Cutaneous involvement in angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin's nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia, and cutaneous involvement. We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression.     

Lymphocytic Thrombophilic Arteritis: An Enigma

A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report.     

SCALP MENINGIOMA

Primary extracranial meningiomas occur very rarely. We present a rare case of extracranial meningioma of the transitional variant which was excised satisfactorily. There was no suggestion of any connection to the intracranial compartment or cranial nerves. The underlying galea was uninvolved, suggesting the true extracranial nature of this tumour. This rare diagnosis should nonetheless be kept in the differential diagnosis of scalp tumors.     

MYXOID NEUROTHEKEOMA: A RARE SOFT TISSUE TUMOR OF HAND IN A 5 MONTH OLD INFANT

Myxoid Neurothekeoma is a rare benign nerve sheath tumor, commonly seen in young females. Patients usually present with a small nodule in different anatomical sites, commonly involving the face and the upper limb. We present the case of a five-month-old boy, who presented with a nodule on the left thumb. Punch biopsy and immunostaining confirmed the diagnosis of myxoid neurothekeoma. We believe this is the first reported case of myxoid neurothekeoma below 12 months of age.     

Eccrine Spiradenoma in Knee

Eccrine spiradenoma is an uncommon benign adnexal tumor of the eccrine sweat glands. Although it can occur at any age, it is most common in young adults without any sex predilection. Malignant transformation is rare, presenting as rapid increase in size of a long-standing lesion. Here, we report a case of eccrine spiradenoma in a 35-year-old man who presented with swelling over the right knee, with cytological atypia but no recurrence until date.     

Interleukin-1 Gene Polymorphisms and their Relation with NFκB Expression and Histopathological Features in Psoriasis

Background:

Psoriasis is a chronic inflammatory disease driven by exaggerated production of pro-inflammatory cytokines and interleukins. Various genetic polymorphisms including IL-1 are implicated in pathogenesis of psoriasis. The exact role of IL-1 gene polymorphisms and their interaction with NFκB is not yet determined. We aimed to study various genetic polymorphisms of IL-1 in psoriasis and their influence on NFκB and histopathological features.

Materials and Methods:

112 newly diagnosed cases of psoriasis vulgaris were included in this prospective study. Histology was done on sections and genotyping was done for the IL-1β and IL-1 receptor antagonist (IL-1RA) genetic polymorphisms. In addition, NFκB immunostaining was performed on 89 sections and the intensity of staining was evaluated in the epidermis, basal cells, and the lymphocytes.

Results:

A strong association of IL-1β 511 C/T polymorphism was found with both genotypes and alleles in psoriasis. A strong correlation was also detected between the IL-1β genotype and the grade of NFκB immunostaining in the epidermis (P = 0.012). The grade of NFκB lymphocyte staining showed a strong correlation with the IL-1RA genotype (P = 0.025) but not with the IL-1β genotype (P = 0.226). The genetic polymorphisms did not show any correlation with the histological features.

Conclusions:

IL-1 genetic polymorphisms may not play a very direct role in pathogenesis of psoriasis. However, their interaction with NFκB appears to be a significant factor in this direction as NFκB is activated by pro-inflammatory genetic polymorphisms and therefore may influence the severity of psoriasis.     

Primary Cutaneous Synovial Sarcoma: An Extremely Rare Report of Superficial Synovial Sarcoma

Synovial sarcoma is a type of malignancy which usually occurs near the joints of the arm, neck, or leg. It is a sarcoma of soft tissue and accounts for 5–10% of all adult soft tissue sarcomas in the world. We present a case with primary superficial cutaneous synovial sarcoma without involvement of the underlying knee joint. It is a very rare condition, and to the best of our knowledge, it is the second report of this topic. Although it is rare among soft tissue tumors, the dermatologists should have precise attention to this skin tumor, as early diagnosis is associated with lower metastatic rate and therefore better prognosis.     

A Curious Case of Sweating Blood

Hematohidrosis is a very rare condition in which an individual sweats blood. It may occur in an individual who is suffering from extreme levels of stress. Various causative factors have been suggested like component of systemic disease, vicarious menstruation, excessive exertion, psychogenic, and unknown causes. Fear and intense mental contemplation are the most frequent causes. It may also occur in bleeding disorders. We here report a case where bloody sweat was discharged from the forehead, face, and body episodically in a 12-year-old healthy girl with no bleeding disorder or any other underlying cause. All investigations done were within normal limits, except low intelligent quotient and loss of insight. The patient was given atropine sulphate transdermal patch with marked improvement in severity.     

