Epidermolysis Bullosa Pruriginosa successfully treated with concomitant topical and systemic agents.

Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, manifests at birth or late in life and is characterised by intense pruritus, resulting in lichenified or nodular prurigo-like lesions and scarring most prominent on the shins. Treatment is unsatisfactory. We report a patient treated with success using a combination of topical and systemic agents.

Case report of recalcitrant allergic contact eczema successfully treated with teriflunomide.

Allergic contact dermatitis (ACD) is a type IV, delayed-type reaction caused by skin contact with low-molecular-weight organic chemicals and metal ions that activate antigen-specific T cells, primarily T-helper 1 (Th1), in a sensitized individual, leading to skin eczema.First-line treatments are based on avoidance of causal agents and topical corticosteroids/immunomodulators. In recalcitrant cases, chronic oral immunosuppressive agents may be used, but they may have serious adverse effects and do not address the immunological disfunction. We report a case of severe ACD, unresponsive to topical or oral immunosuppressive therapy, which resolved itself after treatment with teriflunomide (TF) 14 mg/daily used for multiple sclerosis. TF is a once-daily, oral selective and reversible dihydroorotate dehydrogenase inhibitor, revealing a new treatment option for ACD.

Pyodermatitis vegetans of the vulva.

Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. No standardized treatment plan is available for PV. Its evolution tends to follow the progression of the underlying disorder. We report a patient with vulvar PV with good response to systemic antibiotics and corticosteroids.

Late onset pityriasis rubra pilaris type IV treated with low-dose acitretin.

Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. We report a 19-year-old male patient with a 2-week history of pruritic, scaling dermatosis of the hands, feet, elbows, and knees. He had no family history of skin disease. On physical examination, we observed circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma. The skin biopsy showed acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of pityriasis rubra pilaris. The patient started treatment with oral acitretin, 25 mg every other day. The treatment was tolerated well, and after 6 months the lesions had resolved completely. Pityriasis rubra pilaris is a chronic papulosquamous disorder of unknown pathogenesis, characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. There is still no consensus regarding the treatment, but therapeutic options include systemic retinoids, particularly acitretin in the recommended dose of 0.5 to 0.75 mg/kg/day. In our case, the patient was treated with a low-dose regimen of acitretin, which was effective and well tolerated.

Brachioradial pruritus in a patient with cervical disc herniation and Parsonage-Turner syndrome.

Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-year old woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900 mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.

Brachioradial pruritus in a patient with cervical disc herniation and Parsonage-Turner syndrome

Abstract Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-yearold woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.

Vulvoperineal Crohn's disease responsive to metronidazole.

Crohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized. Due to the paucity of reports of Crohn's disease at this location and in the absence of randomized trials, there are no standard treatments for the cutaneous disease. We describe the case of a 47 year-old woman with vulvoperineal Crohn's disease without digestive involvement, that was successfully managed with metronidazole.

Vulvoperineal Crohn's disease responsive to metronidazole / Doenca de Crohn vulvoperineal responsiva ao metronidazol

Crohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized. Due to the paucity of reports of Crohn's disease at this location and in the absence of randomized trials, there are no standard treatments for the cutaneous disease. We describe the case of a 47 year-old woman with vulvoperineal Crohn's disease without digestive involvement, that was successfully managed with metronidazole.

A doença de Crohn é uma doença granulomatosa multissistêmica inflamatória crónica que afecta primariamente o tracto gastrointestinal. Na maioria dos casos, as manifestações cutâneas sucedem a doença intestinal, mas, ocasionalmente, as lesões dermatológicas são o primeiro evento e podem constituir o único sinal da doença. O envolvimento vulvoperineal é raro, pode preceder os sintomas intestinais em meses ou anos, e pode passar despercebido. Devido à escassez de relatos de doença de Crohn com esta localização e na ausência de ensaios clínicos randomizados, não há nenhum tratamento padrão para a doença cutânea. Descrevemos um caso de uma mulher de 47 anos com doença de Crohn vulvoperineal sem envolvimento digestivo, que foi tratada com sucesso com metronidazol.

Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management.

Antiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It\'s characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done. Cutaneous manifestations are common and frequently the first sign of the disease. Although extremely diverse it\'s important to know which dermatological findings should prompt consideration of antiphospholipid syndrome and the appropriate management for those patients. Much has been debated about when to consider antiphospholipid syndrome and consensus still does not exist, however in spite of being a diagnostic challenge clinicians should know when to look for antiphospholipid antibodies since an early diagnosis is important to prevent further and serious complications. In this article we focus on the cutaneous features that should raise suspicion on the presence of antiphospholipid syndrome and on the complex management of such patients. Many other dermatological signs related to this syndrome have been described in the literature but only occasionally and without consistency or statistic impact and therefore will not be considered here.

Photoallergic reaction to cyamemazine.

A 50-year-old man presented with a scaly erythema of the face, upper chest, forearms, and dorsum of the hands. He has been treated with cyamemazine for 6 months. Photopatch tests were performed and the patient was diagnosed with photoallergic reaction to cyamemazine. The drug was discontinued and a course of oral steroids was prescribed. The patient was advised to avoid light exposure. There has been no evidence of recurrence during a six-month follow-up period. Photoallergic reactions are much less frequent than phototoxic disorders. It is well known that several drugs including neuroleptics of the phenothiazine family may produce a skin eruption on light-exposed areas by dose-dependent (phototoxic) or photoallergic mechanisms. It is believed that photopatch testing, which is the clinical investigation of choice for suspected photoallergic reactions, is significantly underused in Europe and probably world-wide.

Case for diagnosis.

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.

Case for diagnosis / Caso para diagnóstico

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.

Descrevemos um caso clínico de uma variante rara de pênfigo - pênfigo herpetiforme - que combina os aspectos clínicos da dermatite herpetiforme com os achados imunológicos do pênfigo. Devido à sua apresentação atípica, é frequentemente diagnosticado equivocamente como dermatite herpetiforme. Caracteriza-se essencialmente pelo padrão herpetiforme das lesões cutâneas, prurido intenso e presença de espongiose eosinofílica no exame histopatológico. Enfatizamos a excelente resposta terapêutica à dapsona.

Vesico-bullous subacute cutaneous lupus erythematosus--an uncommon entity successfully treated with dapsone and hydroxychloroquine.

Vesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquine.

Letter: Pellagra as the initial presentation of Crohn disease.

Pellagra is a nutritional disease caused by the deficiency of niacin. We describe a case of pellagra as the initial presentation of Crohn disease, which has been rarely described in the literature.