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Erythromelalgia is an infrequent syndrome with a profound impact on quality of life. Its management is usually challenging and multiple treatments have been reported with variable response rates. To the best of our knowledge, we present the first case of erythromelalgia successfully treated with topical oxymetazoline.

Oral submucous fibrosis (OSF) is a precancerous condition of the oral cavity associated with habitual chewing of quid, with a high incidence among populations of the Indian subcontinent and Southeast Asia. Clinically, its initial manifestation may mimic oral lichen planus or lichen sclerosus. If the habit is not halted, the mucosa gets leathery and thickened, and fibrous bands form causing significant morbidity. Microscopically, it is characterized by atrophic epithelium, loss of rete ridges, and hyalinization of lamina propria. Of note, these hallmark histopathological features may be overlooked in the unusual presence of lichenoid interface changes, which may lead to the wrong diagnosis. We present herein five cases in which the rare joint appearance of OSF and lichenoid reaction features posed a diagnostic challenge. Due to its progressive nature and malignant potential, the presence of oral lichenoid changes overlying submucous hyalinization, in the right clinical and demographic setting, should raise suspicion of OSF and prompt actions directed at quid-chewing discontinuation.

Acropachy, also called clubbed fingers, presents as increased nail convexity over a thickened distal phalanx. Its origin is multifactorial, with the most frequent cause being neoplasms, although it can also be idiopathic. They can be found in isolation, or as part of hypertrophic osteoarthropathy. The importance of its diagnosis lies in the high frequency of underlying malignant pathology, so it must be rapid and comprehensive. Two cases are presented of patients with acropachy associated with underlying neoplasia.

Resumen Las acropaquias, tambien llamadas dedos en palillo de tambor, se presentan como un aumento de la convexidad ungueal sobre una falange distal engrosada. Su origen es multifactorial, siendo la causa mas frecuente las neoplasias, aunque tambien puede ser idiopatico. Pueden encontrarse de forma aislada o formando parte de la osteoartropatia hipertrofica. La importancia de su diagnostico radica en la alta frecuencia de enfermedad maligna subyacente, por lo que debe ser rapido y exhaustivo. Presentamos 2 casos de pacientes con acropaquias asociados a neoplasia subyacente.

Plica neuropathica (PN), also known as plica polonica, felting, matting or bird's nest hair, is an acquired hair condition in which hair becomes twisted, leading to the formation of a compact mass. Psychiatric disorders are an important etiologic factor for PN. We report a case of PN in a woman with severe reactive depression and present the trichoscopic findings.

Interstitial granulomatous dermatitis (IGD) is a rare dermatosis generally seen in the setting of rheumatic diseases, but also hematological disorders, internal malignances, infections, or drug induced. Herein, we report an exceptional case of an IGD with a clear chronological association with tocilizumab onset and cessation in a patient with adult-onset Still's disease. We review the granulomatous cutaneous reactions so far reported with this novel therapy: sarcoidosis, granuloma annulare, and IGD. Tocilizumab is a humanized anti-interleukin 6 receptor monoclonal antibody useful for the treatment of various systemic inflammatory disorders. Lately, it has found useful also for granulomatous diseases such as giant cell arteritis and even a promising response in IGD. Therefore, we believe our case adds the possibility of an IGD presenting as a paradoxical reaction.

Resumen Los farmacos inhibidores del factor de necrosis tumoral alfa (anti-TNF alfa) son ampliamente utilizados en diversas especialidades medicas. El principal efecto adverso de estos farmacos es el aumento del riesgo de infecciones. Presentamos el caso de un varon de 30 anos con espondilitis anquilosante, en tratamiento desde hacia 2 semanas con golimumab, que consulta por lesiones asalmonadas en tronco, palmas y plantas de 10 dias de evolucion. Con la sospecha de un secundarismo luetico se solicitaron pruebas treponemicas y no treponemicas que confirmaron el diagnostico. Asimismo se solicito una puncion lumbar, aunque no existia sintomatologia neurologica, para descartar neurosifilis. Los casos de sifilis en pacientes en tratamiento con anti-TNF alfa son excepcionales en la literatura y no hay protocolos establecidos que nos guien sobre como actuar ante esta situacion.

