Multiple cutaneous and uterine leiomyomatosis or reed syndrome: a retrospective study of 13 cases.

INTRODUCTION: 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. CONCLUSION: 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.

Dermanyssus gallinae (chicken mite): an underdiagnosed environmental infestation.

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.

Compounding as a current therapeutic option in dermatology.

Compounding continues to play a key role in the treatment of skin conditions, despite the abundance of products made by the pharmaceutical industry. Right from the earliest days of dermatology, compounding proved very useful in the treatment of diseases for which no specific drugs were available. However, as new products came onto the market, this usefulness was called into question, and doubts over safety, stability, and effectiveness were raised. Today, compounding is regaining the place it once held in routine dermatological practice. We review the advantages and disadvantages of compounding, the most common indications, current legislation in our setting, and the latest developments in active ingredients and vehicles.

Nail psoriasis: treatment with tazarotene 0.1% hydrophilic ointment.

INTRODUCTION: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. MATERIAL AND METHODS: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. RESULTS: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = .007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. CONCLUSION: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis.

Serum prolactin levels in psoriasis and correlation with cutaneous disease activity.

BACKGROUND: Prolactin (PRL), a neuropeptide secreted by the anterior pituitary gland, possesses a variety of physiological actions. It has been implicated as an important immunomodulator and exerts a proliferative effect in cultured human keratinocytes via specific receptors. Some studies have indicated an increase in serum PRL levels in psoriasis and exacerbation of psoriasis when a prolactinoma is present. AIM: To evaluate the correlation between serum PRL levels and Psoriasis Area and Severity Index (PASI). METHODS: Serum PRL levels were measured in 20 patients (10 mean, 10 women, age range 18-88 years) with plaque-type psoriasis before and after a 6-week period of topical treatment with tacalcitol ointment. Results were compared with a group of 20 healthy volunteers. RESULTS: Serum PRL levels were significantly increased in the psoriatic group compared with the control group (P < 0.001) and were significantly reduced after treatment (P = 0.001). There was a correlation between pretreatment serum PRL levels and PASI (r = 0.33; P = 0.02). CONCLUSIONS: These results indicate that serum PRL levels may serve as a biological marker of psoriatic disease activity.

Nail psoriasis: a retrospective study on the effectiveness of systemic treatments (classical and biological therapy).

BACKGROUND: Nail psoriasis represents a challenge for specialists. There is no comparative study of systemic treatment's effectiveness at this site. OBJECTIVE: Evaluate the response of nail psoriasis to classical and biological therapy and to compare the effectiveness and safety of the different treatments. METHODS: We performed a retrospective study of 84 patients with moderate-severe psoriasis seen at our Department between January 2006 and January 2009. RESULTS: Psoriasis was severe in 53.4%. In 75% of cases, the fingernails were affected, and the mixed form was the most frequently subtype. The mean baseline scores on the PASI and the NAPSI were 23.12 and 14.7 respectively; the correlation between the two scores fell at weeks 12 and 24 but had risen again at week 48. The baseline NAPSI score tended to be lower in women and significantly higher in patients over 65 years of age, family history of psoriasis, severe psoriasis and nail matrix involvement. In our series, 58.3% received classical treatment (acitretin, methotrexate, cyclosporin, PUVA, NUVB, REPUVA, RENUVB) and 41.7% received biological treatment (infliximab, efalizumab, etanercept, adalimumab).Significant reductions were found (P < 0.05) in the mean NAPSI scores at 12, 24 and 48 weeks with all the antipsoriatic agents except NUVB; significantly greater with cyclosporine (P < 0.01) and biological as infliximab and adalimumab at 12 and 24 weeks (differences between treatments disappeared at 48 weeks). CONCLUSION: The response to treatment is slower in the nail lesions than in the skin lesions. The improvement of nail psoriasis is significant both with the classical treatments significantly higher in cyclosporin; and biological treatment (infliximab and adalimumab at 12 and 24 weeks).

[Bullous scabies responding to ivermectin therapy]. / Sarna ampollosa con respuesta terapéutica a la ivermectina.

Bullous scabies is a rare disease that is usually diagnosed in elderly patients. The clinical, histological, and immunological findings are identical to bullous pemphigoid. In a review of the literature, we found reports of 24 cases. We present a new case of bullous scabies in a 72-year-old man. The lesions responded to treatment with oral ivermectin.

Nail psoriasis: a combined treatment with 8% clobetasol nail lacquer and tacalcitol ointment.

