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.

Controversies in the Management of Cutaneous Adverse Drug Reactions.

Some cutaneous adverse drug reactions (CADRs) are severe life-threatening conditions due to multisystem involvements with a high morbidity and mortality rates ranging from 25 - 70% and require immediate medical care. But there are huge controversies regarding the management because large clinical trials are lacking. Most frequent discussion and division occur regarding the use of systemic corticosteroid as early intervention with corticosteroids controls inflammation. Corticosteroids are potent agents that target several intracellular processes to modify almost all components of inflammatory and immune responses but their impact on the long term disease course is not known. Controlled relapses of rash and hepatitis may occur as corticosteroids are tapered. A chronic HHV6 activation promoted by systemic steroids could explain these relapses. Second important issue is the use of antitubercular drugs (ATD) in case of CADR due to multidrug therapy of ATD. As both the tuberculosis and CADR are life threatening conditions and we can not spare treatment of tuberculosis for CADR, we should come to a conclusion which is not yet decided. In the same way the use of antileprotic MDT in CADR due to MDT raises a similar controversy. So, here we focus on those controversies and discuss the issues.

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.

Drug Reaction with Eosinophilia and Systemic Symptoms: An Update and Review of Recent Literature.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, is also known as drug induced hypersensitivity syndrome and by various other names. It is now recognised as one of the severe cutaneous adverse reaction (SCAR) and can be potentially life-threatening. Historically, it was most frequently linked with phenytoin and was initially described as phenytoin hypersensitivity syndrome. However, it was later found to be caused by various other medications. Anticonvulsants and sulfonamides are the most common offender. Characteristically DRESS has a latent period of 2 to 6 weeks. The pathophysiology remains incompletely understood but involves reactivation of viruses and activation of lymphocyte. It is manifested most commonly with a morbilliform cutaneous eruption with fever and lymphadenopathy. The severity of this syndrome is related to the systemic involvement, which can result in multi-organ failure. Most important step in the management of DRESS is early diagnosis and immediate cessation of the suspected offending drug. Patients of DRESS syndrome should be managed in an intensive care set up for appropriate supportive care and infection control. Topical corticosteroids can give symptomatic relief, but systemic therapy with steroid and other immunosuppressant is usually required.

A clinical rating scale for the assessment of facial aging in Indian population.

BACKGROUND: Estimation of facial aging has assumed growing importance due to the advent of several antiaging therapies. Evidence-based estimation of global facial aging is often necessary, especially for validation of these treatment modalities. Most available methods are expensive and have been used in fair skinned individuals. AIM: We attempted to develop a clinical rating scale for the estimation of global facial aging applied on an Indian population which has brown to black skin. We have also measured the association of this rating scale score with the chronological age. METHODS: Initially, a 14- item summated rating scale was developed with inputs from five dermatologists and a clinical pharmacologist. The rating scale was applied to 105 consenting subjects with healthy facial skin between 30 to 90 years of age. Intra- and inter-rater reliability was assessed. RESULTS: The summated rating score showed a significant positive correlation with the chronological age (Pearson's correlation coefficient 0.834, P < 0.001). We omitted one item from the scale due to a low inter-rater agreement. The resulting 13-item rating scale was internally consistent (Cronbach's alpha: 0.905), with substantial inter- and intra-rater reliability (intraclass correlation coefficient: 0.973 and 0.788, respectively). Principal components and predictive equation for perceptible age were identified on further computation. LIMITATIONS: Participants of this study were limited to a particular ethnic group from West Bengal and other neighboring states of Eastern India. CONCLUSIONS: We have developed and validated a 13-item rating scale for the quantification of global facial aging suitable for Indian (brown to black) skin type. This scale can be utilized effectively for clinical estimation of global facial aging.

Zinc-responsive acral hyperkeratotic dermatosis-A novel entity or a subset of some well-known dermatosis?