Subungual squamous cell carcinoma is very rare, but it is the most frequent primary malignant tumor of the nail bed. Therefore, its diagnosis and treatment are crucial to ensure a favorable prognosis. We present the sonographic findings in a case of a subungual squamous cell carcinoma. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:212-214, 2018.

Palmoplantar epidermoid cysts can range in clinical presentation from an asymptomatic slowly enlarging mass to a painful nodule. We report two cases: an epidermoid cyst on the sole and another on the palm. This article reviews the possible etiology, diagnosis, and prognosis of palmoplantar epidermoid cysts.

Resumen Introduccion La leiomiomatosis cutanea y uterina multiple (MCUL) o sindrome de Reed se caracteriza por la presencia de leiomiomas cutaneos de origen pilar, leiomiomas uterinos en las mujeres y, en algunos casos, asociacion con carcinoma renal. Este sindrome, de herencia autosomica dominante, se produce por una mutacion heterocigotica en la linea germinal del gen de la fumarato hidratasa, una enzima del ciclo de Krebs que actua como supresor tumoral. Objetivo Revisar los casos de MCUL diagnosticados en 2 hospitales universitarios durante un periodo de 5 anos (2008-2013). Material y metodos Estudio retrospectivo de 13 casos de MCUL, en el que se recogieron caracteristicas demograficas, clinicas e histologicas, asi como posibles asociaciones con otras enfermedades y tratamientos recibidos. Resultados Trece pacientes fueron diagnosticados de MCUL (10 mujeres y 3 hombres, con una edad media al diagnostico de 53 anos). El 100% de los casos presentaba leiomiomas cutaneos multiples, distribuidos de forma difusa (69%), agrupada (92%) y/o lineal (7,7%). El 90% de las mujeres presentaba ademas miomas uterinos y todas ellas habian precisado histerectomia por ese motivo. No encontramos ningun caso de carcinoma renal en los pacientes explorados (9/13), pero si lesiones renales benignas (4/9). Conclusion Describimos 13 casos de MCUL, que presentan caracteristicas clinicas e histologicas similares a las descritas en la literatura, siendo la manifestacion cutanea mas frecuente la segmentaria tipo 2. Es importante que el dermatologo identifique los casos de leiomiomas cutaneos y conozca su posible relacion con MCUL.

Background Multiple cutaneous and uterine leiomyomatosis (MCUL), or Reed syndrome, is characterized by the presence of cutaneous leiomyomas arising from the arrector pili muscles and, in women, by uterine leiomyomas. In some cases, MCUL is associated with renal cell carcinoma. This syndrome is an autosomal dominant disorder caused by a heterozygous germline mutation of the gene that encodes fumarate hydratase, a Krebs cycle enzyme that acts as a tumor suppressor. Objective To review the cases of MCUL diagnosed at 2 university hospitals over a 5-year period (2008-2013). Material and methods This was a retrospective study of 13 cases of MCUL that investigated demographic, clinical, and histologic characteristics, as well as possible associations with other diseases and treatments received. Results We identified 13 patients (10 women and 3 men) who had been diagnosed with MCUL. The mean age at diagnosis was 53 years. All the patients had multiple cutaneous leiomyomas; in 12 (92%) the distribution was clustered and 9 (69%) also had disseminated solitary lesions. In 1 patient (7.7%), the pattern of distribution was linear. Uterine fibroids requiring hysterectomy were present in 90% of the women. Nine patients were screened for renal lesions; no cases of renal cell carcinoma were detected but benign renal lesions were found in 4 patients. Conclusions The clinical and histologic characteristics of the 13 cases of MCUL reviewed were similar to those reported in the literature. The most common cutaneous manifestation was a type 2 segmental pattern. It is important for dermatologists to identify cutaneous leiomyomas and be aware of the possible association with MCUL.

Inhibitors of tumor necrosis factor-alpha (anti-TNF-alpha) are widely used in different medical specialties. The main adverse effect of these agents is the increased risk of infection. We report the case of a 30-year-old man with ankylosing spondylitis who had begun receiving golimumab two weeks earlier. He presented with a 10-day history of salmon-colored lesions on trunk, palms and soles. The clinical suspicion was secondary syphilis. Treponemal and nontreponemal tests confirmed the diagnosis of syphilis. Lumbar puncture was also performed, although there was no neurological involvement, to rule out neurosyphilis. Cases of syphilis in patients in treatment with TNF-alpha inhibitors are uncommon in the literature and there are no established protocols.

