorca: None declared, Norberto Ortego: None declared, Leticia Lera-Gómez: None declared, Maite Leonardo: None declared, Ana Peñalba: None declared, María Jesús Cabero: None declared, Luis Martín-Penagos: None declared, Jose Alberto Miranda-Filloy: None declared, Antonio Navas Parejo: None declared, Javier Sanchez Perez: None declared, Maximiliano Aragües: None declared, Esteban Rubio: None declared, MANUEL LEON LUQUE: None declared, Juan María Blanco-Madrigal: None declared, E. Galindez: None declared, Javier Martin Ibanez: None declared, Santos Castañeda: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD and Roche, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Raquel López-Mejías: None declared

Background:Cytokines signaling pathway genes represent a key component of the genetic network implicated in the pathogenesis of Immunoglobulin-A vasculitis (IgAV) [1], an inflammatory vascular pathology.Interleukin (IL)17Ais a genetic risklocusfor autoimmune diseases, such as giant cell arteritis [2] and spondyloarthritis [3].Objectives:To determine the potential influence ofIL17Aon IgAV.Methods:FiveIL17Atag polymorphisms (rs4711998, rs8193036, rs3819024, rs2275913 and rs7747909) were genotyped in 360 Caucasian patients with IgAV and 1,003 sex and ethnically matched healthy controls.Results:No statistically significant differences between patients with IgAV and healthy controls were observed when eachIL17Agenetic variant was analyzed independently. Similarly, no statistically significant differences between patients with IgAV and healthy controls were found when the fiveIL17Apolymorphisms were evaluated combined conforming haplotypes. In addition, there were no statistically significant differences in genotype, allele and haplotype frequencies ofIL17Awhen patients with IgAV were stratified according to the age at disease onset or to the presence/absence of gastrointestinal or renal manifestations.Conclusion:Our results do not support an influence ofIL17Aon the pathogenesis of IgAV.References:[1]Autoimmun Rev 2018; 17: 301-15[2]Ann Rheum Dis 2014; 73: 1742-5[3]Mediators Inflamm 2018; 2018: 1395823.Acknowledgments:This study was supported by European Union FEDER funds and “Fondo de Investigaciones Sanitarias” (grant PI18/00042) from ‘Instituto de Salud Carlos III’ (ISCIII, Health Ministry, Spain). RL-M is a recipient of a Miguel Servet type I programme fellowship from the ISCIII, co-funded by the European Social Fund (ESF, `Investing in your future´) (grant CP16/00033). SR-M is supported by funds of the RETICS Program (RD16/0012/0009) (ISCIII, co-funded by the European Regional Development Fund (ERDF)). VP-C is supported by a pre-doctoral grant from IDIVAL (PREVAL 18/01). LL-G is supported by funds of PI18/00042 (ISCIII, co-funded by ERDF).Disclosure of Interests:Fernanda Genre: None declared, Sara Remuzgo Martinez: None declared, Verónica Pulito-Cueto: None declared, D. Prieto-Peña: None declared, Belén Atienza-Mateo: None declared, Belén Sevilla: None declared, Javier Llorca: None declared, Norberto Ortego: None declared, Leticia Lera-Gómez: None declared, Maite Leonardo: None declared, Ana Peñalba: None declared, María Jesús Cabero: None declared, Luis Martín-Penagos: None declared, Jose Alberto Miranda-Filloy: None declared, Antonio Navas Parejo: None declared, Diego de Argila: None declared, Maximiliano Aragües: None declared, Esteban Rubio-Romero: None declared, MANUEL LEON LUQUE: None declared, Juan María Blanco-Madrigal: None declared, E. Galindez: None declared, Javier Martin Ibanez: None declared, Santos Castañeda: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD and Roche, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Raquel López-Mejías: None declared

