Drug hypersensitivity is an unpredictable, immunologically mediated adverse reaction, clustered in a genetically predisposed individual. The role of "hapten concept" in immune sensitization has recently been contested by the "pharmacological interaction" hypothesis. After completion of the "human genome project" and with the availability of high-resolution genotyping, genetic susceptibility to hypersensitivity for certain drugs has been proved beyond doubt though the trend is ethnicity and phenotype dependent. Application of this newly acquired knowledge may reduce or abolish the morbidity and mortality associated with cutaneous drug hypersensitivity.

Pemphigus is a dreaded disease encountered not infrequently in dermatology settings. While scoring systems in various dermatological conditions exist, objective parameters for assessing disease activity and therapeutic responses in pemphigus are not uniform and foolproof. This article presents various scoring systems in pemphigus.

Background: Few studies have been done on the relation between acetylator status and allergic diseases. Aim: To determine any possible association between acetylating phenotype in pediatric patients with atopic dermatitis (AD) and the disease prognosis. Patients and Methods: Thirty-six pediatric patients and forty two healthy children as a control group were participated in the study. All participants received a single oral dose of dapsone of 1.54 mg/kg body weight, after an overnight fast. Using high performance liquid chromatography (HPLC), plasma concentrations of dapsone and its metabolite (monoacetyldapsone) were estimated to phenotype the participants as slow and rapid acetylators according to their acetylation ratio (ratio of monoacetyldapsone to dapsone). Results: 72.2% of pediatric patients with AD showed slow acetylating status as compared to 69.4% of control individuals. Also, 73% of AD patients with slow acetylating phenotype had familial history of allergy. The severity of AD occurred only in slow acetylator patients. The eczematous lesions in slow acetylators presented mainly in the limbs, while in rapid acetylators, they were found mostly in face and neck. Conclusion: This study shows an association between the N-acetylation phenotype variation and clinical aspects of AD.

Background: Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. Aim: We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children. Materials and Methods: We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population. Results: Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients. Conclusion: This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.

Background: The metabolic complications and pathologic changes that occur in diabetes mellitus (DM) influence the occurrence of various dermatoses. Aim: To study the impact of control of diabetes on the pattern of cutaneous disorders. Materials and Methods: A cross-sectional descriptive study of patients attending diabetic clinic in a tertiary care hospital. A total of 500 consecutive patients were studied. Detailed history, clinical examination and relevant investigations were done to diagnose diabetic complications and cutaneous disorders. Dermatoses with or without known pathogenesis were correlated with age, gender, fasting plasma glucose (FPG), duration of diabetes, and complications of DM. Statistical analysis was carried out using Student "t" test and Chi-square test with 5% confidence interval (P value 0.05). Results: Majority of patients had well-controlled (FPG<130 mg/ml, 60%) type 2 DM (98.8%). No statistically significant difference (P>0.05) between the patients with or without DM specific cutaneous disorders was noticed with reference to age and gender distribution, duration of DM and FPG. Signs of insulin resistance, acrochordon (26.2%), and acanthosis nigricans (5%) were common, followed by fungal (13.8%) and bacterial (6.8%) infections. Eruptive xanthoma (0.6%), diabetic foot (0.2%), diabetic bulla (0.4%), diabetic dermopathy (0.2%), generalized granuloma annulare (0.2%), and insulin reactions (6.2%) and lipodystrophy (14%) were also seen. Conclusion: Well-controlled diabetes decreases the prevalence of DM-specific cutaneous disorders associated with chronic hyperglycemia. It is necessary to have a dermatologist in the diabetic clinic for early detection of potentially grave or predisposing conditions.

Background: The incidence of uncomplicated psoriasis is 1-3% in the general population. The involvement of palm and sole is seen in 7-14.5% of cases. There are different topicals and systemic therapies available for treating the case of psoriasis but none is satisfactory for longer duration. Aim: The study involved the comparative therapeutic evaluation of the different topical regimens and narrow band ultraviolet B (NB-UVB) therapy in combination with systemic methotrexate. Materials and Methods: The study was held in out-patient department of Skin, VD and Leprosy of B.R.D. Medical College, Gorakhpur, from July 2007 to December 2008. The group included 98 new cases of palmoplantar psoriasis. These cases were divided into eight groups according to the eight regimens involved in the study. The severity of psoriasis was assessed by the ESIF (erythema, scaling, induration and fissuring) score. Results: The study showed that all the regimens had significant response rates. The combination of NB-UVB with systemic methotrexate had maximum response rate (64.85±4.52%) that was statistically significant (paired "t0" at 16d.f. = 33.329, P<0.001) with minimum number of recurrences after stopping the treatment. The combination of halobetasol ointment with systemic methotrexate also had significant response rate (paired "t" at 19d.f. = 13.5183, P<0.001) but had maximum number of cases with recurrence (70%) after stopping the treatment. Conclusion: These results suggest that the combination of every regimen with systemic methotrexate resulted in an early and a good improvement in the quality of life of patients suffering from psoriasis. It also shows that NB-UVB in combination with systemic methotrexate is more efficacious and has minimum recurrence rate and side effects in the treatment of palmoplantar psoriasis.

