MYXOID NEUROFIBROMA: AN UNUSUAL PRESENTATION

Myxoid neurofibroma (MN) is a benign tumor of perineural cell origin, which is demonstrated with a positive immunohistochemical staining for S-100 protein. The most common locations of the MN are the face, shoulders, arms, periungual and in the feet. To our knowledge, this is the first time that a trunk location is reported. MN should be included in the differential diagnosis of tumors on this location.     

HYPERTROPHIC LUPUS VULGARIS: AN UNUSUAL PRESENTATION

Lupus vulgaris is the most common form of cutaneous tuberculosis occurring in previously sensitized individuals with a high degree of tuberculin sensitivity. Various forms including plaque, ulcerative, hypertrophic, vegetative, papular, and nodular forms have been described. A 30-year-old male patient presented with a very large hypertrophic lupus vulgaris lesion over left side of chest since 22 years. Histopathological examination showed granulomatous infiltration without caseation necrosis. The Mantoux reaction was strongly positive. Hypertrophic lupus vulgaris of such a giant size and that too at an unusual site is extremely rare and hence is being reported.     

UNUSUAL PRESENTATION OF GENERALIZED MACULAR AMYLOIDOSIS IN A YOUNG ADULT

Macular amyloidosis is a common problem seen dermatology out-patient department. Generalized macular amyloidosis presenting with a poikilodermatous appearance is rare. In our case, an 18-year-old male presented with generalized hypopigmented macules with a poikilodermatous appearance of 10-year duration. His developmental milestones were normal with negative family history of similar complaints. Histopathology of hyperpigmented lesions revealed hyperkeratosis and acanthosis of epidermis and hypopigmented lesion showing only hyperkeratosis. Both lesions were showing the deposition of amorphous, hazy material in the tips of papillary dermis with perivascular inflammatory infiltrate. Congo red staining of the amorphous material was positive for amyloid.     

PATCH TESTING WITH DERMATOPHAGOIDES AND ITS CORRELATION WITH CHRONIC ECZEMA AND ATOPIC DERMATITIS

Background:

Chronic eczema is commonly encountered in the Indian set up. So also is atopic dermatitis. House dust mites (Dermatophagoides) are implicated in various diseases like atopic dermatitis, asthma, and perennial rhinitis. It has also been proven that patch testing with Dermatophagoides pteronyssinus (DP) is important for detection of contact sensitization in chronic dermatitis.

Aims:

To study clinical characteristics of DP mix positive patients with regards to chronic dermatitis and atopic dermatitis.

Methods:

Dermatology outpatients presenting to the department of Skin and STD of Kasturba Medical College (KMC), with clinically diagnosed atopic dermatitis and chronic eczema were chosen for the study. Inclusion and exclusion criteria were well demarked. Eighty six randomly selected patients of dermatitis were subjected to patch testing with standard series and DP mix.

Results:

Of the 86, 50 (58%) showed positive reaction to DP mix. Among these positive patients, chronic dermatitis was seen in 42 (84%) with involvement of exposed parts in 37 (74%). Atopic dermatitis was seen in 19 patients (38%) from DP positive group whereas it was observed in 4 patients (17%) from the other group.

Conclusion:

Dermatophagoides mix positivity was statistically significant in chronic eczema as well as atopic dermatitis. Patch testing is an important tool to detect delayed type allergy to house dust mite.     

AN UNUSUAL LOCATION OF BASAL CELL CARCINOMA: THE CLITORIS AND THE VULVA

Vulvar basal cell carcinoma (BCC) is rare, accounting for less than 5% of all vulvar neoplasms and less than 1% of all BCCs. Vulvar BCCs are usually diagnosed late because they are often asymptomatic and tend to grow at slow rates. They may be invasive and destructive if neglected or improperly treated. Nevertheless, they have a very low propensity for metastatic spread, but frequently recur after simple excision. We report a 78 year-old woman presenting with the complaint of painful vulvar ulceration and vaginal bleeding. The physical examination revealed a 3 × 2 cm indurated nodulo-ulcerative lesion involving the clitoris, both labia minora and left labium majus. The histopathology was consistent with the “solid type BCC” that invaded the subcutaneous tissue without lymph node metastasis. The patient underwent wide local excision with clitoral amputation and remained disease free at post-surgical follow-up after 18 months.     

RARE PRESENTATION OF KYRLE'S DISEASE IN SIBLINGS

Background:

Kyrle''s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings.

Materials and Methods:

Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction.

