Fatal paraneoplastic pemphigus after removal of Castleman's disease in a child.

Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman's disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.

Bilateral retro-auricular milia en plaque: a case report and review of the literature.

Milia en plaque, a rare inflammatory plaque type of milia is generally located in the periauricular area. Bilateral retroauricular milia en plaque is very rarely reported. Here, we report another case of bilateral retroauricular milia en plaque and review the previous cases.

Erosions on a prolapsed uterine in an old woman: an unusual manifestation of pemphigus vulgaris.

Vaginal involvement in pemphigus vulgaris has previously been described. In all those cases a pelvic examination was needed to explore the lesions. We describe a patient with pemphigus vulgaris who had pemphigus erosions on a prolapsed uterus (i.e., on the everted surface of vagina). The patient had widespread lesions of pemphigus in other mucosal and cutaneous sites. Biopsy, antibodies against desmoglein 1 and 3, and direct and indirect immunofluorescence were confirming. The erosions on the prolapsed uterus were resistant to treatment; other mucosal and cutaneous lesions responded rapidly to prednisolone and azathioprine. After lowering the dose of prednisolone the patient was referred to a gynecologist for a vaginal hysterectomy. This case was unique because her vaginal lesions could be easily examined and followed.

Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris.

BACKGROUND: Pemphigus is a severe autoimmune blistering disease affecting the skin and mucosa. Mortality is high in the absence of treatment. Nowadays, treatment is based mainly on corticosteroids and cytotoxic drugs; however, because of the rarity of the disease worldwide, there is not yet a standard treatment based on randomized controlled trials, and the treatment used is based mainly on the experience of experts. OBJECTIVE: The aim of this study was to compare the efficacy and safety of 4 treatment regimens for pemphigus vulgaris: prednisolone alone, prednisolone plus azathioprine, prednisolone plus mycophenolate mofetil, and prednisolone plus intravenous cyclophosphamide pulse therapy. METHODS: One hundred twenty new cases of pemphigus vulgaris were enrolled. These patients were randomly allocated into 1 of 4 treatment groups (each comprising 30 patients) and received prednisolone (P), prednisolone and azathioprine (P/A), prednisolone and mycophenolate mofetil (P/MM), and prednisolone and intravenous cyclophosphamide pulse therapy (P/PC). They were followed up for 1 year at the Pemphigus Research Unit. RESULTS: In groups P, P/A, P/MM, and P/PC, 23 (76.5%), 24 (80%), 21 (70%), and 22 (73.3%) of the patients, respectively, followed the regimen for the full 1-year period. The mean total dose of prednisolone administered in groups P, P/A, P/MM, and P/PC was 11631 mg (standard deviation [SD] = 7742), 7712 mg (SD = 955), 9798 mg (SD = 3995), and 8276 mg (SD = 810), respectively. The mean total dose of prednisolone in group P (prednisolone alone) was 11,631 mg, The mean total dose of prednisolone in the 3 cytotoxic groups was 8652 mg. By using analysis of variance, the difference was statistically significant (P = .047). In the cytotoxic groups, there was a significant difference between the P/A and P/MM groups (P = .007), but not between P/A and P/PC (P = .971), and P/MM and P/PC (P = .670). Side effects were not significantly different among the 4 groups. LIMITATIONS: Larger sample sizes and blind design are suggested for future studies. CONCLUSION: The efficacy of prednisolone is enhanced when it is combined with a cytotoxic drug. The most efficacious cytotoxic drug to reduce steroid was found to be azathioprine, followed by cyclophosphamide (pulse therapy), and mycophenolate mofetil.

KID syndrome.

KID syndrome is a rare congenital disorder characterized by keratitis, ichthyosis, and deafness. We have described a 4-year-old girl who is treated with bland emollients and topical keratolytics such as urea and surprisingly observed marked improvement in skin hyperkeratosis and palmoplantar keratoderma. We think that along with urgent ophthalmologic and otolaryngologic measures, simple topical therapies may improve skin condition in KID syndrome precluding the possible hazards of systemic retinoid therapy.

Anti-thyroid peroxidase antibody and vitiligo: a controlled study.