BACKGROUND: We are reporting a series of interesting cases, which presented to us with psoriasiform lesions distributed over the acral regions of the body. The cases are unusual because they were resistant to conventional treatment modalities like topical corticosteroids, tacrolimus and oral methotrexate but showed significant improvement on oral zinc therapy. MATERIALS AND METHODS: Ten patients with characteristic clinical features of distinctive hyperkeratotic plaque in the acral areas, who were resistant to treatment by different modalities including potent topical steroids and oral methotrexate, were included for detailed investigations. A proper history was taken and relevant laboratory investigations were done which included blood count, urine, liver function, renal function, hepatitis-C virus serology and serum zinc levels. Patients were followed up every 2 weeks. Histopathological examinations of the lesional tissue were done at baseline and after 6 weeks of therapy. Patients were given oral zinc daily and no other treatment during the 6 weeks course. RESULTS: All our patients were non-reactive to hepatitis-C. Of the ten patients only one patient (10%) showed low titer of serum zinc, another (10%) showed higher zinc level, while the rest of the patients had normal zinc level. Five of our patients had chronic renal failure, one had Grave's disease and the remaining had no associated systemic illness. Histopathology mostly showed hyperkeratosis, acanthosis, prominent granular layer, spongiosis and dermal infiltrate. After 6 weeks of follow up, all patients showed rapid and remarkable therapeutic response with zinc. CONCLUSIONS: We here report a series of patients, discernible because of their uniform clinical presentation of acral hypekeratotic plaques and in showing a noticeable response to zinc. Clinical, histopathological and laboratory investigations were done to rule out diseases of similar morphology including psoriasis, acral necrolytic erythema and lichen simplex chronicus. Authors understand that further studies with greater number cases and more detailed investigations are required to establish exact etio-pathogenesis and nomenclature of this distinct subset of patients.

Eccrine angiomatous hamartoma: late onset facial presentation.

Eccrine angiomatous hamartoma (EAH) is a very rare benign neoplastic condition characterized by hamartomatous proliferation of eccrine glands and accompanying blood vessels and lymphatics. These lesions are more often present at birth or appear during early infancy and childhood and present as solitary nodule or plaque with occasional pain and sweating. They are generally present on the extremities, mostly the palms and soles. We report here a case of EAH in a 20-year-old female who presented with a solitary angiomatous plaque close to the lower eyelid of the right eye with occasional pain and sweating for its rarity.

A case of ichthyosis hystrix: unusual manifestation of this rare disease.

Ichthyosis hystrix is a term used to describe an ichthyosiform dermatosis which is characterized by hyperkeratotic spiny scales mainly over extensor aspects of limbs with palmoplantar keratoderma and occasionally associated with deafness and neurological deficit. It is a rare autosomal dominant form of ichthyosis and very few cases are reported in literature. We are presenting a 46 years-old-male patient of ichthyosis hystrix with unusual presentation. He had lesions mainly over the face and scalp with palmoplantar keratoderma and significant nail changes.

Ichthyosiform large plaque parapsoriasis: report of a rare entity.

Large plaque parapsoriasis (LPP) is an idiopathic, chronic scaly dermatosis classified within parapsoriasis group of diseases, occurring commonly in middle aged patients of all races and geographic regions. LPP and its variants are closely related to the patch stage of mycosis fungoides. The two types of LPP mostly described are the poikilodermatous and retiform parapsoriasis. We are reporting an ichthyosiform LPP for its rarity.

Diogenes syndrome: a case report.

Cessation of normal skin cleansing seen in geriatric or self-neglected patients can cause accumulation of keratinous crusts on the skin. In the extreme end of this spectrum is a condition known as Diogenes syndrome (DS). These patients may have psychiatric disorders like paranoid disorders, mood affection, or temporofrontal dementia. Subjects are mainly the elderly but few cases in younger age group of patients have also been reported. Lesions of DS are usually found over upper central chest, back, and groin. In the young, lesions are mainly found over scalp, face, or arms. Absence of normal skin cleaning causes keratin and dirty debris to accumulate and with time form a thick shell. These debris can be secondarily infected by bacteria, fungus, and so forth. These skin lesions are not usually seen in individual with proper hygiene. We report a case of Diogenes syndrome in a 34-year-old young male patient who had associated schizophrenia.

Keratoacanthoma centrifugum marginatum with atypical scar.

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma (KA). It is characterized by a progressive peripheral expansion and central healing leaving atrophic scar. It is sometimes confused with squamous cell carcinoma (SCC) both clinically and histopathologically. We here report a case of KCM over the extensor aspect of the right forearm in a 57-year-old man with an abnormal looking scar.