Phacomatosis pigmentokeratotica is characterized by the coexistence of nevus sebaceus, papular nevus spilus and associated neurologic abnormalities. We report a case of phacomatosis pigmentokeratotica in a 28-year-old male who presented with palmar-plantar dysesthesia and ipsilateral brain hemiatrophy. As a characteristic neuroimaging finding of the disorder, we found multiple hypointense lesions involving the ipsilateral hemisphere.

Background:Pemphigus vulgaris is an autoimmune blistering disease that may initially present as localized lesions. It rarely remains localized throughout its clinical course.Observations: A 53-year-old woman with non-progressive pemphigus vulgaris localized to the tongue for 18 years is presented. Clinical examination showed erosions and ulcerations limited to the lateral margins of the tongue. Patient was treated with sublesional triamcinolone-acetonide injections as lesions recurred. Finally, triamcinolone- acetonide injections at three weeks intervals for three months induced a longterm sustained clinical remission for 18 months. The indirect immunofluorescence did not correlate with disease activity. Anti-desmoglein 3 antibodies (ELISA) remained elevated throughout the clinical course and during remission.Conclusions:This case highlights the recognition of localized pemphigus vulgaris and demonstrates the importance of local therapy and its potential to induce longterm remission. Similar report of additional cases may create a standard of care for non-progressive, localized pemphigus.

Background:Paracoccidioidomycosis is an important medical and social problem mainly in rural areas of Brazil, because of the high incidence of the diseases, its long clinical evolution, frequent recurrences and sequels leading to anatomical and functional incapacities.Main observation:We present a 73-year-old patient with paracoccidioidomycosis showing significant lymph node manifestations, which are only common in children and teenagers.Conclusions:Paracoccidioidomycosis may have a long incubation period, and it can be diagnosed outside of the endemic regions, where it was acquired. Thus, all dermatologists should maintain a high index of suspicion, especially in unusual cases of extensive lymph node enlargement in the elderly patients.

Background:Hepatitis C viral infection is a significant public health problem; 170 million persons are infected worldwide and the prevalence in the southern part of the United States exceeds two percent. Extrahepatic manifestations of hepatitis C viral infection are common; notably, 15-20% of patients will develop cutaneous manifestations of their disease. There are numerous dermatologic diseases associated with hepatitis C infection, including lichen planus, leukocytoclasticvasculitis, and porphyria cutaneatarda.Main observation:Recently, epidemiological studies have also demonstrated an association between hepatitis C infection and the development of non-Hodgkin lymphoma, especially marginal zone B-cell lymphoma. Herein we report the unusual case of a systemic marginal zone lymphoma in a patient with hepatitis C infection presenting clinically as localized lipoatrophy.Conclusions:Lipoatrophy can be a rare and diagnostically challenging presentation of secondary cutaneous marginal zone B-cell lymphoma. The importance of early recognition and detection cannot be over emphasized, as new and effective anti-viral treatments can lead to lymphoma regression in up to 75% of patients. To our knowledge, this is the first case of hepatitis C viral infection associated marginal zone lymphoma to present as localized lipoatrophy.

Background:Pustular psoriasis of the digits (acrodermatitis continua of Hallopeau) may be localized to one or more digits for over an extended period of time. Characteristic presentation is that of tender, diffusely eroded, and fissured pustular plaques on one or more digits. Transition to other forms of psoriasis and to generalized pustular psoriasis is known to occur. These patients have an increased risk of acute generalized exanthematous pustulosis (AGEP) compared to the general population. Pustular psoriasis is often therapy resistant.Main observations:We report the case of a 54-year-old Caucasian woman who presented with a pustular psoriasis flare complicated by AGEP. Treatment course included hospital admission, cyclosporine, acitretin, and discontinuation of cephalexin.Conclusion:The precipitating factor in the course of treatment is thought to be cephalexin. When treating patients with pustular psoriasis the occurrence of druginduced complications should be carefully examined. Our case suggests that avoidance of β-lactam antibiotics in these patients is warranted unless absolutely indicated.

Background:Despite encouraging reports on the efficacy of intravenous immunoglobulin (IVIg) in antiphospholipid syndrome, the clinical value of this treatment is not well established, and most of the data are based on case reports and small series of patients.Observation:We describe the significant improvement of leg ulcers with IVIg in a 61-year-old female, with diabetes mellitus, venous peripherical insufficiency and secondary antiphospholipid syndrome to systemic lupus erythematosus.Conclusions: This case illustrates a rare cause of leg ulcers and documents that IVIg may be an effective adjuvant treatment in the management of selected patients with antiphospholipid syndrome when conventional strategies using subcutaneous heparin and low-dose aspirin are insufficient.