A 10-year retrospective descriptive study on pure neuritic leprosy from a tertiary referral centre

Context: Pure neuritic leprosy is a risk factor for grade 2 disability owing to the early nerve damage. Aims: To study the clinical patterns of neuritic leprosy, to determine the percentage of patients manifesting grade 2 disability at the time of diagnosis and to identify any risk factors for the same. Settings and Design: Retrospective descriptive study from previous case records of pure neuritic leprosy patients who attended a tertiary centre from 1st July 2007 to 30th June 2017. Subjects and Methods: Data on patients who satisfied the World Health Organization (WHO) cardinal criteria for diagnosis of leprosy, who had no skin lesion of leprosy and had acid-fast bacilli negative status on skin smears were collected using a pre-set proforma. Statistical Analysis Used: The Chi-square test was used to assess statistical significance and logistic regression model was applied to avoid the effects of confounding factors. Results: A diagnostic delay of >1 year was observed in 44% patients. At the time of diagnosis, grade 2 disability was documented in 60 (80%) of patients. No statistically significant risk factor was identified for grade 2 disability. Limitations: Retrospective nature and the study conducted in a tertiary care centre not reflecting the status in the community were the limitations. Conclusions: Grade 2 disability noted in 80% of patients points to the inherent nature of disease to cause early nerve damage. Diagnostic delay of >1 year documented in 44% of patients underscores the diagnostic challenges in the absence of skin lesions

Severe cutaneous adverse drug reactions: A clinicoepidemiological study

Background: Drug eruptions range from transient erythema to the life threatening severe cutaneous adverse reactions (SCAR) that encompass Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), acute generalized exanthematous pustulosis (AGEP) and drug reaction with eosinophilia and systemic symptoms complex (DRESS). Aims and Objectives: To study the clinical and epidemiological aspects of cutaneous adverse drug reactions (CADR). Materials and Methods: Ethical clearance was obtained from the institutional ethics committee. All patients admitted in the Dermatology ward of our tertiary care hospital with CADR (those who fit in the category of probable or possible drug reaction as per WHO casuality assessment) from first September 2011 to 31 st August 2012 were included in this cross sectional study after obtaining written informed consent. The drug reaction patterns observed in the study population were determined and the common offending drugs were identified. Results: In the study, population of males outnumbered females and the majority were between 46 and 60 years of age. The commonest reaction pattern observed was SJS- TEN spectrum of illness and aromatic anticonvulsants were the common offending drugs. Prompt withdrawal of the culprit drug and administration of systemic steroids with or without I/V Ig reverted the adverse reaction in all except one. Conclusion: Severe drug reactions predominated as the study population was comprised of inpatients of a tertiary referral centre. Though; previous authors had reported a mortality rate of up to 20% in DRESS, all our patients with this reaction pattern, responded well to treatment. The mortality rate among TEN cases was much lower than the previous reports. Early diagnosis, prompt withdrawal of the suspected drug, careful monitoring for development of complications and immediate intervention can improve the prognosis of CADR

Restrictive dermopathy: Report of two cases

Restrictive dermopathy is a rare entity that is fatal in the neonatal period itself. The rigidity of the skin leads to erosions, contractures, and restriction of respiratory movements. Diagnosis is often made clinically with classical features such as low-set ears, micrognathia, small, and persistently open fixed “o”-shaped mouth, translucent, shiny, rigid skin with prominent superficial blood vessels, and pseudocontractures of limb joints. We report two cases of restrictive dermopathy observed in our center within 2 years period and suggest that this condition may not be as rare as believed

Pigmented purpuric dermatosis or mycosis fungoides: A diagnostic dilemma

Pigmented purpuric dermatoses (PPD), a group of vascular disorders with variable clinical picture is reported in all races and age groups with a male predilection. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression of PPD to cutaneous T-cell lymphoma. The diagnostic dilemma is compounded by PPD manifesting histological similarity to mycosis fungoides. Currently, it is believed that PPD with monoclonal T-cell population is more likely to progress to malignancy. We report a 31-year-old male patient who presented with the lichenoid clinical variant of PPD lesions that mimicked mycosis fungoides on histopathology. Gene rearrangement studies identified a polyclonal T-cell population. The patient responded to photochemotherapy, which is beneficial in both PPD and mycosis fungoides. Our case signifies the limitations of current diagnostic modalities in accurately distinguishing PPD from cutaneous lymphoma. Data on disease progression in similar cases may enable us to formulate better diagnostic definitions

Sporadic Case of dyschromatosis universalis hereditaria showing moderate response to narrow-band ultraviolet-B

Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis where the affected manifests mottled pigmentation with intermingled hyper- and hypo-pigmented macules. On most occasions, the lesions begin on limbs and then extend to trunk. Most of the cases reported in literature are from Japan. It is rarer in Indians. In this report, we describe a 12-year-old male child with DUH, who showed response to narrow-band ultraviolet-B therapy