Morphea: a practical review of its diagnosis, classification and treatment

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Case for diagnosis. Sclerodermiform manifestations of porphyria cutanea tarda secondary to hepatitis C

Abstract: A 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.

Localized scleroderma: clinical spectrum and therapeutic update

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

Pulmonary manifestations in connective tissue disorders: Hospital-based study at a Tertiary Care Hospital.

Background. The pleuro-pulmonary manifestations frequently occur in patients with the connective tissue disorders (CTDs), and limited data are available on this topic from India. Methods. Between January 2002 and December 2006, 195 patients with various CTDs having respiratory symptoms were evaluated for respiratory system involvement. Results. Interstitial lung disease (ILD) was the commonest (38.5%) presentation of CTDs. It was observed in nearly threefourth of the cases with scleroderma followed by rheumatoid arthritis (RA) cases (44.5%). Pulmonary arterial hypertension was observed in 53.8% and 60% of cases with scleroderma and mixed connective tissue disorder (MCTD), respectively. In RA and systemic lupus erythematosus (SLE), pulmonary hypertension was a rare presentation. Pulmonary function tests were abnormal in all the patients with MCTD, 89.9% patients with RA, 84.6% patients with scleroderma and nearly one-third patients with SLE. Restrictive defect was the most common abnormality [RA (88.7%), scleroderma (72.7%), SLE (66%)]. High resolution computed tomography revealed reticulonodular lesions (n=78); pleural effusion (n=15, mainly in patients with RA and SLE); honeycomb appearance (n=14; mainly in patients with RA and scleroderma); and bronchiectasis (n=9, mainly in patients with RA). Conclusions. Pulmonary manifestations are common in patients with CTDs, ILD being the most common pulmonary involvement. All patients with CTDs should be systematically evaluated and monitored for pulmonary involvement.

Atrofodermia idiopática de Pasini-Pierine: revisäo / Idiopathic atrophoderma of Pasini-Pierini: review

Os autores fazem uma revisäo da literatura de atrofodermia idiopática de Pasini-Pierini (AIPP), destacando suas principais características clínicas, histopatológicas e histoquímicas. Säo abordados aspectos etiológicos e fisiopatogênicos da AIPP, e é discutida sua provável correlaçäo com outras afecçöes atróficas

Trauma-induced isomorphic lesions in morphea: a brief case report

We describe a case of morphea which presented further typical lesions of the disease at the sites of mechanical trauma. It can be suggested that cutaneous lesions of morphea may be locally developed due to physical stimuli as an isomorphic response in patients or subclinical cases of the disease.

Líquen escleroso e atrófico / Lichen esclerosus et atrophicus

O líquen escleroso e atrófico, descrito por Hallopeau em 1887, percorreu um longo caminho até ser considerado como uma doença autônoma, independente do líquen plano e da esclerodermia circunscrita, embora possa eventualmente associar-se à última. Sua etiologia ainda é desconhecida, mas há indícios de que seja multifatorial, envolvendo fatores hormonais, imunológicos e genéticos. A falta de intercâmbio entre ginecologistas, dermatologistas e patologistas é, em grande parte, responsável pelas controvérsias que cercam o líquen escleroso e atrófico genital, no que tange à nomenclatura e ao risco de malignizaçäo. A testosterona tópica oferece excelentes resultados no líquen escleroso e atrófico genital. Nos caso extenso, o ácido paraaminobenzóico parece ser útil