Nailfold capillaroscopy as a prognostic marker for connective tissue diseases: An observational clinico-epidemiological study.

Background Nailfold capillaroscopy (NFC) is useful in the diagnosis and assessment of various connective tissue diseases. In this study, NFC findings were studied in patients of systemic sclerosis (SS), systemic lupus erythematosus (SLE) and dermatomyositis. Aims To study the nailfold capillaroscopic findings in patients with connective tissue disorders, their correlation with disease severity, and changes in findings following treatment or disease progression. Methods This observational prospective time-bound clinico-epidemiological study was conducted in 43 patients over 20 months at Topiwala National Medical College and BYL Nair Ch. Hospital, Mumbai. NFC was performed using the polarising mode of a USB 2.0 video-dermatoscope in all 10 fingernails at 50X and 200X. It was repeated at three follow-up visits to look for changes in findings. Results Among SLE patients, eleven (52.4%) had non-specific NFC patterns and eight (38.1%) had SLE patterns. Among systemic sclerosis patients, eight (42.1%) cases had active and late SS patterns respectively, while one (5.3%) each had SLE, non-specific and early SS patterns. After three follow-ups, 10 out of 11 (90.9%) cases with improvement in NFC also showed clinical improvement; this figure was significantly more than 11 out of 23 (47.8%) cases who had no change in NFC but showed clinical improvement. Two of the three dermatomyositis patients showed a non-specific pattern while one showed a late SS pattern at baseline. Limitations A larger sample size would have yielded results with more validity. Standardisation of the interval between baseline and last follow-up to equal to or more than six months would have yielded results with more accuracy. Conclusion Capillary findings change significantly over time and reflect changes in the clinical status of patients of both SLE and systemic sclerosis, and hence are an important prognostic marker. Rather than an overt change in NFC pattern, reduction or increase in abnormal capillaries is a better predictor of change in disease activity.

Factors affecting the duration of phase I of dexamethasone - cyclophosphamide pulse therapy.

BACKGROUND: The introduction of dexamethasone-cyclophosphamide pulse (DCP) therapy for the pemphigus group of disorders by Pasricha has revolutionized the therapy for pemphigus. There are very few studies regarding factors affecting duration of phase I of the DCP. AIMS: Our purpose was to study the relationship between various factors and duration of the phase I. METHODS: A retrospective study of 98 patients of pemphigus on Dexamethasone Pulse therapy was conducted. Patients were classified according to duration of Phase 1 as those with phase I less than 6 months and those more than 6 months and analyzed for variable factors affecting duration of phase I. RESULTS: Disease severity in pemphigus significantly prolonged the duration of phase I of DCP. Longer duration was also observed in patients on concurrent oral steroid therapy (both statistically significant). CONCLUSION: The findings from our study help us to address patient expectations and apprehensions regarding duration of therapy. A detailed understanding of the various patient and disease related factors responsible for affecting Phase I duration will help in better management of the patient, and the disease.

Subungual hyperkeratosis nail biopsy: a better diagnostic tool for onychomycosis.

BACKGROUND: Onychomycosis is one of the most common nail disorders. Mycological examination by potassium hydroxide (KOH) mount and fungal culture is the most commonly used diagnostic method. However, it is associated with a low sensitivity. AIMS: To evaluate the technique of subungual hyperkeratosis nail biopsy in diagnosing onychomycosis in HIV-infected and immunocompetent adults and compare it with mycological examination. METHODS: 34 HIV-positive patients who presented clinically with onychomycosis were recruited in the study from the beginning. There was no screening done for patients with onychomycosis. This has been clarified in manuscript under the heading of methods. RESULTS: All the fungal cultures yielded dermatophytes correlating with the biopsy findings. Only hyphal form of fungus was detected in KOH examination, indicating it was not a contaminant. Clinical types of onychomycosis are stated in discussion. CONCLUSIONS: PAS stain of subungual hyperkeratosis nail biopsy was the most sensitive in the diagnosis of onychomycosis in both HIV-infected and non-infected groups.

Trigeminal trophic syndrome: a rare entity.

Trigeminal trophic syndrome is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a classic triad of anaesthesia, paraesthesia, and a secondary persistent or recurrent facial ulceration. We describe a 60 year-old woman who developed this syndrome as a sequel to the gasserian ganglion block for trigeminal neuralgia. She had also developed melasma within 1 year. A remarkable benefit was achieved by proper patient education and topical antibiotics which led to the healing of all ulcerations within 4 weeks. In the case reported here, the diagnosis of the trigeminal trophic syndrome was made primarily as a result of the physician's experience with the syndrome previously.

Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin.

Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis associated with connective tissue disorders, 3) in some instances of pseudoxanthoma elasticum (PXE), disease-specific calcified elastic tissue is extruded, producing a clinical picture indistinguishable from other types, may also be seen in patients undergoing hemodialysis and 4) EPS induced by long-term treatment with D-penicillamine is observed in patients suffering from Wilson's disease. Long term D-penicillamine therapy causes an alteration in the dermal elastic tissue. D-penicillamine induced EPS has a distinctive histopathologic feature - serrated appearance of elastic fibers due to perpendicular budding from their surface giving a "lumpy-bumpy" look. D-penicillamine induced elastic fiber alteration may not always manifest clinically as EPS. We report a case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features.

Subcutaneous phaeohyphomycosis caused by Cladophialophora boppii.

Subcutaneous phaeohyphomycosis is an infection of the skin and subcutaneous tissue, caused by dematiaceous fungi. An adult male presented with a history of multiple reddish nodules over the face and hands. Histopathological examination of the skin biopsies showed a dense granulomatous infiltrate of macrophages, containing intracytoplasmic basophilic bodies throughout the dermis. Gomori methenamine-silver stained sections revealed yeast cells within macrophages. Multiple cultures on Sabouraud's dextrose agar grew Cladophialophora boppii. The patient was treated with oral itraconazole for a year and the response monitored with dermal ultrasound. This is the first case report of subcutaneous phaeohyphomycosis caused by Cl. boppii in India.

Acroangiodermatitis of mali: a rare vascular phenomenon.

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is an unusual, benign condition which clinically presents as purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. It resembles aggressive conditions like Kaposi's sarcoma and requires histopathological examination for its diagnosis. We report two such cases of acroangiodermatitis. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red blood corpuscles (RBCs), hemosiderin deposits, and hyperplastic granulation tissue. Both were treated with oral antibiotics and topical steroids. The ulcers showed a good response within 2 months of treatment.

Photodynamic therapy in dermatology.

Photodynamic therapy is a new modality of therapy being used for the diagnosis and treatment of many tumors. It is now being increasingly used for skin tumors and other dermatological disorders. With its range of application it is certainly the therapy of the future. Its mechanism of action is by the Type II photo-oxidative reaction. The variables are the photosensitizer, the tissue oxygenation and the light source. It has been used to treat various disorders including Bowen's disease, actinic keratoses, squamous cell carcinomas, basal cell carcinomas, and mycosis fungoides. The side-effects are fortunately mild and transient. Newer photosensitizers like methyl aminolevulinate hold a lot of promise for better therapy.