Objectives The frequency and clinicopathologic characteristics of cutaneous lesions in sepsis are not well known. This study aimed to analyze cutaneous lesions in bacterial septic vasculopathy. Methods The study population comprised 32 patients with bacterial sepsis, cutaneous lesions, and skin biopsy-proven septic vasculopathy. The clinical and histologic characteristics of the lesions were analyzed. Findings in non-immunosuppressed patients (NISPs) and immunosuppressed patients (ISPs) were compared. Results Nine of 32 patients were immunosuppressed. The foci of sepsis were variable; in 17 patients, the focus was not identified. Although Neisseria meningitidis was the most common causal agent, several microorganisms were identified. Cutaneous manifestations were an early event in 90.6% of patients. The most common skin signs were purpuric lesions and petechiae. Overall mortality was 28.1%; 65.5% of patients survived without sequelae. Skin biopsies showed thrombi in 100% of cases. Other common findings were inflammatory infiltrate, blood extravasation, and epidermal involvement. Bacteria within the vascular wall were seen in 21.9% of cases and fibrinoid necrosis in 25%. A comparison of ISPs with NISPs disclosed that meningococcemia was more common in the latter group, and the presence of pustules was more common in the former. Histopathology testing revealed that fibrinoid necrosis and bacterial invasion of the vessel wall were more common in ISPs than in NISPs. Conclusions Several microorganisms can cause septic vasculopathy. Clinical presentation is variable and does not depend on the microorganism or the pathogenic mechanism. Histopathologically, septic vasculopathy is a thrombo-occlusive vasculopathy with variable morphology. Cutaneous lesions are an early event and allow for rapid diagnosis.

The coexistence of skin-limited Langerhans cell histiocytosis (LCH) and Rosai-Dorfman disease (RDD) is an exceptional finding. The association of lymphomas and histiocytosis is also infrequent. We report the case of a 68-year-old man which presented an exceptional association of cutaneous LCH and RDD and splenic marginal zone lymphoma. He was stable for few years. Suddenly, the patient was admitted into Hematology Department with a remarkable enlargement of spleen and liver without enlargement of lymphadenopathies or skin lesions flare. He died 24 h later despite treatment with systemic chemotherapy combined with prednisone. Pre-mortem biopsy showed infiltration with histiocytic sarcoma. We think that a transdifferentiation phenomenon could explain our case, although we could not show a clonal relationship between the cutaneous and the liver diseases. We also want to pay attention to the fact that a fast transformation to a more aggressive disease can occur long time after the presentation of the first lesion, a problem that stresses the importance of performing a close and permanent follow-up of these patients.

Background The most severe form of cutaneous acute graft-versus-host disease (aGVHD), stage IV, is characterized by the appearance of vesicles and blisters. Objective To describe the clinicopathological characteristics and evolution of stage IV cutaneous aGVHD presented in our hospital. Method Retrospective study. The following criteria for inclusion were applied: (i) patients subjected to allogeneic stem cell transplantation between 1st January 1984 and 31st of December 2006; (ii) development of vesicles and/or blisters; (iii) extracutaneous coincidental aGVHD manifestations; and (iv) presence of histopathological features consistent with aGVHD. Results Fifteen cases (10 females and 5 males) were studied. The mean age was 38.1 years. The lesions appeared after a median interval of 19 days, always following a milder stage of GVHD. Two patterns of clinical evolution were found. Mucosal involvement was observed in nine patients. Nikolsky's sign was positive in eight patients. Nine of the patients had biopsies of the vesiculobullous stage which showed a subepidermal blister with epidermal necrosis and basal vacuolar degeneration. Only two patients survived. Conclusion Stage IV cutaneous aGVHD is a severe and unusual complication after haematopoietic stem cell transplantation. Prognosis is poor with a very high mortality rate, although the cause of death is varied and not strictly linked to the cutaneous disease.

Late reactions to iodinated contrast media are frequent. Cutaneous manifestations are the commonest, in which maculopapular exanthema, a type of cutaneous presentation, is widespread. Controversy exists about the utility of the skin test in the management of these reactions. The aim of this study is to analyse the clinical characteristics, the histopathological findings, and the results of the patch test in patients who developed a late skin reaction (LSR) to the nonionic, dimeric, iodinated contrast media Visipaque®. We retrospectively reviewed the patients with LSR to Visipaque®, seen in the Dermatology Department between 1999 and 2005. A total of 12 patients participated in this study (7 men and 5 women), ages ranging from 39 to 76 years (mean 56). 11 of the patients had significant medical history. All the patients developed a maculopapular exanthema between 2 hr and 3 days after the radiological examination, involving the trunk and proximal limbs, although some of the patients showed involvement of distal areas. The skin biopsy, performed in 6 patients, showed nonspecific findings consistent with drug reaction. In 3 patients, patch tests to Visipaque® and iodixanol were positive. The most frequent manifestation of LSR to iodixanol is a maculopapular exanthema, involving the trunk and the limbs, although distal involvement can be seen. Histopathological findings are nonspecific and cannot be distinguished from other drug reaction. Patch tests have a limited value, and in cases where they were negative, reintroduction of the drug triggered a new LSR.