Results and Conclusions:

A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle''s disease. This helps to differentiate the rare primary Kyrle''s disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.     

RECURRENT IMPETIGO HERPETIFORMIS WITH DIABETES AND HYPOALBUMINEMIA SUCCESSFULLY TREATED WITH CYCLOSPORINE, ALBUMIN, INSULIN AND METFORMIN

We report the case of a patient with recurrent impetigo herpetiformis associated with diabetes mellitus, hypoalbuminemia, and hypocalcaemia; who was refractory to corticosteroids. Cyclosporine along with other supportive measures proved to be life-saving with maintenance of pregnancy.     

VITILIGO TREATMENT WITH VITAMINS, MINERALS AND POLYPHENOL SUPPLEMENTATION

Background:

Mammalian pigmentation results from the synthesis and accumulation of photo protective epidermal melanin. Melanin was formed from the amino acid precursor L-tyrosine within specialized cells, the melanocytes. Oxidative stress has been suggested to be the initial pathogenetic event in melanocyte degeneration with H₂O₂ accumulation in the epidermis of patients with active disease. Auto immunity has been also suggested as another hypothesis in the pathogenesis of depigmentation disorders. Topical corticosteroids and phototherapy as common treatment modalities have been prescribed in patients with vitiligo. However, they are often not effective and safe (epidermal atrophy). Therefore, research for alternative therapies continues.

Aims:

To evaluate the beneficial effects of a supplementation with antioxidant vitamins (A, C, E) and minerals (zinc, selenium) for vitiligo treatment.

Methods:

Forty experimental autoimmune vitiligo mice C57BL6, aged from 5 to 12 months showing visible signs of induced vitiligo, were sequentially randomized into five parallel groups (8 mice per group). Each group mice was allocated an identical pre coded cage. the first group (SZV) received the ED + 1,4 g zinc (Zn) + 0.04 g selenium (Se) + vitamins (A 118 UI, C 8,5 mg, E 5,4 UI) /kg diet, the second group (PSZV) received the ED + 1,4 g zinc (Zn) + 0.04 g selenium (Se) + vitamins (A 118 UI, C 8.5 mg, E 5,4 UI)/kg diet + Polyphenol orally, the group 3 (PSZ) received the ED + green tea decoction prepared from 100 g/l (polyphenol orally) + 1,4 g Zn + 0.04 g Se, the 4 (P) received the ED + green tea decoction prepared green tea decoction prepared from 100 g/l, the control group 5(C) received the ED + distilled water. Cure was defined as repigmentation of treated sites. Photographic and optical techniques were used both at the baseline and on weekly basis.

Results:

By the end of the study, mices showed visible repigmentation. Using the investigator''s global assessment, therapeutic success in terms of a clear repigmentation documented in 70% of treated mice.

Conclusion:

Our findings suggest that an antioxidant supplementation is significantly beneficial in contributing superior clinical efficacy to cure vitiligo.     

THYROID DYSFUNCTION AND THYROID ANTIBODIES IN IRANIAN PATIENTS WITH VITILIGO

Vitiligo is a common skin disorder, and the pathogenesis is unknown. An increased prevalence of autoimmune thyroid diseases has been described in these patients. The aim of this study is to assess the prevalence of thyroid dysfunction and hypoparathyroidism in patients with vitiligo.

Materials and Methods:

One hundred and nine patients (38 males and 71 females with vitiligo were enrolled. Thyroid physical examination was carried out. Thyroid function tests, thyroid antibodies, calcium and phosphorus were assessed. The collected data were analysed by SPSS version 11.

Results:

Thyromegaly was found in 30.1% of patients. Hypothyroidism was found in 16 (15.7%) out of 109 cases. Two of them had clinical and 14 had subclinical hypothyroidism. One patient had Grave''s disease. Antibody positivity was the most common disorder (anti-TPO and anti-tg were positive in 36.7 and 32.1%, respectively). No patient had hypoparathyroidism.

Conclusion:

According to our study, thyroid dysfunction, particulary hypothyroidism and thyroid antibodies increase in patients with vitiligo. We recommend thyroid antibodies assessment and thyroid function evaluation in these patients.     

ACUTE GENERALIZED EXANTHEMATOUS PUSTULOSIS: AN UNUSUAL SIDE EFFECT OF MEROPENEM

A male patient was hospitalized as a case of pneumonia. He was diabetic, hypertensive and post Hepatitis “C” “H-C”. He reported skin eruption following administration of meropenem. Skin biopsy revealed acute generalized exanthematous pustulosis. To elucidate this side effect, we conducted a literature search - this is the second case induced by meropenem. The diagnosis was made after excluding all other possible causes. Dermatologists and clinicians must be aware of this an unusual side effect.     

