Clinicopathological Study of Primary Cutaneous Amyloidosis in a Tertiary Care Center of Eastern India Reveals Insignificant Association with Friction, Scrubbing, and Photo-Exposure: How valid is the "Keratinocyte Hypothesis"?

INTRODUCTION: Primary cutaneous amyloidosis (PCA) can be classified into four principal categories: macular amyloidosis, lichen amyloidosis, biphasic, and nodular amyloidosis. Some unusual variants such as widespread diffuse hyperpigmentation without papules, poikiloderma like involvement, lesions following Blaschko's line, etc., have also been reported. However, not much data are available regarding the demography, epidemiology, clinical patterns, and distribution and histopathological findings, especially from the eastern part of India. AIMS: We conducted a cross-sectional, institution-based study to evaluate clinicopathological pattern and factors of PCA in eastern India. MATERIALS AND METHODS: We recorded clinical and histopathological findings of 100 consecutive patients of PCA presenting to a tertiary care institution of Kolkata in eastern India. RESULTS: We found female patients of PCA outnumber male (M:F =1:1.9) with majority of patients being young adults (56%) between 20 and 40 years of age. More than half (54%) of the patients were pruritic. The severity of pruritus is significantly more associated with lichenoid and biphasic variants over macular amyloidosis. Positive family history was recorded in 17% of cases. Macular variant was the most common variant constituting 48% of the total PCA. We also found that the association with history of friction and scrubbing and photo-exposure were statistically insignificant. However, duration of the disease has statistically significant association with the disease morphology. Congo red stain showed these deposits as reddish orange substance in 28 patients out of 64 patients' samples on which Congo red could be performed. CONCLUSION: Our study revealed that many concepts of pathogenesis of PCA including friction and photoexposure might have lesser importance. However, morphological types were significantly associated with the duration of the disease and symptom severity.

Topical Steroid Damaged/Dependent Face (TSDF): A Study from a Tertiary Care Hospital in Eastern India.

BACKGROUND: Awareness against abuse of topical corticosteroids (TC), especially over the face, has been going on for last 5 years in India. In spite of that we are getting lots of cases in our hospitals. AIMS: The aims of this study were to ascertain the demographics, magnitude and clinical features of TC misuse on the face among the dermatology outpatient department (OPD) attendees and to analyze its causes. METHODS: This study was conducted in a tertiary care medical center of eastern India. Patients with relevant facial dermatoses were asked about their current use of topical formulations and confirmed to be TSDF were included in the study. RESULTS: A total of 748 patients with facial dermatoses were screened, of which 271 (36.22%) were using TC. Of them mostly young adults between 20 and 29 years (37.10%) were using TC. Average duration between starting of use of medication and the onset of symptoms was 5 months. Ninety-eight (36.16%) patients were using topical corticosteroid for the treatment of acne and 74 (27.30%) were using as depigmenting cream. About 108 (39.85%) patients bought medicine over the counter being recommended by pharmacist/shop owner. Rosacea like features with photosensitivity was the most common adverse effect found in 79 (29.15%) patients whereas comedonal acne/acne exacerbation were found in 68 (25.09%) patients. Most of them (227, 83.76%) were unaware about the side effects of steroids. CONCLUSIONS: TC misuse in patients with facial dermatoses is still quite common even after efforts to grow the awareness among population.

Stamping a Case of Cutaneous Adverse Drug Reaction: Proving Beyond Causality Assessment.

Different types of skin testing with a suspected drug have been reported to be helpful in determining the cause of cutaneous adverse drug reactions (CADRs). It is of utmost importance for practicing dermatologists to have a first hand knowledge of different types of skin tests available in a case of CADR. In each suspected case, a detailed investigation with the suspected agent and correlation of the positive skin reaction with clinical variant of CADR is advocated. Drug skin tests are performed 6 weeks to 6 months after complete healing of the CADR. Drug patch tests are performed similar to the methods used in patch testing in studying contact dermatitis. The commercialized form of the drug used by the patient is tested at 30% dilution. The pure drug is tested at 10% dilution. In severe CADR, drug patch tests should be performed at lower concentrations. It is also of value to test on the most affected site of the initial CADR.

Acute Generalised Exanthematous Pustulosis: An Update.

Acute generalised exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction and is attributed to drugs in more than 90% of cases. It is a rare disease, with an estimated incidence of 1-5 patients per million per year. The clinical manifestations characterised by the rapid development of sterile pustular lesions, fever and leucocytosis. Number of drugs has been reported to be associated with AGEP, most common being the antibiotics. Histopathologically there is intraepidermal pustules and papillary dermal oedema with neutrophilic and eosinophilic infiltrations. Systemic involvement can be present in more severe cases. Early diagnosis with withdrawal of the causative drug is the most important step in the management. Treatment includes supportive care, prevention of antibiotics and use of a potent topical steroid.