Leukemia cutis used to be a late manifestation of leukemia. On the other hand, Grover's disease has been described in the setting of cancer. Case Report: A patient diagnosed as having a chronic myelomonocytic leukemia presented with lichenoid, slightly infiltrated plaques on the anterior aspect of his thorax and abdomen and papulovesicles on his back. A skin biopsy showed the co-existence of leukemia cutis, Grover's disease, and syringoma-like features. Discussion: We discuss and comment upon this unusual association.

Background Sweet's syndrome (SS) has been reported in association with many conditions, including malignancy, infections, autoimmune disorders, pregnancy and drugs. Materials and methods We reviewed patients with SS-like lesions on the lymphoedema area seen in our department. Clinical manifestations, histopathologic characteristics, treatment and outcome data were recorded and analysed. Results We report seven women with a history of surgery for breast cancer with axillary lymphadenectomy. Six of them were on tamoxifen. All of them had various lesions consistent with SS localized predominantly on the limb affected by the postmastectomy lymphoedema, and on the ipsilateral chest, trunk and back. One of them presented bullous lesions. Three of the cases underwent spontaneous remission, two resolved with antibiotic therapy, one healed with corticosteroids, and one with corticosteroids plus antibiotic. Conclusions Erythematous tender plaques on the area of postmastectomy lymphoedema could be considered an unusual manifestation of Sweet's syndrome. We have found only three similar cases in the literature. Although it is difficult to elucidate the pathogenesis of this entity, it has been suggested that it could be due to immune surveillance impairment.

Cutaneous pseudolymphomas are inflammatory diseases of the skin that simulate malignant lymphomas in their clinical and/or histological form. Those induced by drugs are infrequent and are characterized by their clinical and histological polymorphism. There is no consensus regarding the definition of these dermatoses, and they can be seen in different forms of presentation. There are two main groups of drug-induced pseudolymphomas: those that clinically and histologically simulate cutaneous lymphomas, and those known as hypersensitivity syndromes. Pseudolymphomas cannot be differentiated from true lymphomas through clinical, pathological or molecular findings. The definitive test for diagnosis is the resolution of the lesions after the medication involved is suspended. We present three cases of carbamazepine-induced cases of pseudolymphomas that histologically simulate mycosis fungoides, with different clinical presentations.

Summary The coexistence of non-Hodgkin lymphoma (NHL) and Hodgkin disease (HD) in the same patient, although previously reported, is very unusual. This situation is extremely rare when the first diagnosis is a cutaneous B NHL, and exceptional if there is no personal background of cytostatic treatment. We report a 44-year-old man who developed cutaneous nodules over a period of two years. A marginal zone cutaneous B-cell lymphoma was diagnosed. On staging investigation a mass in the lingual tonsil was found and excision biopsy showed a classical Hodgkin lymphoma.

Resumen —El metotrexato es un inhibidor competitivo del acido folico e interfiere en la sintesis de ADN. Las reacciones cutaneas por metotrexato son poco frecuentes y muy variadas y pueden o no ser signo de toxicidad. Se presentan 2 pacientes tratados con 35 mg semanales de metotrexato, uno por psoriasis y otro por una artritis reumatoide, que desarrollaron lesiones mucosas y cutaneas relacionados con el farmaco. El paciente con psoriasis presento ademas erosion y sangrado de las placas de psoriasis y toxicidad hepatica y hematologica. Los cambios histologicos sugerian un efecto citotoxico por quimioterapia, y presentaban una discreta dermatitis de interfase vacuolar con marcada dismaduracion epidermica, atipia celular y siringometaplasia escamosa ecrina. En ambos pacientes las lesiones mucocutaneas se interpretan como un signo de toxicidad por el farmaco.

