Successful treatment of lichen planus with sulfasalazine in 20 patients.

BACKGROUND: Lichen planus (LP) is a disturbing pruritic cutaneous disease that may have an spontaneous resolution or exhibit a more chronic course during some weeks or months. OBJECTIVE: Our objective was to demonstrate that sulfasalazine is effective in the treatment of LP. METHODS: Twenty patients were diagnosed in our department with LP of the skin and/or mucosa between 1985 and 2001 on the basis of clinical and histologic findings. RESULTS: All patients were treated with sulfasalzine at initial doses of 1.5 g/day, increasing by 0.5 g/week to 3 g/day for 4-16 weeks. Some patients also received descendent doses for 2-12 months. Complete responses were observed in 13 patients and partial responses in seven patients. All patients reported an early resolution of the pruritus. No changes were detected in mucosal LP. Most of the patients tolerated the treatment well and only eight patients presented some minor side-effects. CONCLUSION: Sulfasalazine is a successful therapeutic option for cutaneous LP, constituting an alternative to corticosteroids and retinoids.

Treatment of oral vascular anomalies by transfixion technique.

BACKGROUND: Vascular anomalies (hemangiomas and vascular malformations) are frequently located in the oral cavity. They can be treated by cool steel surgery, cryotherapy, laser therapy, embolization, and sclerosis. The choice of treatment should depend on the type of vascular anomaly, its site, the general condition of the patient, and the doctor's experience. OBJECTIVE: The objective of this article is to assess the usefulness, indications, and advantages of transfixion technique in the treatment of oral vascular lesions. METHODS: We present seven patients with various vascular anomalies in the oral cavity that were treated using transfixion technique. This procedure is performed with local infiltration anesthesia and it consists of interlacing a polyglycolic acid suture a number of times in an upward direction until the vascular anomaly becomes bloodless. RESULTS: In all seven patients we completely eliminated the vascular lesion without significant long-term complications. We detected a transitory retractil scar in only one patient and edema in the postoperative period in another one. CONCLUSIONS: Treatment of vascular anomalies of the oral cavity by transfixion technique is an effective, simple method that does not cause major complications and is low in cost.

[The main etiopathogenic mechanisms of neurocutaneous diseases]. / Principales mecanismos etiopatogénicos de las enfermedades neurocutáneas.

Neurocutaneous syndromes constitute a large and complex group of diseases in which recent medical advances, particularly in the field of molecular biology and genetics, have afforded a deeper understanding of the way in which these diseases originate. In this article, we review the advances concerning pathogenic mechanisms. First, we discuss the malformations disorders of the central nervous system associated with skin disorders, which range from spinal and/or cranial dysraphism with skin lesions to fustrated forms of malformations of the neural tube, such us membranous aplasia cutis. Neurocutaneous vascular disorders can be due to malformational disease, such as in Sturge-Weber syndrome, as well as to autoimmune diseases. The analysis of mutations affecting the capacity for migration and differentiation of melanocyte precursors enables us to gain a better understanding of disorders of the cells of the neural crest, such as piebaldism and Waardenburg's syndrome. Mutations in tumor suppressor genes play an important part in the development of hamartomatous and neoplastic lesions in neurofibromatosis and tuberous sclerosis. Genetic mosaicism, both of the functional and the genomic kind, accounts for the great diversity of phenotypes and the distribution of neurocutaneous diseases. Lastly, neurocutaneous syndromes such as the paracrinopathies form an attractive hypothesis, which is as yet to be confirmed.

[Neuropeptides in dermatologic therapy]. / Los neuropéptidos en la terapéutica dermatológica.

The presence of neuropeptides and their specific receptors has been detected in the skin and the epithelial tissues. They are involved in innervation, immunomodulation, glandular secretion, control of cellular proliferation and regulation of blood flow. The fact that they act in so many different ways means that neuropeptides and their agonists and antagonists are now being regarded as potential therapeutic agents in dermatologic diseases. Among the substances which act as antagonists, particular attention should be paid to capsaicin, which has therapeutic potential for three types of indication: peripheral neurologic pain, affections with a neurogenic inflammatory component and pruriginous dermatosis; peptide T with therapeutic potential for psoriasis; and spantide, which might prove useful in dermatoses related to substance P. The influence of topical corticosteroids on the mechanism of action of neuropeptides can explain its efficacy in the treatment of many dermatoses. Among the agonists, the possibility of taking advantage of the vasodilatory activity of the calcitonin gene-related peptide is being considered in Raynaud's disease and erectile disfunction of the penis; and the immunomodulatory and anti-inflammatory action of the alpha-melanocyte stimulating hormone is being studied as a potential means of controlling inflammatory dermatoses of immunological origin.

Epidermal naevi associated with trichilemmal cysts and chromosomal mosaicism.

We present the case of a 29-year-old woman who has had a widespread epidermal naevus since birth, located on the left side of her body, upon which multiple trichilemmal cysts developed several years later. The karyotype of peripheral blood lymphocytes revealed chromosomal mosaicism 46, XX, t (1;9) (p36:q34). The possible pathogenic mechanisms include paracrinopathy, germinal or somatic mutation in the multipotential embryo cells, or a combination of the two.

Mucinosis of the mammary areolae and mycosis fungoides.

We present a patient with cutaneous mucinosis of the mammary areolae who developed multiple erythematous macules with clearly defined, geographical edges 1 year after diagnosis. These lesions demonstrated mycosis fungoides on histology. We discuss a possible association between the mucinosis and the mycosis fungoides.

Malignant atrophic papulosis. A report of two cases with altered fibrinolysis and platelet function.

Malignant atrophic papulosis is a systemic vaso-occlusive disorder characterized by typical skin lesions. We report two new cases with impairment of blood fibrinolytic activity and alterations in platelet function. The first case showed an increase in plasminogen activator inhibitor-1 (PAI-1) activity, and the second case had a decrease in platelet aggregation induced by adenosine diphosphate and adrenaline but normal with collagen. The impairment of blood fibrinolytic activity and platelet aggregation may have pathogenic and therapeutic implications in malignant atrophic papulosis.

Subcutaneous nodules as the first clinical manifestation of sarcoidosis.

We report the case of a 61-year-old male who presented with a febrile illness accompanied by arthralgia and myalgia. Two months later he developed multiple subcutaneous nodules and enlarged parotid glands. Later two erythematous plaques, clinically compatible with erythema nodosum (EN), were observed. Laboratory investigations revealed abnormal levels of angiotensin converting enzyme and chest radiography showed bilateral hilar enlargement. The biopsy of the cutaneous lesions demonstrated multiple non-caseating granulomas in the subcutaneous tissue without any alterations in the epidermis and the dermis. The cultures for Mycobacteria and fungi were both negative. The clinical picture and histopathological findings were compatible with subcutaneous nodular sarcoidosis. The response to steroid treatment was satisfactory.