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

Clinicopathological study of 1016 consecutive adnexal skin tumors

Adnexal tumors [ATs] are primary skin tumors with benign or rarely, malignant behavior. They have been classified based on differentiation towards hair follicle, sebaceous, apocrine or eccrine gland. Few large-scale studies have focused on ATs. To determine the prevalence of ATs and to assess clinical and histopathological trend of ATs. A retrospective descriptive study of all ATs diagnosed in Razi hospital between 2006 and 2010 was performed. A total of 30,000 pathology records were reviewed, and 1016 ATs were included. The prevalence of ATs was 3.3%. 518 patients [51%] were female, with a mean age of 34.5 years. 953 tumors [93.8%] were benign. ATs were most commonly located in the head and neck area [822, 83.5%]. The most common histopathological origin of ATs was sebaceous gland [536, 52.7%]. Sebaceous nevus of Jadassohn was the most prevalent single tumor type [40.6% of all ATs]. In 63.6% [646] of tumors, ATs were clinically suspected by the clinician prior to biopsy. The most common malignant AT was sebaceous carcinoma [23, 36.5% of all malignant ATs]. ATs are infrequent lesions, most commonly occurring in 3rd and 4th decade of life. Diagnosis of ATs is made by histopathological studies as they often express indistinctive clinical features. Malignant ATs are rare, occur at an older age, and are often hard to recognize clinically

Low dose psoralen plus ultraviolet a [PUVA] is an effective and safe method for the treatment of chronic graft versus host disease

Chronic graft versus host disease [ch.GVHD] is the most frequent late complication after allogenic stem-cell transplantation. Systemic immunosuppressive agents are usually required to control the disease. Psoralen plus UVA [PUVA] has been used for the treatment of ch.GVHD with variable beneficial effects. The objective of this study was to assess the efficacy and safety of a relatively lower dose of oral psoralen compared with previous reports, for the treatment of ch.GVHD patients with PUVA. Eleven patients who received allogenic bone marrow transplantation and had severe progressive ch.GVHD that was unresponsive to conventional immunosuppressive treatments were treated with oral 8-methoxypsoralen [0.2 mg/kg, up to 10 mg] two hours before exposure to UVA. The patients received a median of 43 treatments [range: 18 to 72]. Mean cumulative dose of UVA was 200.5 J/cm2 [range, 116.5-306.5 J/cm2]. In four of the 11 patients, there was a complete resolution of cutaneous ch.GVHD and the remaining seven patients achieved partial response with PUVA treatment. Complete and partial remission was observed in four and six patients with lichenoid lesions, respectively, but all of the four patients with sclerodermoid GVHD showed partial response to PUVA treatment. We observed no side effects like phototoxicity, nausea and vomiting, and exacerbation of GVHD. Liver enzymes raised in five patients, causing no significant morbidity for them. Low-dose psoralen plus UVA can be a safe and effective therapy for chronic cutaneous GVHD. Although the number of treatments and total cumulative exposure to UVA was rather high in our study, we observed no phototoxic reaction or severe irreversible liver damage due to phototherapy, which may be because of a relatively lower dose of methoxsalen used in our patients. Psoralen plus UVA is effective particularly in lichenoid GVHD lesions but sclerodermoid lesions may also benefit from this therapy

Comparison of efficacy of cryotherapy versus trichloroacetic acid in the treatment of solar lentigo on hands

Solar lentigines are common sun-induced benign melanocytic proliferations that present a significant cosmetic problem for many middle- aged and elderly patients. The purpose of this study was to assess the efficacy of cryotherapy in comparison to trichloroacetic acid [TCA] 33% on solar lentigines on the back of the h and s [SLBH]. Each h and of 33 patients with SLBH was treated randomly with either cryotherapy or TCA 33% solution. Photographs of the h and s were taken prior to and two months following the treatment. Therapeutic response and side-effect were compared using chi-square test. Twenty five patients completed the study. Cryotherapy was more likely to produce substantial lightening of the solar lentigines than TCA 33% solution [P=0.025] but was more painful and took longer to heal. Post inflammatory hyperpigmentation [PIH] was almost equal in two types of treatment. Statistically, better results were seen in lower Fitzpatrick's skin types. Cryotherapy appears to be superior to TCA 33% solution in the treatment of SLBH particularly in lower Fitzpatrick skin types. PIH is the major complication of both particularly in higher Fitzpatrick skin types. Generally, the major criterion for treatment of SLBH with cryotherapy or TCA is Fitzpatrick's skin type

Proteus syndrome: a case report

The Proteus syndrome comprises an association of asymmetrical overgrowth of almost any part of the body, verrucous epidermal naevi, vascular malformations and lipoma-like subcutaneous hamartoma. Both sexes are affected with equal frequency and severity and it is not transmitted genetically. Here a 16-year-old man with asymmetric overgrowth of the extremities, macrodactyly, cerebriform hypertrophy of the soles and various skin and bony lesions is reported diagnosed as Proteus syndrome

[ family with Bart-Pumphrey syndrome]

All member of family referred to our clinic complaining of white nails. Physical examination revealed clinical features of leukonychia totalis and also the presence of sensorineural hearing loss [SNHL], palmoplantar keratoderma [PPK] and knuckle pads, the four essential criteria for the diagnosis of Bart Pumphrey syndrome. Three generations were affected with variable presentations in male and female and autosomal dominant pattern of inheritance

