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.

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.

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.

Benign cutaneous Degos' disease.

Malignant atrophic papulosis is a rare systemic vaso-occlusive disorder characterized by thrombosis of vessels of the dermis, gastrointestinal tract, central nervous system and, occasionally, other organs. Cutaneous lesions consist of erythematous, dome-shaped papules that develop a central area of necrosis to leave a porcelain-like scar. The most accepted theory of pathogenesis is based on endothelial cell damage. There is no effective treatment of the disease. We describe a 26-year-old man with Degos' disease, a diagnosis based on the clinical and histologic pattern of skin lesions. The good response to antiplatelet therapy and the absence of systemic involvement over 8 years' follow-up is noteworthy. We believe that this case represents the benign form of the disease, typically referred to as benign cutaneous Degos' disease.

Psoriasis in association with prolactinoma: three cases.

We report three women with plaque-type psoriasis in whom increase in severity and extent of the skin lesions correlated with the development of a prolactinoma. In all three patients, administration of bromocriptine, a dopamine agonist that suppresses the secretion of prolactin, improved the therapeutic response of the psoriasis. To our knowledge, there are no reports of an association between psoriasis and prolactinoma. In recent years it has become apparent that prolactin plays an important part in the immune reactions and exerts a proliferative effect on human keratinocytes. We, therefore, discuss the part that prolactin may play in the pathogenesis of psoriasis.

[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.

[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.

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.

[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.

[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.