BACKGROUND: Vitiligo is an acquired depigmenting disorder due to destruction of melanocytes. Although many theories have been suggested for its pathogenesis, the role of autoimmunity is the most popular one. The association of vitiligo with autoimmune thyroid diseases and the increased prevalence of autoantibodies including thyroid autoantibodies in vitiligo favor this role. Our objective was to compare the frequency of thyroid peroxidase antibody (anti-TPO) in vitiligo patients with healthy subjects in Iran. METHODS: Ninety-four cases of vitiligo (46 female and 48 male) and 96 control subjects (49 female and 47 male) were enrolled in this controlled study. Patients with known thyroid disease, history of thyroid surgery and those receiving thyroid medications were not included. The two groups were matched regarding gender and age. The demographic data, symptoms related to thyroid diseases and results of skin and thyroid examinations were recorded in a questionnaire for each subject. Thyroid function tests including free T3, free T4 and TSH-IRMA were performed. Anti-TPO levels were assessed as well. The collected data were analyzed by SPSS version-11 in vitiligo patients and subgroups according to gender, age, extent, and duration of the disease compared with the control group. RESULTS: Anti-TPO was detected in 17 (18.1%) of patients affected by vitiligo, while this figure was 7 (7.3%) in the control group; the difference was significant with p-value < 0.025 (Phi & Cramer's V = 0.162). When analyzing subgroups, the difference in the frequency of anti-TPO remained significant only in females (p-value < 0.044) (Phi & Cramer's V = 0.207) and in patients in the age ranges of 18-25 (p-value < 0.05) (Phi & Cramer's V = 0.28) and 26-35 year-old (p-value < 0.042) (Phi & Cramer's V = 0.304). The difference of the frequency of anti-TPO was not significant regarding the duration and extent of vitiligo. In addition, there was no significant difference in the levels of free T3, free T4, and TSH in vitiligo patients compared with the control group. CONCLUSION: According to our study, anti-TPO was shown to be significantly more common in vitiligo patients especially in young women, compared with control group. As this antibody is a relatively sensitive and specific marker of autoimmune thyroid disorders including Hashimoto thyroiditis and Graves' disease, and considering the fact that vitiligo usually precedes the onset of thyroid dysfunction, periodic follow-up of vitiligo patients for detecting thyroid diseases is further emphasized especially in young women with increased level of anti-TPO.

Bilateral cutaneous leishmaniasis of upper eyelids: a case report.

An 11-year-old girl presented with a 6-month history of bilateral upper eyelid erythema, induration, and blepharitis resistant to conventional antibiotic therapy. The lesions gradually progressed to symmetrical ulcerative nodules within 3 months. Cutaneous smear and biopsy revealed numerous amastigotes in macrophage cells. Therapy with systemic pentavalent antimonial resulted in successful healing of lesions. We conclude that cutaneous leishmaniasis must be kept in mind in the differential diagnosis of bilateral chronic eyelid swelling in endemic regions of the world.

Koebner phenomenon in xanthelasma after treatment with trichloroacetic acid.

A 47-year-old woman presented with a history of yellow plaques on her eyelids. These lesions had been diagnosed clinically as xanthelasma and treated five times with topical applications of trichloroacetic acid (TCA) 33 percent. Despite flattening of the original lesions, the patient noticed extension of the lesions on the site of treatment following each session. Skin biopsy showed characteristic findings of xanthelasma. It appears that, in rare instances, xanthelasma palpebrarum may progress following TCA application by a Koebner-like phenomenon.

Pruritus in hemodialysis patients.

BACKGROUND: Pruritus is one of the most bothersome symptoms in patients on maintenance hemodialysis (HD), however little progress is seen in our understanding of its pathogenesis. The aim of this study was to evaluate the frequency of pruritus in HD patients in Tehran, Iran, and to correlate its presence and intensity with relevant clinical and laboratory parameters. METHODS: One hundred sixty-seven patients on maintenance HD at three out-patient HD units were enrolled in the study. Itch intensity was scored as mild, moderate and severe. Some relevant clinical and laboratory parameters (age, sex, xerosis, presence of neuropathy, duration of dialysis, history of atopy and laboratory findings including hematocrit, creatinine, urea, calcium, phosphorus, parathyroid hormone [PTH] and alkaline phosphatase) were evaluated. RESULTS: Pruritus was found in 41.9% of patients. The intensity of itching was mild, moderate and severe, in 51.4%, 11.4% and 37.7% of patients, respectively. In 22 patients (31.4%) pruritus intensified during and after dialysis. There was no significant difference in the serum levels of creatinine, blood urea nitrogen, calcium, phosphorus, alkaline phosphatase, PTH and hematocrit between patients with and without pruritus. Age, sex, xerosis, underlying renal disease, history of atopy and duration of haemodialysis were not significantly different between the two groups. However, neuropathy was significantly more common in the pruritic group (63.8% versus 42.1%) (pv = 0.006). CONCLUSION: Clinical neuropathy was the only significant finding in the pruritic group in our study. This finding justifies further research on nerve function and neurotransmitters in hemodialysis patients and the introduction of new drugs targeting neuropathy.

Erythroderma: a clinical study of 97 cases.