[Report of a rare case of bullous mycosis fungoides]

Mycosis fungoides [MF], on extremely rare occasions, can be associated with vesiculobullous eruptions. We describe a 74-year-old man with previous documented histopathologic diagnosis of poikilodermic type of MF who recently developed some flaccid acral bullae on erythemoatous MF plaque and normal skin. Histopathology and direct immunoflourscence studies revealed extensive lichenoid changes with intraepidermal bulla. Atypical lymphocyte infiltration was presented in dermoepidermal junction and bulla fluid and peripheral blood smear, but absent in lymph node and bone narrow tissue specimens. The plausible cause of blister formation in cutaneous lymphoma bullosa may be due to excessive epidermotropism or toxic effects of cytokines secrected by the tumoral infiltrate

[Association of pemphigus vulgaris and tuberous sclerosis: a case report]

Pemphigus vulgaris is a blistering autoimmune disease with suprabasal cleft formation, which is the most common autoimmune blistering disease in eastern countries, such as Middle East. Predisposition to pemphigus is linked to genetic factors. Tuberous sclerosis is also a genetic disorder of hamartoma formation in many organs, particularly the skin, brain, eye, kidney and heart. We report a 17-year-old boy with pemphigus vulgaris associated with tuberous sclerosis, which to our knowledge the first report of this association

relationship of skin type with minimal erythema dose [MED]

Phototherapy is an important modality in dermatology and the number of skin diseases which can be controlled with it is increasing. In order to start treatment, the first dose of phototherapy is determined by measurement of minimal erythema dose [MED] in each patient individually or according to patient's skin type. To determine the relationship of skin type with MED in patients under UV phototherapy referred to Razi Hospital in Tehran .In patients referred to Razi Hospital in 1381[2002-2003] for UVB phototherapy, skin type was determined according to Fitzpatrick's classification and MED was measured using an UV-meter. Age, sex and the disease of the patients were also recorded. The data were analysed using one way ANOVA and Welch tests. Eighty patients including 39 males and 41 females were studied. The mean age of the patients was 32 years [SD=15.8]. Their diseases include psoriasis, vitiligo, parapsoriasis, pityriasis lichenoides chronica, perforating disorder and patch stage mycosis fungoides. The mean of MED in 17 patients with skin type II was 111.8 +/- 39.2, in 42 patients with skin type III was 105.9 +/- 43.1, and in 21 patients with skin type IV was 114.3 +/- 39.2 mJ/cm [2], with no significant statistical difference. The first dose of phototherapy could not be determined based on skin type and measurement of MED is necessary in each patient

Treatment of chronic cutaneous graft versus host disease [GVHD] with photochemotherapy with psoralen [PUVA]: a report of five cases

Chronic graft versus host disease [GVHD] remains the most common late complication of allogenic stem cell transplantation and the most frequent cause of morbidity and mortality in these patients. To control this condition, immunosuppresive drugs are usually administered at a high dose and for a long time, which may result in several side effects. Five patients with clinically and histopathologically established cGVHD [3 lichenoid, 1 sclerodermoid and 1 mixed lichenoid-sclerodermoid], who failed to respond to conventional immunosuppressive therapy were treated with psoralen and UVA [PUVA].Treatment was administered in a standard protocol three times a week on nonconsecutive days and were continued at least for 12 weeks. All 3 lichenoid cases showed complete improvement. In the sclerodermoid case there was partial response and in the mixed type case the partial response of sclerodermoid lesion and complete response of lichenoid lesions were observed. With the exception of a slight increase in liver tranferases, no other adverse reactions occurred. In conclusion, PUVA can be a safe and effective therapy in conjunction with systemic agents for chronic GVHD especially in lichenoid form

Anti-gliadin antibody in psoriasis: a case-control study

Psoriasis is a chronic inflammatory skin disorder with a wide variety of clinical presentations. Presence of anti gliadin antibody [AGA] has been reported in 16% of psoriatic patients. This study was designed to determine the frequency of the presence of AGA in psoriatic patients. After recording the demographic data and the severity of disease according to PASI scoring system, serum of 100 psoriatic patients and 100 healthy controls were studied for the presence of AGA using indirect immunofluorescence method. AGA was present in sera of 4 [3 males and one female] psoriatic patients while all controls were negative. Three patients were from 20 to 40 years of age. All four AGA positive patients sufferred from plaque type psoriasis. The disease severity was mild in one, moderate in two, and severe in one patient. None of them sufferred from gastrointestinal problems. Although AGA may be present in the serum of some psoriatic cases, a significant relation between skin lesion and hypersensivity to gluten has not been determined yet. More studies using more specific and sensitive assessment methods and larger sample sizes are suggested

[Cutaneous larva migrans: a case report]

Cutaneous larva migrans is a self-limited cutaneous eruption caused by larva of roundworms that do not normally parasitize humans. Ankylostomabrazielensis, the dog and cat hookworm, is the most common agent. The disease is found in tropical and subtropical regions and may be seen in travelers from these regions. We report a case of a 37-year-old Iranian man who had cutaneous larva migrans after traveling to Mexico. The patient was successfully treated with oral thiabendazole