In May 1994, a 40-year-old woman with chronic myeloid leukemia received an allogeneic bone marrow transplant (BMT) from her human leukocyte antigen (HLA) identical sister, after a conditioning regimen with cyclophosphamide and busulfan. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine (CsA) and methotrexate. Facial and palmoplantar erythema and moderate cholestasis developed on day 14 after the BMT. A diagnosis of acute GVHD was made and she was successfully treated with low doses of corticosteroids. On day 150 after the BMT, despite the prophylactic treatment of GVHD with CsA (150 mg/12 h), she developed several burning white plaque-like striae over the buccal mucosa and numerous itching violaceous lichenoid papules on the fingertips. Biopsy specimens obtained from both the skin of the fingertips and the oral mucosa (Fig. 1) revealed patchy to diffuse subepithelial lymphocytic inflammation and necrosis of individual squamous cells, consistent with a diagnosis of chronic lichenoid GVHD. Despite therapy with CsA, topical and systemic corticosteroids (prednisone 60 mg/24 h), the oral lichenoid lesions persisted. On day 750 after the BMT, 2 months after withdrawal of immunosuppressive therapy, she developed several erythematous, pruriginous, and slight indurated lesions over the neck. These lesions coalesced into plaques, adopting a white atrophic-like appearance with follicular plugs similar to lichen sclerosus et atrophicus (Fig. 2). Histopathologic examination showed hyperkeratosis with follicular plugging, atrophy of the stratum Malpighii with hydropic degeneration of the basal cells, homogenization of the collagen, incontinence of the pigment, and a discrete lymphoplasmocytic inflammatory infiltrate in the upper dermis (Fig. 3). Systemic corticosteroid therapy was re-introduced. On day 850 after the BMT, physical examination revealed patchy hyperpigmentation affecting the back and limbs, and diffuse thickening and hardening of the skin of the legs, forearms, and dorsa of the hands, resulting in Figure 1. Diffuse subepithelial lymphocytic inflammation and satellite cell necrosis of squamous cells in oral mucosa (hematoxylin and eosin, ×25) Download figure to PowerPoint Figure 2. Atrophic and confluent plaques with follicular plugs on the neck Download figure to PowerPoint Figure 3. Follicular plugging, atrophy of the stratum Malpighii, hydropic degeneration of basal cells with a split visible at the dermoepidermal junction, and homogenization of the collagen in the upper dermis (hematoxylin and eosin, ×25) Download figure to PowerPoint a slight limitation of movements. Various smooth, shiny, and indurated plaques, characteristic of morphea, were present on the flexural areas of the arms (at vein puncture sites). Lichenoid lesions over the oral mucosa and the atrophic plaques on the neck were still present. No dysphagia was observed. Liver function tests revealed a pattern of cholestasis. Antinuclear antibodies were detected (1 : 80); no antibodies against centromere antigens and DNA topoisomerase I (SCL70) were found. Reduced lacrimal secretion (Schirmer test

Herpes zoster (HZ) occurs with an increased incidence in immunosuppressed patients, in whom it frequently displays atypical clinical presentations. Herpetic syringitis, the involvement of the eccrine epithelium by herpes virus infection, is an infrequently described histologic pattern that has been rarely and almost exclusively reported in HIV-infected patients. We report the case of a woman with Burkitt lymphoma who developed 2 nodular, asymptomatic lesions while receiving treatment with chemotherapy and radiotherapy for her hematological disease. Histology showed viropathic changes in the epithelium of eccrine glands not in the epidermis. PCR was positive for varicella-zoster virus (VZV). Nodular herpes zoster seems to be an exceptional clinical presentation. We report another such case which is, as far as we know, the first report of herpetic syringitis with no concomitant epidermal involvement.

Background and Objective. Papular-purpuric gloves-and-socks syndrome (PPGSS) is a recently described dermatosis in which human parvovirus B19 (HPV B19) has been implicated as etiologic agent; however, it is suspected that PPGSS may be caused by various agents. This study was designed to survey the general characteristics of PPGSS and to determine the role of HPV B19 in its etiology. Methods. We analyzed data from 21 patients and examined serum samples from three new cases for various viruses. Results. The PPGSS displays a striking uniform clinical pattern. Histologic and immunofluorescence findings are nonspecific. Seroconversion of HPV B19 was reported in six cases and confirmed in two of our patients. In only one case was a possible causative role of Coxsackie virus B6 suggested consistently. Conclusions. The PPGSS represents a distinctive dermatosis and a manifestation of HPV B19 infection. Unlike erythema infectiosum, anti-HPV B19 antibodies seem to develop later after onset of the skin eruption and while viremia is still present.