La leiomiomatosis cutanea y uterina mu ltiple (MCUL), tambien conocida como sindrome de Reed, leiomiomatosis cutis et uteri y leiomiomatosis mu ltiple, fue descrita en 1973 por Reed et al. Se caracteriza por la presencia de leiomiomas cutaneos de origen pilar en ambos sexos, leiomiomas uterinos en las mujeres y existe una variante asociada al carcinoma renal. Esta variante incluida dentro del mismo sindrome fue descrita en 2001 por Kiuru et al. , y se conoce como leiomiomatosis hereditaria y carcinoma de celulas renales (HLRCC). Este sindrome, de herencia autosomica dominante, afecta a ambos sexos por igual y se produce por una mutacion heterocigotica en la linea germinal del gen que codifica la fumarato hidratasa (FH), una enzima del ciclo de Krebs que actu a como supresor tumoral. Tanto la MCUL como la HLRCC presentan mutacion de la FH, por lo que se consideran variantes de la misma entidad.

Dermanyssus gallinae is a mite that normally parasitizes small birds but may occasionally bite humans. We report an unusual case of an 82-year-old woman who presented with pruritus and bite-like lesions over her trunk. Other members of the household were also affected. On physical examination, mites < 1 mm in size were found on the patient's body. The family were residing in the city centre and had no pets, but there were pigeon nests in close proximity to the house. Most dermatologists have difficulties identifying ectoparasitosis. In the case of D. gallinae, the small size of the mites and the fact that they leave the host after feeding means that they may not be seen at presentation, thus such infestations are likely to be underdiagnosed. Physicians should be aware that infection with this mite is possible even in patients from urban areas, and it should be included in the differential diagnosis of conditions causing recurrent pruritus unresponsive to standard treatments.

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.

Kaposi's sarcoma (KS) associated with AIDS (AIDS-KS) is the most prevalent neoplasia among patients with AIDS. Its incidence has decreased dramatically in recent years due to the widespread use of highly active antiretroviral therapy (HAART). Paradoxically, in relation to the introduction of HAART it may occur a worsening or development of AIDS-KS despite the improvement of the immune function. This unusual process is called immune reconstitution syndrome in Kaposi's sarcoma (IRIS-KS) and should not be confused with a failure of HAART. Early identification of AIDS-KS and previous or combined use of chemotherapy with HAART appears to be the most effective treatment to prevent the consequences of IRIS-SK.

Introduction Recently topical 3% diclofenac is commonly used to treat actinic keratosis (AK). It is proposed that it may also have a potential effect on hair growth. Case Presentation Three male patients were visited in the Dermatology department and received treatment with topical 3% diclofenac gel because of scalp AK. All patients also had concomitant androgenetic alopecia (AGA). During the follow-up visits, patients presented terminal hair growth on non-prior haired areas of the scalp. To the authors’ best knowledge, these are the first reported cases of hair growth due to topical diclofenac treatment. Conclusions AGA is a heritable, androgen-dependent process. Various hypotheses proposed to explain its causes. Authors hypothesized that the inhibition of the COX-2 shown by diclofenac might act over the perifollicular micro-inflammation and the prostaglandins misbalance observed in the AGA, leading to hair growth. This finding may open further research lines to develop new treatments for AGA.

Extramammary Paget disease is a rare condition that most commonly affects the anogenital region in the elderly. The treatment of choice has been surgical excision of the affected area with adequate depth and lateral margins, criteria that cannot always be fulfilled, especially when the vulva, anal canal, or penis are involved. More recently radiotherapy has been suggested as a suitable treatment when surgical excision or other modalities are not appropriate. We report a case of anogenital extramammary Paget disease and the clinical response to treatment with radiotherapy. The aim of this article is to review relevant aspects of radiotherapy as a first-choice curative treatment in specific situations of anogenital extramammary Paget disease in situ.

Cobb syndrome is a rare non-inherited neurocutaneous disease, in which there are metameric cutaneous and spinal vascular malformations of the trunk. In cases of segmentally distributed multiple cutaneous vascular lesions, early diagnostic imaging of the spinal cord allows prompt intervention in order to reduce permanent neurological sequelae. We report case of Cobb syndrome in a 12-year-old boy and present a systematic review of the literature.