Background: The scars of the cutaneous leishmaniasis and psychological problems of this disease need different interventions for its correction. Aim: Our objective in this study was to compare the efficacy of 50% trichloroacetic acid (TCA) solution and CO ₂ laser for treatment of the atrophic scars due to leishmaniasis. Materials and Methods: This was a randomized clinical trial performed in 92 patients. Patients were randomized into two groups: the first group was treated with 50% TCA solution, once monthly and for a maximum of 5 months, and the second group was treated with CO ₂ laser which was performed for only one time. Patients were followed-up at 3 and 6 months after starting the treatment. The improvement of scar was graded by a 6-point scale using digital camera and the collected data were analyzed using SPSS software. Results: In this study, 74 females and 18 males were enrolled. The improvement of scar was 48.13% in the TCA group and 44.87% in the CO ₂ laser group. This difference was not statically significant (P = 0.55). There was also no significant difference regarding side effects between these two groups. Conclusion: The results of our study showed that efficacy of focal with 50% TCA solution is compared with CO ₂ laser in treatment of leishmaniasis scar. Because of the low cost and simple application of TCA solution in comparison with CO ₂ laser, we suggest use of this treatment for correction of leishmaniasis scar or the atrophic scars.

Background: Therapeutic low-frequency ultrasound (US) has been used for many years to improve wound healing in chronic wounds like venous leg ulcers. No human data are available for the possible effects of single US applications on microcirculation and their frequency-dependency. Aims: To investigated the role of therapeutic low-frequency US on microcirculation of venous leg ulcers in vivo. Patients and Methods: This is a pilot study on an inpatient basis. We use a newly developed low-frequency continuous-wave US-equipment composed of a US transducer based on piezo-fiber composites that allow the change of frequency. In this study, we apply US of 34 kHz, 53.5 kHz, and 75 kHz respectively. Twelve patients with chronic venous leg ulcers are analyzed. As an adjunct to good ulcer care, therapeutic US is applied, non-contacting, once a day, in a subaqual position for 10 minutes. Microcirculation is assessed in the ulcers adjacent to skin before US-therapy, immediately after the treatment and 30 minutes later. We use a micro-light guide spectrophotometer (O2C, LEA Medizintechnik GmbH, Gieίen, Germany) for calculation of blood flow velocity, hemoglobin oxygen saturation (SCO ₂ ) and relative hemoglobin concentration (rHb) in 2 and 8 mm depth. Contact-free remission spectroscopy (SkinREM3, Color Control Chemnitz GmbH, Chemnitz, Germany) allows contact free measurements in the VIS-NIR range of the spectrum (400 ± 1600 nm). Results: It is seen that therapeutic US is well tolerated. One patient dropped out from a treatment series since he developed erysipelas responding to standard antibiotic. Effects were seen at 34 kHz only. The SO ₂ values increased after single US application. The values for rHb were higher in the superficial layer of the wound bed (depth 2 mm) compared to deeper parts (8 mm depth). US treatment did not result in significant changes of rHb and blood cell velocity. The data obtained by remission spectroscopy disclose an increase of oxygenized hemoglobin. Conclusions: The major findings are that continuous-wave low-frequency US of 34 kHz, but not, 53.5 kHz or 75 kHz, has a temporary stimulatory effect on microcirculation mainly due to an improved oxygenation. Further studies with treatment series are necessary.

Vitiligo is a disorder that causes the destruction of melanocytes. It has three important factors underlying this destruction. The depigmented skin has many aberrant functions such as a muted response to contact allergens, a phenomenon also seen in mice that depigment. The white skin of those with vitiligo does not form non-melanoma skin cancers although the white skin of albinos, which has a similar color as vitiligo, is highly susceptible to skin cancer.

Lewandowsky and Lutz dysplasia, also known as epidermodysplasia verruciformis (EV), is an inherited disorder in which there is widespread and persistent infection with human papilloma virus, defect in cell-mediated immunity and propensity for malignant transformation. Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail. Cases were followed up for 7 years. Detailed history, clinical features and investigations, including skin biopsy from different sites at different times, were examined. Generalized pityriasis versicolor like hypopigmented lesions in both the cases, together with variable pigmented nodular actinic keratosis like lesions on sun-exposed areas, were present. Multiple skin biopsies done from various sites on different occasions revealed features typical of EV along with lesions, i.e., actinic keratosis, Bowen's disease, basal and squamous cell carcinoma, in the elder sibling. However, skin biopsy of the other sibling showed features of EV and seborrheic keratosis only till date. This study reveals that the disease progression is variable among two individuals of the same family. Malignant lesions were seen only on sun-exposed areas and may be associated with other skin lesions or infections such as angiokeratoma of Fordyce and tinea cruris, as seen in this report.

A case of alkaptonuria, a rare disorder with autosomal recessive inheritance, is reported here. The patient had palmar pigmentation in addition to the usual features of alkaptonuria.