BACKGROUND: Erythroderma is a rare skin disorder that may be caused by a variety of underlying dermatoses, infections, systemic diseases and drugs. METHODS: We reviewed the clinical, laboratory and biopsy material of 97 patients diagnosed with erythroderma who were treated in our department over a 6-year period (1996 through 2002). RESULTS: The male-female ratio was 1.85:1. The mean age at diagnosis was 46.2 years. The most common causative factors were dermatoses (59.7%), followed by drug reactions (21.6%), malignancies (11.3%) and idiopathic causes (7.2%). Carbamazepine was the most common drug (57.1%). The best clinicopathologic correlation was found in cutaneous T-cell lymphoma and pityriasis rubra pilaris related erythroderma. Apart from scaling and erythema that were present in all patients, pruritus was the most common finding (97.5%), followed by fever (33.6%), lymphadenopathy (21.3%), edema (14.4%) and hyperkeratosis (7.2%). CONCLUSION: This study outlines that underlying etiologic factors of erythroderma may show geographic variations. Our series had a high percentage of erythroderma secondary to preexisting dermatoses and a low percentage of idiopathic cases. There was no HIV-infected patient among our series based on multiple serum antibody tests. The clinical features of erythroderma were identical, irrespective of the etiology. The onset of the disease was usually insidious except in drug-induced erythroderma, where it was acute. The group associated with the best prognosis was that related to drugs.

Eruptive syringomas.

Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Usually, they present as soft, flesh-colored to slightly yellow dermal papules on the lower eyelids of healthy individuals. We report an 18-year-old man with rare presentation of eruptive syringomas involving his trunk and extremities, with linear arrangement on the arms and forearms. A biopsy obtained from the lesions of the dorsum of the hands showed eccrine syringoma with a lymphocytic inflammatory infiltration around superficial blood vessels and eccrine ducts. We used the 585-nm and 595-nm pulsed dye laser for treatment of inflammatory lesions of forearm and trunk with no success.

Pigmented colloid milium associated with exogenous ochronosis in a farmer with long-term exposure to fertilizers.

BACKGROUND: Colloid milium is a rare cutaneous deposition disorder characterized by translucent papules developing on sun-exposed regions such as the face, neck and dorsal aspects of the hands and back. Exogenous ochronosis is caused by the accumulation of homogentisic acid resulting from long-term application of certain topical agents. Histology is characterized by yellow-brown pigment deposits in the papillary dermis. Prolonged use of hydroquinone may result in the development of the pigmented form of colloid milium, sometimes in association with ochronosis. CASE REPORT: A 53-year-old man presented with a 3-year history of multiple slow spreading pigmented papules on the dorsa of his hands, nose and ears. The patient had a long history of exposure to sun and fertilizers with no history of using hydroquinone bleaching creams. A later biopsy revealed the diagnosis of pigmented colloid milium associated with exogenous ochronosis. CONCLUSION: UV light damage and long contact with fertilizers may have a role in the development of pigmented colloid milium associated with exogenous ochronosis.

Tongue lesions in psoriasis: a controlled study.

BACKGROUND: Our objective was to study tongue lesions and their significance in psoriatic patients. METHODS: The oral mucosa was examined in 200 psoriatic patients presenting to Razi Hospital in Tehran, Iran, and 200 matched controls. RESULTS: Fissured tongue (FT) and benign migratory glossitis (BMG) were the two most frequent findings. FT was seen more frequently in psoriatic patients (n = 66, 33%) than the control group (n = 19, 9.5%) [odds ratio (OR): 4.69; 95% confidence interval (CI): 2.61-8.52] (p-value < 0.0001). BMG, too, was significantly more frequent in psoriatic patients (28 cases, 14%) than the control group (12 cases, 6%) (OR: 2.55; 95% CI: 1.20-5.50) (p-value < 0.012). In 11 patients (5.5%), FT and BMG coexisted. FT was more frequent in pustular psoriasis (7 cases, 53.8%) than erythemato-squamous types (56 cases, 30.4%). On the other hand, the frequency of BMG increased with the severity of psoriasis in plaque-type psoriasis assessed by psoriasis area and severity index (PASI) score. CONCLUSIONS: Nonspecific tongue lesions are frequently observed in psoriasis. Further studies are recommended to substantiate the clinical significance of these seemingly nonspecific findings in suspected psoriatic cases.

Ocular involvement in pemphigus vulgaris.

Pemphigus vulgaris (PV) is an autoimmune disorder affecting the skin and mucous membranes. Ocular involvement in PV has been reported but its prevalence and clinical characteristics are not well defined. This prospective cross-sectional study of 103 PV patients was designed to determine the prevalence, clinical types and epidemiological trends of ocular involvement in a population of Iranian patients with PV. Ocular involvement was present in 17 (16.5%) patients. Conjunctivitis was the most prevalent type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was noted in only one patient (5.9%). The most commonly reported symptoms were eye irritation (76.5%) and redness (76.5%). No significant relation was found between ocular involvement and disease activity (partial remission or relapse). Mucoid discharge was significantly more common in patients with conjunctival erosions as compared to patients with conjunctivitis (P = 0.038). We conclude that ocular involvement is not rare in PV; 16.5% of PV patients develop ocular disease independent of the disease activity and extension. Conjunctivitis is the most common type of involvement, however, palpebral conjunctival erosion is more frequent than previously realized.