Background. Mixed cryoglobulinemia (MC) is a systemic disorder, characterized by a typical clinical triad: purpura, weakness, and arthralgias, with visceral complications such as liver and renal involvement. The objective was to study the association between hepatitis C virus (HCV) infection and essential mixed cryoglobulinemia (EMC). Patients and Methods. Markers of HCV infection in 11 patients with cryoglobulinemia were examined and hepatitis C virus (HCV) was detected in eight of them. These patients were included in a clinical and histologic study. Anti-HCV antibodies were determined by a second-generation enzyme-linked immunosorbent assay (ELISA-2) in sera and cryoprecipitates. Studies on HCV-RNA were performed by a two-stage polymerase chain reaction (PCR) in the serum. A control group, consisting of 28 patients with other cutaneous disorders, was studied for HCV infection using ELISA-2 and PCR. Results. All patients had liver dysfunction, arthralgias, and purpura. Three patients had involvement of the peripheral nervous system, two had renal involvement, and one patient had Sjogren's syndrome. Cryocrits ranged from 3% to 20%. Six patients had type III cryoglobulinemia and the remaining two had type II. Markers for hepatitis B virus (HBV) were negative in all serum samples. Anti-HCV antibodies and HCV-RNA were positive in the serum of all the cases with MC. Anti-HCV antibodies were positive in all cases except for one of the cryoprecipitates tested. Four patients received recombinant interferon alfa. In two of them, serum aminotransferases became normal and cryoglobulins disappeared. Conclusions. The results strongly suggest that HCV infection is responsible for the cryoglobulinemia and vasculitis in patients with MC and that treatment with interferon alfa is presently the treatment of choice for such patients.

A 28-year-old man had a circumscribed hypertrichotic area on the left lumbar region since birth. A biopsy specimen showed numerous normal-appearing hair follicles and arrector pili muscles. In the subcutaneous layer a mass of dense collagenous connective tissue formed the background in which ill-defined foci of meningothelial cells in nests and pseudovascular spaces were seen. Collagenous structures (pseudopsammoma bodies), some of which showed calcification (psammoma bodies), were also seen. A diagnosis of cutaneous meningioma was made.

Diffuse plane normolipaemic xanthomatosis (DPNX) is a well-defined clinicopathological entity that characteristically presents with yellow-orange plaques in the periorbital areas, the neck, the upper trunk and the flexural folds. DPNX has been reported in association with lymphoproliferative disorders and, occasionally, with miscellaneous, probably coincidental disorders. A case of DPNX in a patient with an IgG lambda monoclonal gammopathy and systemic amyloidosis is reported. Clinical and histopathological findings revealed typical features of DPNX and amyloidosis coexisting in the cutaneous lesions. To our knowledge this is the first reported case showing coexistence of xanthoma and amyloidosis in cutaneous lesions in a patient with a monoclonal gammopathy.

We describe the case of a 10-year-old boy with a porokeratotic eccrine ostial and dermal duct nevus (PEODDN) of late onset. The patient had an 8-year history of multiple keratotic papules on the dorsal surface, and multiple yellowish pitting lesions on the plantar surface of the right foot. Light-microscopic studies of both lesions showed multiple cornoid lamella-like parakeratotic columns, which exclusively arose over eccrine sweat ducts in which the acrosyringium was dilated. Although PEODDN is considered to be a congenital hamartoma of eccrine origin, a review of the literature showed us that the frequency of a late-onset variant may be as high as 26%.

Background: Nephrogenic fibrosing dermopathy (NFD) is a new skin-fibrosing disorder associated with renal dysfunction. It is marked by the acute onset of induration involving the upper and lower limbs, and it is characterized by distinctive histopathologic findings. Methods: We report the case of a patient on hemodialysis who presented initially with the characteristic clinical and pathological features of NFD. The patient progressively developed painful hyperkeratotic spicules on both thighs and bone metaplasia was confirmed. Results: Histological studies were performed at different stages of the disease showing an evolution from the well-known initial phase of NFD, characterized by thickening of the dermis, increased number of fibroblast-like cells, and mucin deposits, to an end stage characterized by the presence of dermal ossification. Conclusions: We report the exceptional finding of bone metaplasia in the setting of a case of NFD.