BACKGROUND: Nail involvement is a common and distressing feature in the course of psoriasis. Although much progress has been made in the treatment of the disease, the presence of psoriasis in the nail continues to pose a challenge. In recent years, vitamin D3 analogs and a new formulation containing 8% clobetasol-17-propionate in a colourless nail lacquer vehicle have produced good results for the control of nail psoriasis. OBJECTIVE: To determine the efficacy and safety of the combined treatment of 8% clobetasol-17-propionate in a lacquer vehicle and tacalcitol ointment in nail psoriasis. METHODS: Fifteen patients with both nail bed and nail matrix psoriasis were included in the study. They were treated with a colourless nail lacquer containing 8% clobetasol-17-propionate applied at bedtime at the weekend, and with tacalcitol ointment under occlusion on the remaining days, for 6 months. RESULTS: All 15 patients responded well to treatment. The therapeutic effect was very fast and directly related to the length of therapy. All nail alterations, including nail pain, were reduced, and the modified target Nail Psoriasis Severity Index fell by an average of 78% compared to baseline levels (+/-59.6, P < 0.0001). CONCLUSIONS: Combined treatment with tacalcitol ointment and 8% clobetasol-17-propionate in a nail lacquer is a safe, effective treatment for nail bed and nail matrix psoriasis.

Optimization of narrow-band uvb with a 5% oleic acid cream in the treatment of psoriasis.

Oleic acid is a monounsaturated fatty acid with a known action of penetration enhancer which has been used for various purposes, such as a tanning increaser. Narrow-band ultraviolet B (UVB) is a also first-line treatment for psoriasis. The purpose of this study was to evaluate if the use of a 5% oleic acid emulsion previous to the phototherapy sessions was useful in reducing the total dosage necessary for whitening in patients with psoriasis. Forty-four patients were included, 24 received application of the emulsion before phototherapy and 20 received phototherapy with no emulsion. Patients received the UVB sessions just to achieve a reduction of 80% of the basal PASI. The total dose received and number of sessions were compared within the 2 groups. A reduction in these parameters (29.68 J/cm(2) vs. 18.16 J/cm(2); 24 vs. 19 sessions) was seen in the group that received application of the emulsion. However, this was not statistically significant. The fact that we did not achieve the statistical significance may be due to the small sample size. These results must be cautiously interpreted and confirmed with further studies.

Topical tacrolimus for the treatment of psoriasis on the face, genitalia, intertriginous areas and corporal plaques.

Tacrolimus is an immunosuppressive drug that has proved effective in the treatment of psoriasis when administered systemically. Topically, it seems only useful in thin psoriasis plaques located on the face, genitalia, and intertriginous areas. We present an open-label clinical trial to test the efficacy of 0.1% tacrolimus ointment in patients with psoriasis on the face, intertriginous areas, both, and in corporal plaques. Efficacy was assessed with the evaluation of erythema, desquamation, infiltration, reduction of the PASI, and reduction of itching. A total of 15 patients were enrolled in the study. In all the localizations evaluated, each of the signs (erythema, desquamation, and infiltration) showed a statistically significant improvement when compared to the baseline (p < .001). Itching also improved rapidly. PASI was also reduced from a mean of 12 at baseline to 2.2 at the end of the study. Of the 15 patients, only 2 experienced an adverse effect (13%), which was described as a warm sensation in facial lesions which was transient and self-limited. In conclusion, tacrolimus ointment may be an alternative to classical options for the treatment of psoriasis, not only for intertriginous, genital, and facial areas, but also for corporal plaques without occlusion, with good tolerance.

[Actinic granuloma]. / Granuloma actínico.

We report a case of starting actinic granuloma. A revision about the subject is realized emphasizing the importance of histopathologic findings in starting lesions.

[In vitro cellular immunity in lymphoproliferative skin diseases. Inhibitory factor of macrophages]. / Estudio de la inmunidad celular "in vitro" en las enfermedades linfoproliferativas cutáneas. Factor inhibidor de los macrófagos (MIF)

27 patients affected of lymphoproliferative processes, 9 with different non-granulomatous dermatosis and 40 controls are studied by the authors. The technique David & David is used to determine the inhibition of the migration of macrophages in capillary tubes, adding to the culture medium the serum of the patient. They find MIF-like activity in the serum of three patients affected by the sezary syndrome. All sutdied cases of cutaneous lymphoma, non granulomatous dermatosis and healthy controls are negative.

[Generalized Mibelli's porokeratosis in transformation to spinocellular carcinoma. Clinical, histologic and ultrastructural study. Favorable response to RO-109359]. / Poroqueratosis de Mibelli generalizada en transformación a carcinoma espinocelular. Estudio clínico, histológico y ultraestructural. Respuesta favorable a RO-109359.

