Paulo Rowilson Cunha

Endemic pemphigus foliaceus (fogo seivagem). I. Clinical features and immunopathology

Endemic pemphigus foliaceus is an autoimmune disease that has remarkable features. Endemic foci are found in characteristic environments within the interior of Brazil. The epidemiologic data strongly suggest that an environmental factor initiates the autoantibody response in the host. As such it is an important disease for in-depth study. A group of interested investigators in both Brazil and the United States has been formed to attempt to do just that. As part of the overall effort, this Cooperative Research Group for the Study of Fogo Selvagem presents a definition of the disease, a proposed clinical classification for various forms of the disease, and an outline of what is currently known of its immunopathologic characteristics.

Efficacy of gabapentin in the improvement of pruritus and quality of life of patients with notalgia paresthetica

BACKGROUND notalgia paresthetica is a subdiagnosed sensory neuropathy presenting as a condition of intense itching and hyperchromic macule on the back that interferes with daily habits. OBJECTIVES To determine the efficacy of treatment of notalgia paresthetica using oral gabapentin, assessing the degree of improvement in itching and influence on quality of life. Moreover, to evaluate the signs and symptoms associated with notalgia paresthetica. METHODS We conducted an experimental, non-randomized, parallel, non-blinded study including 20 patients with clinical and histopathological diagnosis of notalgia paresthetica. After application of the visual analogue scale of pain adapted for pruritus and of the questionnaire of dermatology life quality index (DLQI), ten patients with visual analogue scale > 5 were given treatment with gabapentin at the dose of 300 mg/day for four weeks. The other ten were treated with topical capsaicin 0.025% daily for four weeks. After the treatment period, patients answered again the scale of itching. RESULTS The use of gabapentin was responsible for a significant improvement in pruritus (p=0.0020). Besides itching and hyperchromic stain on the back, patients reported paresthesia and back pain. It was observed that the main factor in the worsening of the rash is heat. CONCLUSION Gabapentin is a good option for the treatment of severe itching caused by nostalgia paresthetica.

Pruritus: still a challenge

Abstract: Prurido e um sintoma que pode estar associado a uma gama ampla de patologias, sejam elascutâneas, sistemicas ou mesmo sem causa definida . Conforme sua intensidade e evolucao pode com-prometer de forma significativa a qualidade de vida dos pacientes. Novas abordagens terapeuticas estaosendo descritas para o controle do prurido, onde a liberacao da histamina nao e o fator principal. Assim,baseando-se na origem de seus mediadores, faz-se uma revisao desses recursos e ressalta-se que, embo-ra de grande importância no arsenal terapeutico, os anti-histaminicos nao sao a panaceia para todos ostipos de prurido.Palavras-chave: Antipruriginosos; Diagnostico; Prurido; Resultado de tratamento Resumo: Pruritus is a symptom that may be associated with a wide array of diseases - skin diseases, sys-temic diseases or even those without a defined cause. According to its severity and evolution, it maycompromise the patients’ quality of life in a significant way. New therapeutic approaches, in which his-tamine release is not the main event, are being described for pruritus control. Therefore, taking intoaccount the origin of the mediators of pruritus, we conducted a review of the available resources emp-hasizing that, although very important, anti-histamines are not the answer for every kind of pruritus.Keywords: Antipruritics; Diagnosis; Pruritus; Treatment outcome

New approach in the treatment of refractory vitiligo: CO2 laser combined with betamethasone and salicylic acid solution.

The aim of this study is to evaluate the use of fractional carbon dioxide laser (CO2) with betamethasone and salicylic acid solution in the treatment of patients with refractory vitiligo in hands. Each hand of the patient was randomly assigned to one of two groups: lesion treated with fractional carbon dioxide laser associated with betamethasone and salicylic acid solution administration or lesion treated only with betamethasone and salicylic acid solution. We conclude that combined treatment with fractional carbon dioxide laser and betamethasone associated with salicylic acid solution could effectively and safely be used in the treatment of refractory vitiligo.

New insight in the treatment of refractory melasma: Laser Q-switched Nd: YAG non-ablative fractionated followed by intense pulsed light.

The purpose of our study was to verify the results of the association of Q‐switched Nd: YAG non‐ablative fractionated with intense pulsed light, in order to treat patients with refractory melasma. The combination of these two devices seems to be the best treatment to combat hyperpigmentation produced by melasma, with low occurrence of side effects, which may be justified by the selective photothermolysis at subcellular level.

Cutaneous neonatal lupus with cutis marmorata telangiectatica congenita-like lesions

Neonatal lupus is a rare disease caused by the transplacental transfer of maternal autoantibodies to the foetus, characterized by transient clinical manifestations such as cutaneous, haematological, and hepatobiliary events or permanent such as congenital heart block. The typical cutaneous manifestations include erythematous, scaly, annular or arched lesions on the face, with slight central atrophy and photosensitivy, clinically and histologically similar to subacute cutaneous lupus. However, in some cases, the lesions may resemble those in cutis marmorata telangiectatica congenita, although this phenomenon is rare and only eight such cases have been reported to date. We report a case of cutaneous neonatal lupus with atypical lesions on the limbs, which had a reddish-purple marbled appearance, resembling the lesions in cutis marmorata telangiectatica congenita.

Disabling Acne Fulminans

Acne fulminans is a rare manifestation that may occur during the evolution of acne vulgaris primarily in male adolescents. Use of isotretinoin, testosterone, and exacerbated immune responses in the body are related triggers. Signs, symptoms and laboratory findings such as fever, hepatomegaly, polyarthralgia, leukocytosis, plaquetose, increased inflammatory markers and transaminases, are characteristic. A bone scan can detect osteolytic lesions in multiple skeletal sites. The treatment is performed with prednisolone, isotretinoin and antibiotics if secondary infection is present. This case describes a male patient with a diagnosis of grade III acne, who developed acne fulminans and bilateral sacroiliitis with inability to ambulate, after initiation of therapy with isotretinoin.

Imunoglobulina intravenosa para tratamento de epidermólise bolhosa adquirida grave refratária a terapia imunossupressora convencional

Acquired bullous epidermolysis is a chronic and rare bullous subepidermal disease. It usually begins in adulthood and its etiology is unknown although it is associated with antibodies against type VII collagen. There are spontaneous and trauma induced formation of blisters that may cause serious complications. Treatment is disappointing and difficult. Apart from conventional therapy with systemic corticosteroid, new therapeutic modalities such as intravenous immunoglobulin are currently being used. This report highlights the extremely difficult clinical management of this rare disease and the important improvement provided by intravenous immunoglobulin.

Scleromyxedema: clinical diagnosis and autopsy findings

Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.

Primary cutaneous follicle center lymphoma simulating basal-cell carcinoma on the nasal ala

Primary cutaneous B-cell lymphomas (PCBCLs) constitute 25% of all primary cutaneous lymphomas. They present in the skin with no evidence of systemic or extracutaneous disease at the time of diagnosis, after adequate staging. Primary cutaneous B-cell lymphomas differ significantly from their nodal counterparts in relation to both clinical behavior and prognosis. The distinction between primary and secondary B-cell lymphomas is essential for defining prognosis/course of action. Such distinction is also very difficult to make, since primary and secondary B-cell lymphomas are clinically and histologically indistinguishable. We report the case of a patient with primary cutaneous follicle center lymphoma who underwent surgical excision.