Different clinical studies have demonstrated flutrimazole's efficacy in the treatment of superficial dermatomycoses when administered either twice daily or once daily for four weeks. The aim of the present study was to compare both dosing schedules for the treatment of superficial dermatomycoses. In this randomized, controlled, double blind study, we included 84 patients suffering superficial dermatophytosis (confirmed by microscopic examination (KOH) and culture) susceptible for topical monotherapy. Forty-one patients received flutrimazole 1% twice daily (TD) and forty-three once daily (OD) for four weeks. The efficacy of treatment was evaluated by clinical and mycological criteria at the end of treatment (D28) and after four weeks without treatment (D58). Clinical and mycological cure rates on D28 were 50% with TD and 65% with OD treatment. Only considering clinical evaluation, clinical cure rates on D28 were 63% (TD) and 70% (OD). Also, clinical and mycological cure rates on D56 were 65% with TD and 72% with OD treatment. Only considering clinical evaluation, clinical cure rates on D56 were of 68% (TD) and 72% (OD). The overall tolerability was similar in both treatment groups. The efficacy assessment at the end of treatment (D28) and four weeks after treatment discontinuation (D56) showed that the OD treatment is not inferior to the TD treatment, with similar clinical and mycological cure rates and clinical cure rates in both cases. The OD administration of flutrimazole cream 1% is the most appropriate dosing schedule as it provides the same efficacy, it improves patient's compliance and the cost per day of treatment.

Atrial myxoma is the most common primary tumour of the heart. Skin manifestations in patients with a cardiac myxoma are frequent and may be due to cutaneous emboli, or may be specific findings as part of more complex syndromes. We present a 33-year-old-man with a history of episodes of pain in both legs and an ischaemic neurological event, who also had episodes of acral papular erythematous lesions on the legs and feet including the soles. The histological finding of dermal vessels occluded by a myxomatous material was the clue to the diagnosis of a cardiac myxoma. The diagnosis of this entity can be very difficult, because of the broad spectrum of clinical features; rarely the skin manifestations lead to the diagnosis of this tumour. The histological recognition of the myxomatous emboli is of vital importance for the diagnosis and treatment of this disease.

background. Scrotal calcinosis is a benign entity characterized by the appearance of calcific masses within the dermis of scrotal skin. Its pathogenesis has not been fully elucidated. objective. A 36-year-old man with massive scrotal calcinosis is described. Our aim was to perform a histologic examination of the surgical piece with subsequent closure of the defect achieving satisfactory cosmetic results. methods. Case report and literature review. results. Histologic studies of multiple nodules showed no epithelial lining and one calcified epidermal cyst. Subtotal excision of the scrotal wall was performed with excellent results. conclusion. Even though the pathogenic mechanism of this entity is still unclear, our findings support the theory of dystrophic calcification of epidermoid cysts. Surgical treatment is the only definitive treatment. Subtotal excision of the scrotal wall in cases of massive calcinosis may be performed with good results.

We report a patient who presented with an intensely pruritic rash, with hyperpigmentation of the whole back and multiple hyperpigmented and hyperkeratotic papules over his forearms and lower extremities. Histologically, he showed deposits of amyloid that reacted positively with monoclonal antibodies against cytokeratins. The lesions were resistant to antihistamines and corticosteroids. Treatment with oral acitretin was started and great improvement of the lesion was noted; the patient became asymptomatic. We review the literature on the effect of retinoids in cutaneous amyloidosis.

Se trata de una mujer de 48 anos, ingresada en la Unidad de Cuidados Intensivos (UCI) por insuficiencia respiratoria, hemoptisis y fracaso renal agudo. Como antecedentes, destacaba un cuadro de dos meses de evolucion consistente en odinofagia, afonia, inyeccion conjuntival bilateral fluctuante, artromialgias y febricula, junto con un episodio de condritis del pabellon auricular izquierdo y aparicion de lesiones cutaneas asintomaticas.

The patient was a 48-year-old woman admitted to the intensive care unit for respiratory failure, hemoptysis, and acute renal failure. Of note, there was a 2 month history of odynophagia, aphonia, fluctuating bilateral conjunctival injection, joint and muscle pain, and low-grade fever, together with an episode of chondritis of the pinna of the left ear and the appearance of asymptomatic skin lesions.

We report a patient with multiple myeloma associated with primary systemic amyloidosis who had a rapid evolution and a very unusual form of presentation. The association of amyloidosis in patients with multiple myeloma is 15%,1 and clinically evident mucocutaneous involvement occurs in up to 40% of patients.2

Thirty-three cases of hairy leucoplakia (HL) in HIV-positive patients were studied. The most frequent clinical presentation was the development of corrugated plaques on the lateral sides of the tongue without spiculations on the surface. In all biopsies, herpetic-type viral inclusions were found. In 12 out of 14 cases in whom an electromicroscopic study was carried out, only viral particles of the herpes group were found. Human papillomavirus (HPV) was not demonstrated, although by means of immunohistochemistry, positive labelling for HPV was found in 95% of the cases. The aetiopathological significance of these findings remains to be evaluated.