Two cases of Disseminated Poroqueratosis of Mibelli with squamous cell carcinomata development of many lesions are reported. Histopathological findings showed us the typical structure of the cornoid limella. Ultrastructural features are described with increased number of Langerhans" cells. Treatment in both cases was RO-109359. Squamous cell carcinomata were treated by surgical excision.

[Hopf's acrokeratosis verruciformis. Communication of a non-familial case]. / Acroqueratosis verruciforme de Hopf. Comunicación de un caso no familiar.

A 46-years-old man with non familial acrokeratosis verruciformis of Hopf is described. We comment on the clinical and histopathological features of this rare dermatosis and the unsuccessful therapy with etretinate.

Incontinentia pigmenti and bipolar aphthosis: an unusual combination.

Incontinentia pigmenti (IP) is an uncommon X-linked dominant multisystem disorder, lethal in the majority of affected males in utero and variably expressed in females. The cutaneous manifestations are diagnostic and classically occur in four stages: vesicular, verrucous, hyperpigmented, and atrophic. The skin lesions are typically spread along the lines of Blaschko, and they are usually present at birth. It may be variably accompanied by dental, ocular, neurologic, bones and joints, and development anomalies. The genes IP has been mapped to Xq28. Mutations in the NEMO/IKKγ gene, located at Xq28, have been found to cause expression of the disease. Behçets disease is a multisystem disorder consisting of recurrent oral aphtae, genital ulcers, pustular skin eruption, and uveitis. Occasionally there are other articular, neurological, intestinal, or vascular abnormalities. This disease is rare in children. Here, we report a case of a 16-year-old female with the rare combination of incontinentia pigmenti and an aphthosis bipolar, and we discuss the probably relationship between these two diseases.

[An update on scalp psoriasis]. / Psoriasis del cuero cabelludo.

Between 50 % and 80 % of patients with psoriasis have involvement of the scalp. The clinical presentation of scalp psoriasis can be very varied, with disease severity ranging from mild to extremely severe.The disease may have a major psychological impact. Treatment should be tailored to each individual in order to achieve a good clinical response or blanching that lasts for as long as possible, with a safe and convenient regimen. Many different treatments have been tried: phototherapy, pulsed magnetic fields, Grenz rays, keratolytics, coal tar, antifungals, dithranol, retinoids, vitamin D analogues, corticosteroids, and systemic treatment. Ideally, for scalp psoriasis, treatment should be effective; applied in the form of a lotion, foam, or emulsion; require few applications per week; and have proven long-term safety. One such treatment is potent corticosteroids and vitamin D3 analogues in combination, which has a fast onset of action and proven long-term safety.

Leiomiomatosis cutánea y uterina múltiple o síndrome de Reed: estudio retrospectivo de 13 casos / Multiple Cutaneous and Uterine Leiomyomatosis or Reed Syndrome: A Retrospective Study of 13 Cases

Introducción: La leiomiomatosis cutánea y uterina múltiple (MCUL) o síndrome de Reed se caracteriza por la presencia de leiomiomas cutáneos de origen pilar, leiomiomas uterinos en las mujeres y, en algunos casos, asociación con carcinoma renal. Este síndrome, de herencia autosómica dominante, se produce por una mutación heterocigótica en la línea germinal del gen de la fumarato hidratasa, una enzima del ciclo de Krebs que actúa como supresor tumoral. Objetivo: Revisar los casos de MCUL diagnosticados en 2 hospitales universitarios durante un periodo de 5 años (2008-2013). Material y métodos: Estudio retrospectivo de 13 casos de MCUL, en el que se recogieron características demográficas, clínicas e histológicas, así 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 diagnóstico de 53 años). El 100% de los casos presentaba leiomiomas cutáneos múltiples, distribuidos de forma difusa (69%), agrupada (92%) y/o lineal (7,7%). El 90% de las mujeres presentaba además miomas uterinos y todas ellas habían precisado histerectomía porese motivo. No encontramos ningún caso de carcinoma renal en los pacientes explorados (9/13), pero sí lesiones renales benignas (4/9). Conclusión: Describimos 13 casos de MCUL, que presentan características clínicas e histológicas similares a las descritas en la literatura, siendo la manifestación cutánea más frecuente la segmentaria tipo 2. Es importante que el dermatólogo identifique los casos de leiomiomas cutáneos y conozca su posible relación con MCUL (AU)

Introduction: 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. Conclusion: 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 (AU)

Masson's pseudoangiosarcoma of the tongue: report of two cases.

Two cases of Masson's vegetant intravascular hemangioendothelioma localized on the tongue are reported. This lesion has sometimes been confused histologically with angiosarcoma but always behaves in a benign fashion.