COMPARISON OF PLASMA MALONDIALDEHYDE, GLUTATHIONE, GLUTATHIONE PEROXIDASE, HYDROXYPROLINE AND SELENIUM LEVELS IN PATIENTS WITH VITILIGO AND HEALTHY CONTROLS

Background:

The etiology and pathophysiologic mechanism of vitiligo are still unclear. The relationship between increased oxidative stress due to the accumulation of radicals and reactive oxygen species and the associated changes in blood and epidermal component of vitiliginous skin have been reported many times. We investigated the possible changes of plasma malondialdehyde, glutathione, selenium, hydroxyproline and glutathione peroxidase activity levels in patients with vitiligo in order to evaluate the relationship between oxidative stress and etiopathogenesis of vitiligo.

Materials and Methods:

Plasma malondialdehyde, glutathione, hydroxyproline and glutathione peroxidase activity levels were measured by spectrophotometric methods, and HPLC was used for measurement of selenium concentrations.

Results:

Our results showed increased malondialdehyde, hydroxyproline and glutathione peroxidase activity levels in plasma of vitiligo group (P < 0.05).

Conclusion:

Support of antioxidant system via nonenzymatic antioxidant compounds and antioxidant enzymes may be useful to prevent of melanocyte degeneration which occur due to oxidative damage in vitiligo.     

SERUM LEPTIN, ATHEROGENIC LIPIDS AND GLUCOSE LEVELS IN PATIENTS WITH SKIN TAGS

Aim:

To investigate the relationship between serum leptin, atherogenic lipid and glucose levels in patients with skin tags and healthy controls.

Materials and Methods:

A total of 58 patients, with at least three skin tags, aged 24 to 85 years, and 31 healthy controls aged 30 to 70 years, were examined in the present study. The subjects in all the groups were selected with statistically similar Body Mass Index (BMI). Fasting concentrations of plasma glucose, serum lipids including triglyceride, total cholesterol, and high-density lipoprotein cholesterol (HDL) and low-density lipoprotein cholesterol (LDL), HbA1c, and leptin were measured by enzyme-linked immunosorbent assay (ELISA). In addition, serum LDL level was calculated using Friedewald''s formula.

Results:

There was no significant difference in age, sex, BMI, HbA1c, triglyceride, HDL and leptin levels between the groups. Skin tags group showed significantly higher levels of total cholesterol and LDL, when compared with the healthy controls groups (P < 0.01). In addition, regression analysis showed that leptin level was positively correlated to serum triglyceride level (r = 0.265, P = 0.044).

Conclusion:

Total cholesterol and LDL serum levels should be controlled in patients with skin tags. On the other hand, glucose, leptin and HbA1c serum levels may not be as important as is being considered in recent times.     

DIFFUSE PLANE XANTHOMATOSIS IN A PATIENT WITH BUDD-CHIARI SYNDROME AND MONOCLONAL GAMMOPATHY

Diffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds. Histology shows foamy histiocytes in the dermis. Approximately half of the cases are associated with lymphoproliferative disorders. Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow. We present a case of diffuse plane xanthoma in a 62-year-old man who developed normolipemic plane xanthomas coinciding with Budd-Chiari syndrome and monoclonal gammopathy. We review the English-language literature regarding the rare association of xanthomas and Budd-Chiari syndrome.     

RARE ASSOCIATION OF HYPER IgE SYNDROME WITH CERVICAL RIB AND NATAL TEETH

Hyper IgE syndrome (HIES) is a rare immunodeficiency syndrome characterized by a triad of cutaneous abscesses, mostly caused by Staphylococus aureus; pneumonia; and raised IgE levels. Nonimmunological associations include course facial features, multiple bone fractures, joint hyperextensibility, and retained primary dentition. Patients require long-term antibiotic therapy. We report here a classical case of HIES with rare associations of natal teeth, bilateral cervical ribs, and conductive deafness. The patient was being treated with monteleukast and dapsone.     