Xanthogranuloma is a benign, asymptomatic, and self-healing disorder of non-Langerhans cell histiocytosis, affecting mostly infants, children, and rarely adults. Diagnosis is easy in typical cases but become more complex in unusual forms. We report a case of a 28-year-old male patient who presented with multiple diffuse brown-to-yellowish papulonodular eruptions over extremities, ears, face, trunk, and extensors of joints with almost bilaterally symmetrical distribution for a period of one month. Histopathological examination of the skin biopsy specimen revealed features of xanthogranuloma. The patient was put on isotretinoin 20 mg once daily. Most of the lesions subsided or flattened within two months of isotretinoin therapy. This case is interesting because of the severity and atypical nature of the disease and also, the patient responded with isotretinoin therapy. But further study is required to observe the effectiveness of isotretinoin in xanthogranuloma.

Pustulotic arthro-osteitis, first described by Sonozaki, is a relatively rare disorder. Its prevalence is however probably underestimated in dermatological literature. Early recognition of the signs can prevent misdiagnosis. We describe a Turkish patient who presented with palmoplantar pustulosis and involvement of the sternoclavicular joint and peripheral oligoarthritis.

Maffucci's syndrome is a rare, congenital, nonhereditary, mesodermal dysplastic disease characterized by venous malformations and benign cartilaginous tumors. The occurrence of endocrine tumors in Maffucci's syndrome is very rare. We report a case of Maffucci's syndrome associated with hyperparathyroidism and multinodular goiter.

Acetaminophen is a widely used analgesic drug. Its adverse reactions are rare but severe. An 89-year-old man developed an indurated edematous and erythematous plaque on his left arm 1 day after acetaminophen ingestion. Cellulitis was suspected and antibiotictherapy was started but there was no improvement of the rash; there was a spectacular extension of the lesion with occurrence of flaccid vesicles and blisters in the affected sites. The diagnosis of generalized-bullous-fixed drug eruption induced by acetaminophen was considered especially with a reported history of a previous milder reaction occurring in the same site. Acetaminophen was withdrawn and the rash improved significantly. According to the Naranjo probability scale, the eruption experienced by the patient was probably due to acetaminophen. Clinicians should be aware of the ability of acetaminophen to induce fixed drug eruption that may clinically take several aspects and may be misdiagnosed.

A 35-year-old female presented with scaly annular and pigmented lesions all over the body of 2 years duration. Her clinical features, histopathology, immunofluorescence findings and positive ANA and anti-Ro antibodies were suggestive of subacute cutaneous lupus erythematosus-lichen planus. We report this case because of its clinical rarity.

Circumscribed palmar or plantar hypokeratosis is a rare benign epidermal malformation of the skin. Clinically it shows asymptomatic, well-circumscribed, and depressed erythema persisting for many years on the palms or soles. Its main histopathologic feature shows a characteristic epidermal depression with an abrupt decrement in the thickness of the stratum corneum, with a sharp stair between normal and involved skin. We describe a case of a 68-year-old woman who presented with an erythematous, asymptomatic, well-circumscribed, depressed patch, on the right thenar eminence which had been present for years.

A 26-year-old male who presented to the dermatology OPD with complaints of a swelling on his lip of 6 months' duration was on examination found to have a solitary ulcerated nodule over the lip and an enlarged submental lymph node. Skin smear and biopsy from the lesion did not yield the diagnosis. Needle aspiration from the draining lymph node revealed the diagnostic Leishman-Donovan bodies. The patient responded to treatment with a combination of oral ketoconazole and intralesional sodium stibogluconate. We report this case because of both the unusual location of the lesion and the unusual method of diagnosis and treatment.

A mixed tumor is a neoplasm that has microscopic features of both epithelial and mesenchymal differentiation. Such mixed tumors are known as pleomorphic adenomas in the salivary glands, and their cutaneous counterparts are called chondroid syringomas. These tumors commonly occur in the head and neck region of middle-aged men. Hyaline cell-rich chondroid syringoma is a rare benign variant of chondroid syringoma composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. Although very few cases have been reported in literature, it is important to be aware of this entity so as to avoid misdiagnosis on histopathological examination. In this report we present a case of hyaline cell-rich chondroid syringoma occurring in the finger.

Basal cell carcinomas (BCCs) are considered to be uncommon around the nail plate. An indolent lesion of this region should arouse suspicion of potential malignancy and a skin biopsy should be undertaken without delay. Early diagnosis can enable the physician to render simpler nondestructive modalities of treatment. In this article, we describe such a case of longstanding BCC of this region mimicking a traumatic ulcer. The nature of the ailment was finally discovered on biopsy and the carcinoma was initially treated with topical Imiquimod.

Black/bluish discolored skin tumor without cuticular covering de novo from site of origin could be a diagnostic feature of melanomas.

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.

Mudi-Chood is a strange dermatosis affecting the nape of the neck and upper back of young women in Kerala State of south India. It was first described by us in 1972. Here we report one more such case recently seen by us.