Denise Miyamoto

Increased serum levels of vascular endothelial growth factor in pemphigus foliaceus patients with erythroderma

Erythroderma is a clinical skin syndrome shared by patients with cutaneous disorders of distinct aetiologies as a result of the combined actions of chemokines, adhesion molecules, and cytokines, such as vascular endothelial growth factor (VEGF).

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience.

BACKGROUND Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.

Gastrointestinal cytomegalovirus disease in a patient with pemphigus vulgaris treated with corticosteroid and mycophenolate mofetil

Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil. She was hospitalized because of a 1-month history of watery diarrhea and oral ulcers. Unfortunately, the patient died suddenly on the ward. The autopsy revealed a bilateral saddle pulmonary embolism, Gram-positive cocci bronchopneumonia, and gastrointestinal cytomegalovirus infection, causing extensive gastrointestinal mucosal ulcers.

Sebaceous Cell Carcinoma: A Persistent Challenge in Clinical andHistopathological Diagnosis

Sebaceous cell carcinoma continues to defy clinicians and pathologists in terms of early diagnosis. The tumor may be mistaken as benign lesions such as chalazion and blepharitis, and also as malignant neoplasms, mainly basal cell carcinoma and squamous cell carcinoma. Despite advances in immunohistochemical analysis and treatment options during the last decades, morbidity and metastasis rates remain high. Prognosis is strongly related to the length of time between diagnosis and initiation of treatment, which reinforces the importance of early recognition of this condition. This article reviews key features of sebaceous cell carcinoma, from epidemiology to treatment, and new strategies to improve outcome.

Exfoliative erythroderma as a clinical manifestation of autoimmune bullous diseases.

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Pemphigus Foliaceus, var. Herpetiformis and Sulphonamide

A 43-year-old woman presented a 2-year history of tender blisters and erythematous and crusted excoriations on the face, trunk and proximal extremities. During her first examination in 1995, erosions and yellow crusts on her face, thorax, upper torso and proximal limbs were present, without mucosal involvement. Laboratory profile confirmed the diagnosis of pemphigus foliaceus, as follows: histopathological analysis exhibited subcorneal abscess with mild spongiosis and scanty acantholytic cells; neutrophilic and linfohystiocitic perivascular infiltrate was present in the superficial dermis; direct immunofluorescence showed intercellular IgG deposits within the entire epidermis; indirect immunofluorescence revealed circulating IgG antibodies (titer=1:1,280) and positive ELISA index (99, recombinant desmoglein 1). Treatment with systemic corticosteroid (prednisone 1 mg/kg/day) was initiated. Clinical remission was achieved after 2 months, followed by gradual corticosteroid tapering. The patient presented reactivation when receiving prednisone 10 mg/day, with vesicles with a herpetiform arrangement on erythematous plaques on her legs, with intense pruritus (Fig. 13.1).