Pemphigus vulgaris activity score and assessment of convergent validity.

Pemphigus is a rare autoimmune blistering disease with different phenotypes. The evaluation of therapeutic interventions requires a reliable, valid and feasible to use measurement. However, there is no gold standard to measure the disease activity in clinical trials. In this study we aimed to introduce the pemphigus vulgaris activity score (PVAS) measurement and to assess the convergent validity with the experts' opinion of disease activity. In PVAS scoring, the distribution of pemphigus vulgaris antigen expression in different anatomical regions is taking in to account with special consideration of the healing process. PVAS is a 0-18 scale, based on the extent of mucocutaneous involvement, type of lesion and the presence of Nikolsky's sign. The sum of the scores of total number of lesions, number of different anatomic regions involvement and Nikolsky's sign is weighted by the type of lesion. In the present study, PVAS was assessed in 50 patients diagnosed with pemphigus vulgaris by one dermatologist. Independently, five blinded experts scored all the patients through physician's global assessment (PGA). The convergent validity with experts' opinion was assessed. The Spearman coefficient of correlation showed the acceptable value of 0.751 (95%CI: 0.534- 0.876). PVAS is a valid, objective and simple-to-use scoring measurement. It showed a good correlation with PGA of pemphigus disease activity in Iranian patients with pemphigus vulgaris.

Pemphigus and pregnancy: a 23-year experience.

BACKGROUND: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. AIM: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. METHODS: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. RESULTS: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). CONCLUSION: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory.

Comparison of efficacy of azithromycin vs. doxycycline in the treatment of rosacea: a randomized open clinical trial.

BACKGROUND: Rosacea is a common inflammatory disorder of the skin. Systemic antibiotics currently used in the treatment of rosacea are sometimes associated with uncomfortable side effects. Therefore, a need for an effective agent with few side effects and good patient compliance exists. Azithromycin, a macrolide antibiotic with prolonged mode of action, has recently been found to be an effective alternative in the treatment of inflammatory acne. METHODS: For evaluation of the efficacy of azithromycin in the treatment of rosacea, we planned a randomized, open, clinical trial study to compare the efficacy of azithromycin with doxycycline in the treatment of this disease. Sixty-seven patients were randomized to receive either azithromycin 500 mg thrice weekly (on Monday, Wednesday, and Saturday) in the first, 250 mg thrice weekly (on Monday, Wednesday, and Saturday) in the second, and 250 mg twice weekly (on Tuesday, and Saturday) in the third month. The other group was given doxycycline 100 mg/day for the three months. Clinical assessment was made at baseline, at the end of first, second, third, and 2 months after treatment. Side affects were recorded. The limitation of this study is that there was no blindness. RESULTS: Statistically significant improvement was obtained with both drugs. Neither drug was shown to be more effective than the other. In the azithromycin group four patients had diarrhea, while epigastric burning was seen in two patients using doxycycline. CONCLUSION: This study indicates that azithromycin is at least as effective as doxycycline in the treatment of rosacea.

Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness.

The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.

Pemphigus vulgaris in Iran: a clinical study of 140 cases.

BACKGROUND: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. It varies in its clinical profile and epidemiologic characteristics in different parts of the world. OBJECTIVE: To determine the clinical features of PV in Iran in a prospective manner. METHODS: The study included 140 patients with newly registered PV attending our dermatology clinic between January 2003 and June 2004. RESULTS The mean age at the onset of the disease was 41.5 +/- 15.7 years, with a female to male ratio of 1.59 : 1. At presentation to our clinic, both skin and mucosal involvement was observed in 95 patients (67.9%). Cutaneous lesions without the involvement of the mucous membranes were seen in nine patients (6.4%), and exclusive mucosal involvement was present in 36 patients (25.7%). The most common initial localization of the disease was the oral cavity, which was involved in 93 patients (77.5%). The most frequent cutaneous and mucosal sites involved were the thorax and oral cavity, respectively. CONCLUSION: Although minor differences were noted, the results of this study are in relatively good agreement with the literature with regard to the age, gender, and initial presentation of PV in Iran. Some skin sites, such as the scalp, thorax, and axilla, may be more commonly affected in men. Patients with initial mucosal lesions were significantly younger than those with initial cutaneous involvement. Mucosal lesions other than the oral mucosa may be more common than previously thought.

Pemphigus and pregnancy: A 23-year experience.

Background: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. Aim: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. Methods: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. Results: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). Conclusion: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory.