Rud's syndrome.

Rud's syndrome is a rare autosomal recessive hereditary disorder characterized by congenital ichthyosis, epilepsy, dwarfism, sexual infantilism, polyneuritis, and macrocytic anemia. We report here an interesting case of this disorder in an 18-year-old girl for its rarity and academic interest.

Genital donovanosis with malignant transformation: An interesting case report.

Donovanosis is a chronic indolent sexually transmitted granulomatous ulceration of genito-inguinal region, caused by Calymmatobacterium granulomatis. It became uncommon due to indiscriminate use of broad spectrum antibiotics. In recent years, much interest is being focused on this condition because genital ulcers facilitate HIV infection. We report an interesting episode of genital donovanosis complicated with squamous cell carcinoma in a middle aged female for its rarity and clinical interest.

The Jarisch-Herxheimer reaction: revisited.

The Jarisch-Herxheimer reaction (JHR) is a transient immunological phenomenon seen commonly in patients during treatment for syphilis, and it manifests clinically with short-term constitutional symptoms such as fever, chills, headache and myalgias, besides exacerbation of existing cutaneous lesions. The complex interplay of its underlying patho-physiological mechanisms continues to elude modern medicine, ever since it was described over a century ago. An increase in the incidence of JHR may be expected among patients co-infected with HIV and other infectious diseases including syphilis. Since this subject has not received much attention in recent literature except for brief mentions in standard textbooks, we felt it important to provide an overview of its various attributes including the current concepts in pathophysiology and management.

Histoid leprosy with erythema nodosum leprosum--a case report.

Histoid leprosy is a variant of lepromatous leprosy characterized by cutaneous and/or subcutaneous nodules and plaques present over an apparently normal skin with unique histopathology and characteristic bacterial morphology. Reactions are uncommon in histoid leposy. Our patient developedtype 2 reaction after initiating MDT for leprosy.

Papillon-Lefevre syndrome with pseudoainhum.

An interesting episode of Papillon-Lefevre syndrome in a 25-year-old female with diffuse palmoplantar keratoderma, periodontitis and pseudoainhum of the toes is reported for academic interest. Her skin lesions improved with topical keratolytics and oral retinoid (acitretin) whereas periodontic problems showed significant improvement with systemic antibiotics and proper implementation of oral hygienic measures. She is undergoing oral rehabilitation with orthodontic surgical procedures.

Combination of surgical avulsion and topical therapy for single nail onychomycosis: a randomized controlled trial.

BACKGROUND: Conventional therapy of onychomycosis is prolonged and often frustrating, which is why combination therapy involving topical, oral and surgical measures has been advocated as the treatment of choice. There are no controlled studies evaluating the efficacy of nail avulsion followed by topical antifungal therapy. OBJECTIVES: To evaluate the efficacy of combined surgical and topical therapy for onychomycosis. METHODS: Forty patients with single nail onychomycosis [28 with distal and lateral subungual onychomycosis, seven with total dystrophic onychomycosis (TDO) and five with proximal subungual onychomycosis] were randomly assigned to four treatment groups. Each group received avulsion of the involved nail, followed by ketoconazole 2% cream without (group I) or with occlusion (group II), or oxiconazole 1% cream without (group III) or with occlusion (group IV). Topical therapies were applied twice daily. The patients were reviewed monthly and treatment was continued until the regrowth of completely normal nail (mycologically negative). In cured cases, further monthly review was carried out for at least 6 months, without any form of therapy. At each visit direct microscopic examination was repeated. RESULTS: There was a high dropout rate, with seven patients (group I), six patients (group II), six patients (group III) and eight patients (group IV) completing the treatment protocol. Out of these, mycological cure was achieved in three (43%) patients in group I, four (67%) in group II, two (33%) in group III and six (75%) in group IV. All the cases of TDO failed to respond to this therapy. Overall, 15 of 27 (56%) patients were cured with this approach. On further follow up, recurrence of onychomycosis was recorded in two patients in group I. No side-effects or long-term complications of the nail avulsion were encountered. Important limitations encountered in the present study included a small sample size, a high dropout rate (32%) and poor patient compliance. CONCLUSIONS: Contrary to earlier reports, surgical nail avulsion with topical antifungal agents was not found to be a very encouraging modality for the treatment of onychomycosis. Both oxiconazole and ketoconazole delivered comparable results. Occlusion improved the treatment outcome, although the difference was not statistically significant. As a subtype, TDO showed poorest response. Surgical nail avulsion followed by topical antifungal therapy cannot be generally recommended for the treatment of onychomycosis.

Cutaneous tuberculosis in children and adolescents: a clinicohistological study.

BACKGROUND AND OBJECTIVES: Resurgence of tuberculosis (TB) in the era of human immunodeficiency virus (HIV) has rejuvenated the interest in this global health problem. Cutaneous TB, an important extra-pulmonary form in children, is commonly seen in our dermatological practice. As detection of acid-fast bacillus (AFB) on smear or culture is not always positive, histopathology is necessary to help in diagnosing and classifying the variants of skin TB. The current study was conducted to analyse the clinicopathological characteristics of cutaneous TB in children and adolescents. MATERIALS AND METHODS: This prospective study included 103 patients (age<19 years). A detailed history and clinical examination was followed by complete investigative work up including fine needle aspiration cytology and culture. Histopathological evaluation was performed specifically noting the epidermal and dermal features. The patients were followed up regularly for one year after the start of treatment. RESULTS: The different patterns of cutaneous TB seen were, scrofuloderma 38 (36.9%), lichen scrofulosorum 34 (33%), lupus vulgaris 22 (21.3%), TB verrucosa cutis 4 (3.9%), papulonecrotic tuberculid 4 (3.9%) and erythema nodosum 3 (2.9%). Systemic associations were seen in 55 (53.4%) patients, namely TB lymphadenitis in 30 (29.2%), pulmonary TB in 13 (12.6%), abdominal TB in 6 (5.8%) and TB arthritis in 6 (5.8%). The histopathological corroboration of clinical diagnosis was seen in 65.7% of cases of scrofulodermas, 72.7% of cases of lupus vulgaris and 67.6% of cases of lichen scrofulosorum. CONCLUSIONS: A large spectrum of clinical patterns and histological characteristics of cutaneous TB exists in children. Lichen scrofulosorum is more commonly seen in comparison to adults. Systemic involvement was a feature in a major proportion of our patients.

Simultaneous detection of Neisseria gonorrhoeae and Chlamydia trachomatis by PCR in genitourinary specimens from men and women attending an STD clinic.

Neisseria gonorrhoeae and Chlamydia trachomatis are the two most common bacterial sexually transmitted infections that manifest primarily as urethritis in males and endocervicitis in females, though the infection may be asymptomatic especially in women. Since complications may occur in untreated symptomatic and asymptomatic infected individuals, early diagnosis and treatment of infected individuals is required to prevent severe sequelae and spread of these diseases. Recently molecular amplification assays like Polymerase Chain Reaction (PCR) and Ligase Chain Reaction (LCR) have been found to be highly sensitive and specific methods for detection of N. gonorrhoeae and C. trachonmatis not only in urethral and cervical specimens but also in urine. The objective of this study was to screen male and female Sexually Transmitted Disease (STD) clinic attenders, with and without symptoms suggestive of urethritis and cervicitis for presence of N. gonorrhoeae and C. trachomatis using a multiplex PCR based assay, to compare its performance with culture for N. gonorrhoeae and Direct Fluorescent Antibody (DFA) staining for C. trachomatis and also to compare the efficacy of PCR test performed on urine and genital swab specimens collected from this high risk group. Genital specimens and urine was collected from STD clinic attenders. N. gonorrhoeae and C. trachomatis was detected in genital specimens by culture and DFA respectively. Multiplex PCR was used to detect N. gonorrhoeae and C. trachomatis infection in both genital and urine specimens. Among men with urethritis, N. gonorrhoeae was detected in 70% by culture and 77% by PCR, while C. trachomatis as detected in 7.5% by DFA and 17.5% by PCR. Among females with endocervicitis, N. gonorrhoeae was detected in 7.7% by culture and 30.7% by PCR, while C. trachomatis was detected in 7.7% by DFA and in 15.4% by PCR. None of the asymptomatic males were positive for N. gonorrhoeae and C. trachomatis by conventional methods, while 43.9% were positive for N. gonorrhoeae and 7.5% for C. trachomatis by PCR. Fifty per cent of asymptomatic women were positive for C. trachomatis by PCR alone. We encountered PCR positive but culture/DFA negative results and also PCR negative but culture/DFA positive results. In view of this a single PCR test cannot be used for diagnosis and treatment of N. gonorrhoeae and C. trachomatis infection unless confirmed by a second test.

Suction blister epidermal grafting for management of eyelid vitiligo: special considerations.

BACKGROUND: Suction blister epidermal grafting is an established technique for the treatment of resistant and stable vitiligo especially over small and cosmetically important areas like the face. However, certain areas, such as lips and eyelids, require special considerations because of their anatomic peculiarities. PATIENTS AND METHODS: The current study was carried out on six patients of stable eyelid vitiligo, not responding to medical treatment. Suction blister was obtained from the thigh using the suction apparatus and the roof was grafted on to the dermabraded eyelid. Certain precautions were taken during the procedure, which will be highlighted in the current study. RESULTS: Response was excellent in all the patients in 3 to 6 months, with no complications. CONCLUSIONS: If carried out properly, suction blister technique is the procedure of choice for cosmetically important sites like eyelids.

En bloc excision of proximal nail fold for treatment of chronic paronychia.

BACKGROUND: Chronic paronychia is a very recalcitrant dermatosis, which is particularly prevalent in housewives. Medical treatment for this condition is unsatisfactory in a significant number of cases. Surgical approach forms an important part of management, however, this area has received little attention. Various surgical approaches have been tried in the past but a comparative analysis has not been attempted. OBJECTIVES: The present study aims at assessing the efficacy of en bloc excision of proximal nail fold (PNF). Moreover, a comparative analysis has been undertaken to assess whether or not simultaneous nail plate avulsion improves the treatment outcome. METHODS: Thirty patients of chronic paronychia with nail plate irregularities were randomly divided into two treatment groups (15 patients each). After a detailed evaluation, en bloc excision of PNF with or without nail plate removal was performed. Postoperative measures were advised and the patients were kept under regular follow-up thereafter. Assessment of postoperative complications was also performed. RESULTS: Twelve patients in group I and 13 patients in group II completed the treatment protocol. Of these, 70% of patients were cured in group II (en bloc excision with nail avulsion) whereas only 41% were cured in group I (en bloc excision without nail avulsion). CONCLUSION: En bloc excision of the PNF is a useful method in recalcitrant chronic paronychia. Simultaneous avulsion of the nail plate improves the surgical outcome. Strict avoidance of irritant exposure is necessary to ensure complete treatment and prevent recurrence.

Diagnosis of nail psoriasis: importance of biopsy and histopathology.

BACKGROUND: Involvement of the nail is quite common in psoriasis and at times may be the sole diagnostic clue. However, the histopathology of nail psoriasis has not been adequately evaluated. A confirmation of the diagnosis is required in cases suspected to have nail psoriasis in order to plan long-term therapy. OBJECTIVES: To assess the diagnostic significance and safety profile of nail biopsy in cases with nail psoriasis. METHODS: Clinical and mycological features were studied in 42 patients with nail psoriasis. Of these, 22 patients gave consent for nail biopsies to be taken and the histopathological changes were assessed. RESULTS: Males were affected more commonly (57%) with a peak incidence in the age group of 10-20 years (29%). Distal onycholysis, discoloration of nail plate, subungual hyperkeratosis and fine nail pitting were the predominant clinical features. In the 22 biopsies done, hyperkeratosis with parakeratosis (91%) was found to be the most common and hypergranulosis was the least common histological finding (36%). Clinicohistological correlation was possible only in 55% cases. Periodic acid-Schiff (PAS) staining was done for all biopsies. CONCLUSIONS: Histopathological examination of nails is a valuable diagnostic aid, especially in the absence of skin lesions. Examination of the PAS-stained sections is necessary before making a histological diagnosis of nail psoriasis because onychomycosis and psoriasis may show similar histology.

IL-10 promoter single nucleotide polymorphisms are significantly associated with resistance to leprosy.

The minor haplotype -3575A/-2849G/-2763C in IL-10 promoter has been defined as a marker of disease resistance to leprosy and its severity in Brazilian population. Our investigation of six single-nucleotide polymorphisms (SNPs) in IL-10 promoter in 282 Indian leprosy patients and 266 healthy controls by direct PCR sequencing, however, showed that the extended haplotype: -3575T/-2849G/-2763C/-1082A/-819C/-592C was associated with resistance to leprosy per se and to the development of severe form of leprosy, using either a binomial (controls vs cases, P=0.01, OR=0.58, CI=0.37-0.89) or ordinal (controls vs paucibacillary vs multibacillary, P=0.004) model. Whereas, IL-10 haplotype -3575T/-2849G/-2763C/-1082A/-819T/-592A was associated with the risk of development of severe form of leprosy (P=0.0002) in contrast to the minor risk haplotype -3575T/-2849A/-2763C in the Brazilian population. The role of IL-10 promoter SNPs in Brazilian and Indian population strongly suggests the involvement of IL-10 locus in the outcome of leprosy.

Congenital becker nevus with lichen planus.

We report an interesting and rare association of congenital Becker nevus with lichen planus occurring in an 11-year-old boy. Both conditions were confirmed histopathologically.

Leprosy with neurofibromatosis--a diagnostic dilemma.

The coexistence of leprosy with neurofibromatosis is a rare finding and can pose a diagnostic dilemma. Neurofibromatosis coexisting with borderline tuberculoid leprosy has previously not been reported. We report such a case in a 13-year-old boy where biopsy of clinically uninvolved nerve revealed the presence of acid-fast bacilli. A careful diagnostic workup is needed in such cases to ensure proper treatment. Both disorders affect Schwann cells and their relationship merits further consideration.

Cutaneous relapse in Hodgkin's disease: a case report.

BACKGROUND: Skin involvement in Hodgkin's disease is rare, can be seen in advanced stages of the disease and indicates a poor prognosis. CASE: A young male presented with multiple nodular lesions on the chest wall and matted cervical lymph nodes. Aspiration smears from skin lesions showed atypical mononuclear cells with a prominent nucleolus, many lymphocytes and plasma cells. Smears from the lymph nodes showed classical Reed-Sternberg cells in a polymorphous background. The cytologic diagnosis of Hodgkin's lymphoma was entertained and later confirmed on skin biopsy. Past history revealed that the patient had been diagnosed with Hodgkin's disease and treated for it 2 years earlier, but had been lost to follow-up during treatment. CONCLUSION: Cutaneous Hodgkin's disease should always be considered in smears from skin lesions showing atypical mononuclear cells in a polymorphous background, even in the absence of a definitive clinical diagnosis at the time of presentation.