A Six-Month Prospective Study to Find Out the Treatment Outcome,Prognosis and Offending Drugs in Toxic Epidermal Necrolysis from an Urban Institution in Kolkata

Toxic epidermal necrolysis is the life-threatening dermatological emergency, most often an adverse cutaneous drug reaction with high mortality. A 6-month prospective study was conducted in our institution to find out the offending drugs, to assess the prognosis on admission using SCORTEN: Severity of illness score and to find out the treatment outcome. Anticonvulsants, NSAIDs and sulphonamides are the common offending agents; but in our study, 2 were due to homeopathic medicines. Out of 20 patients, on the date of admission SCORTEN prognostic score was 2 in 11 patients, 3 in 8 patients and 4 in 1 patient. Eighteen patients were treated with dexamethasone intramuscular injection and 2 patients got intravenous immunoglobulin (IVIG). All patients survived without any mortality. Though improvement was slightly faster with IVIG, early administration of corticosteroids was also of encouraging efficacy and should be considered in developing countries due to low cost. No mortality in our study suggests need to validate the SCORTEN index in our country in a large number of patients.     

Congenital calcinosis cutis of the foot

Calcinosis cutis is a rare disease characterized by deposition of insoluble calcium salts in the skin. Subepidermal calcified nodule is a form of idiopathic calcinosis cutis that commonly affects children but rarely presents at birth. Herein we describe a healthy 10-month-old boy who had a solitary hard nodule on the left foot since birth. Surgical excision of the nodule was done and histopathology confirmed the diagnosis of subepidermal calcified nodule.     

Dermatitis Artefacta: A Review of Five Cases: A Diagnostic and Therapeutic Challenge

Dermatitis artefacta (DA) is a self-inflicted dermatological condition where the underlying motive is to assume a sick role. The act of self-harm is to discharge the inner sense of isolation and emotional distress, which is too great to endure. We, hereby, report five interesting cases of DA with varied presentations, using diverse and innovative means for inflicting injury/injuries. Rarity may be attributed to masquerading presentation, leading to misdiagnosis and paucity of awareness among the physicians. Lack of proper identification of the underlying psychiatric disturbances may be the major cause of the loss of follow-ups. Here, we were fortunate enough to identify the emotional need of most of the patients. A flexible, nonconfrontational yet strong therapeutic rapport is required to improve the therapeutic outcomes.     

STUDY OF THE ROLE OF SERUM FOLIC ACID IN ATOPIC DERMATITIS: A CORRELATION WITH SERUM IgE AND DISEASE SEVERITY

Background:

Most atopic dermatitis (AD) patients have elevated serum immunoglobulin E (IgE). Impaired folic acid (FA) metabolism was found to reduce the intracellular methyl donor pool, associated with a higher prevalence of atopy.

Aim:

To assess serum IgE and FA in AD patients and to correlate their levels with the disease severity, and with each other.

Materials and Methods:

Twenty patients with AD were assessed for serum FA and IgE, compared with 20 age- and sex-matched controls. Patients were classified into three groups (mild, moderate, and severe AD) based on clinical severity according to Nottingham index. In both patients and controls, serum IgE was measured using Enzyme-linked immunosorbent assay technique and serum FA was measured using Microparticle Enzyme Immunoassay technique.

Results:

Serum FA levels were lower in AD patients compared with controls, but the difference was not statistically significant. FA levels did not show statistically significant difference among disease severity groups and did not correlate with serum IgE levels. On the other hand, serum IgE levels were significantly elevated in AD patients compared with controls, and among AD patients, its levels were significantly elevated in severe AD compared with mild and moderate disease.

Conclusion:

Serum IgE is useful in assessment of AD severity and activity. FA contribution to AD needs further investigations.     

DISSEMINATED CYSTICERCOSIS WITH HUGE MUSCLE HYPERTROPHY

Cysticercosis is caused by cysticercus cellulose, which is the larva of Taenia solium, the pork tapeworm. The larvae are carried in the blood stream after penetrating the walls of the alimentary tract and they lodge in different tissues like the skin, skeletal muscles, brain, fundus and heart, to cause disseminated cysticercosis. Cases of disseminated cysticercosis have rarely been reported in the literature. They may inhabit the muscles and cause muscular hypertrophy, which, at times, may assume gross proportions. Morbidity is usually caused by the involvement of the central nervous system or the eyes.     

PIGMENTED BASAL CELL CARCINOMA SUCCESSFULLY TREATED WITH 5% IMIQUIMOD CREAM

Basal cell carcinoma (BCC) is the most common malignant skin tumor, amongst which the nodular, nodulo ulcerative and superficial types comprise nearly 80% of all BCCs. Topical Imiquimod, an immune response modifier has been found to be effective in superficial and nodular types of BCC with histological clearance rates of up to 100%. We report our experience of treatment a large pigmented BCC on the face with topical Imiquimod 5% cream.