Iranian Journal Of Dermatology
Volume:15 Issue: 2, Summer 2012

Pemphigus vulgaris (PV) is an autoimmune bullous disorder that is fatal if left untreated. High dose systemic corticosteroids are the basis of therapy. The addition of immunosuppressive agents has improved the disease outcome and reduced the required corticosteroid dose and related toxicity. Mycophenolate mofetil is increasingly used as a steroid-sparing agent in immunotherapy of PV. Herein، we tried to appraise the efficacy of mycophenolate mofetil and topical clobetasol in the control of the major relapses of pemphigus vulgaris. Seventeen patients with severe relapse of pemphigus vulgaris were included in this study. All patients had complete remission on/off therapy before this period of recurrence. The patients were treated with 2g/day mycophenolate mofetil and 25-35g/day topical clobetasol propionate ointment. All patients were monitored for the side effects of therapy. The patients were followed for a mean period of 12. 7 months. The average length of time from initiating mycophenolate to 50% control (partial remission)، which occurred in all patients، was 6±1. 17 weeks. Fifteen patients achieved complete remission averagely at week 20. 8±7. 70. The average duration of followup after complete disease control was 8 months (ranging from 2-13. 5 months). Three patients were free of lesions for more than 12 months and 10 for more than 6 months. No important mycophenolate mofetil related complication was observed during treatment. The combination of mycophenolate mofetil and topical corticosteroid can be used to control PV relapses and taper-off corticosteroid.

Psoriasis is a common and chronic inflammatory skin disease that has profound adverse effects on patients’ wellbeing. Trace elements are essential to biochemical processes in the body and are involved in immunological and inflammatory reactions such as keratinization and melanin formation. The aim of this study was to evaluate the essential metals in psoriasis in comparision with healthy controls. We investigated serum levels of zinc، copper، and magnesium in 40 psoriatic patients and age and sex matched controls. Psoriatic patients showed significantly higher serum levels of copper / zinc and lower magnesium in comparison with the control group. No significant differences were seen in copper and zinc levels. There was no correlation between serum levels of these elements and psoriasis severity except for zinc; the serum level of zinc was inversely correlated with psoriasis severity. This study demonstrated some disturbances in serum levels of metals in psoriasis. More studies are required to clarify the importance of these findings in etiopathogenesis or treatment.

Pemphigus vulgaris is a rare autoimmune disorder characterized by cutaneous and mucosal blistering. Surprisingly، the management of oral lesions has been detailed only infrequently. As current topical therapies for oral lesions are of limited efficacy، application of calcineurin inhibitors is considered to be a potential option. The aim of this essay was to investigate the efficacy of tacrolimus 0. 1% ointment (Protopic®) versus triamcinolone acetonide 0. 1% paste (Volon-A®) in the treatment of oral pemphigus vulgaris. Fifteen patients were involved in a prospective randomized trial with a split- mouth design. After two weeks of administering study drugs، oral lesions were monitored and quantified pain and mucosal surface involvement scores were obtained. Within 14 days، the degree of involvement and pain scores significantly reduced in both tacrolimus-treated and triamcinolone-treated sites، but there was no significant difference between them. No severe adverse events were observed. This study showed that tacrolimus could be as effective as triamcinolone acetonide in the topical treatment of oral pemphigus vulgaris.

Systemic sclerosis is an autoimmune connective tissue disorder which is characterised by cutaneous and internal organ fibrosis. Raynaud’s phenomenon is the earliest feature and may precede the onset of the disease by months to years. The heart، lungs، gastrointestinal tract، kidneys and other organs may be involved. Our aim was to evaluate skin thickness and echogenicity in systemic sclerosis patients by ultrasound and compare it with the healthy age and sex matched controls. Our study included 15 successively admitted patients (14 females and 1 male) with systemic sclerosis and 15 healthy age and sex matched controls. All the patients met the ACR criteria of diagnosis. The age range of the patients was 25-55 years. The disease duration ranged from 1-8 years. A complete history and physical examination was done for each patient. Skin thickness and echogenicity were measured by a 20MHz ultrasound probe at 6 different anatomical sites which was compared with that of the controls. Skin thickness was more in systemic sclerosis patients (significant p value) as compared to controls، and echogenicity was inversely proportional to the skin thickness. Skin ultrasonography is a noninvasive method which can reflect the severity of skin involvement and periodic assessment of skin thickness and echogenicity can help to monitor the progression of the disease.

Abdominal cutaneous and subcutaneous nodules are uncommon benign or malignant lesions. The majority of the malignant nodules are metastatic in origin and may be the initial presentation of a primary malignancy; hence، an early diagnosis is important. Our aim of this study was to find out the spectrum of lesions (both non-neoplastic and neoplastic) that present as cutaneous and subcutaneous nodules on the abdominal wall and to find out the efficacy of fine needle aspiration cytology in the early diagnosis of such lesions so that the need for histopathology can be minimized. The study was conducted on 46 patients of all age groups who presented with various palpable cutaneous and subcutaneous abdominal nodules. These nodules were assessed by fine needle aspiration cytology. The diagnosis was made cytopathologically and subsequently correlated with the histopathological diagnosis when possible. Out of 46 cases aspirated; there were 13 non-neoplastic cases، 15 benign neoplasms، 17 malignant cases and one case whose tissue sample was inadequate for opinion but turned out to be metastatic deposits from renal cell carcinoma on histopathology. The rate of unsatisfactory fine needle aspiration cytology was 2. 2% and the sensitivity was 89. 47%. The specificity and positive predictive value were 100%. Fine needle aspiration cytology is a simple، minimally invasive، highly accurate and cost effective technique for early diagnosis of malignant metastatic nodules on the abdominal wall and therefore minimizes the need for histopathology and facilitates decision-making regarding the mode of treatment.

Focal dermal hypoplasia or Goltz syndrome is a rare genodermatosis involving all three embryonic layers. Herein، the first case of this syndrome from Iran will be reported. The main clinical features were fat herniation، reticulate pigmentations، telangiectasia، and skeletal defects.

Pilomatricoma is a benign skin adnexal tumour usually seen in the head and neck region of children and young adults. It is underrecognized on cytology، resulting in the overdiagnosis of malignancy. We bring forth a case report of a slow growing nodular swelling in a 10-year-old female child، which was misdiagnosed on Fine Needle Aspiration Cytology (FNAC) as a malignant neoplasm and found to be pilomatricoma histopathologically. Nodular lesions of pilomatricoma، which are predominantly composed of basaloid cells and devoid of other diagnostic features، lead to a false diagnosis of malignancy. To avoid misdiagnosis، pilomatricoma should be considered in differential diagnosis when primitive appearing cells are aspirated in slow growing subcutaneous nodules. Cytomorphological features of pilomatricoma and major pitfalls in diagnosis are also being discussed.

Phakomatosis Pigmentovascularis (PPV) is a rare cutaneous congenital malformation syndrome، defined as simultaneous occurrence of congenital cutaneous vascular and pigmentary anomalies. As most of the reported cases are from Far East countries، especially Japan، we reported a case of PPV type IIa in an Iranian patient. This case was unusual because of the coexistence of unilateral extensive port-wine stains all over the left body side and left hard palate and a right plantar port-wine stain.