Progressive and extensive ulcerations in a girl since 4 months of age: the difficulty in diagnosis of pyoderma gangrenosum

A female child developed multiple, progressive, therapy-resistant, painful large ulcers, vesicles, and pustules since her 4 months of age. The ulcers were large, some even measured more than 8 cm; most had violaceous undermined margin with surrounding erythematous halo, raw and crusted surface and were distributed extensively over scalp, face, ear, trunk, buttocks, thigh, legs, dorsum of hands, and feet without any mucosal involvement. After detail clinical examination and investigation, it was diagnosed as a case of pyoderma gangrenosum. Extensive search did not reveal any systemic abnormality or detect any infective etiology. The case highlights the problems of diagnosis of multiple ulcers at very early age.     

Paraganglioma-Like Dermal Melanocytic Tumor

Paraganglioma-like dermal melanocytic tumor (PDMT) is a rare subtype of benign dermal melanocytic tumor, first described in 2004. Its histopathologic features resemble those of paraganglioma, showing presence of a distinctive partitioning of the tumor into small and large packets, nests, or short cords by delicate fibrous septa (zellballen pattern). But the immunostaining characteristics are those of melanocytic lesions, as PDMT express S-100, melan A, HMB 45 and lack pancytokeratin markers. It has a benign course, although a lesion of low malignant potential cannot be excluded. We describe a case of 60-year-old female who presented with three PDMT lesions on her right leg. To the best of our knowledge this is the first case reported from India.     

SCLEREDEMA DIABETICORUM WITH UNUSUAL PRESENTATION AND FATAL OUTCOME

We present a case of Scleredema Diabeticorum (SD) in a patient with diabetic neuropathy and restrictive respiratory disease with unusual skin lesion distribution. The onset of dermatologic symptoms heralded a progressive respiratory disease with constrictive component. Painful diabetic neuropathy was noteworthy and difficult to relieve. Predominantly, distribution of the skin lesions on the thighs makes the case exceptional. T2-weighted MRI showed abnormal hyperintensities along the muscles of the thighs in correspondence with the skin lesions. Gait and respiratory symptoms progressively worsened. After a transient remitting period, he developed sudden shortening of breath, arrested and expired at home.The atypical distribution of the skin lesions with further involvement of underlying muscles plus concomitant polyneuropathy and respiratory constrictive disease with sudden death is quite unusual and aggressive presentation of SD.     

Xanthelasma Palpebrarum with Arcus Cornea: A Clinical and Biochemical Study

Background:Xanthelasma palpebrarum (XP) is characterized by sharply demarcated yellowish flat plaques on upper and lower eyelids. It is commonly seen in women with a peak incidence at 30–50 years. It is also considered as the cutaneous marker of underlying atherosclerosis along with the disturbed lipid metabolism. XP and corneal arcus are associated with increased levels of serum cholesterol and low-density lipoprotein (LDL) cholesterol.Results:Total 49 patients of XP, 81.6% were females. Maximum, 35% patients were among 50–60 years of age and 69.4% were homemakers by occupation. The average lipid values were-cholesterol 210.57 mg%, triglyceride 123.06 mg%. LDL 142.79 mg% and VLDL 30.95 mg% among patients of XP. Arcus cornea was found in 20% cases of XP.Conclusions:Patients of XP requires proper investigation at the onset and regular follow-up thereafter for any altered lipid profile and early diagnosis of coronary artery disease.     

Alveolar Soft Part Sarcoma: A Rare Diagnosis

Alveolar soft-part sarcoma (ASPS) is an extremely rare disease arising from connective tissues with a propensity for recurrence and metastasis. Clinically, it can be confused with hemangioma or arterio-venous malformations. Thus, a high index of suspicion and histopathological examination are required to make a definitive diagnosis. We report a case of recurrent ASPS in a young female with multiple sites involvement without any features of metastasis who has been treated with excision of the symptomatic lesions followed by chemotherapy.     

Acanthosis nigricans associated with transitional cell carcinoma of the urinary bladder

An elderly man from the region of Ladakh presented with recurrent episodes of lower respiratory tract infection, rapidly progressive Acanthosis nigricans, Acanthosis palmaris and plantar keratoderma. Detailed investigations revealed underlying metastatic transitional cell carcinoma of the bladder. This case is being reported for its rarity in the literature.     

Altered Serum Uric Acid Level in Lichen Planus Patients

Background:Lichen planus (LP) is a common disorder whose etiopathogenesis is not clear. Recently, it has been suggested that increased reactive oxygen species (ROS) play important roles in the underlying mechanism of LP.Objectives:The principal aim of this study was to evaluate serum uric acid (UA) levels as a measure of the antioxidant defense status in LP patients.Methods:Serum UA levels were determined in 58 LP patients and 61 controls.Results:Serum UA levels were significantly decreased in patients with respect to controls. Moreover, serum UA level was decreased according to increasing duration of disease.Conclusions:The results of our study suggest that LP is associated with decrease of UA levels in serum. UA may be a potential, useful biomarker of antioxidant status in LP for elaboration of treatment strategy and monitoring.     

Cutaneous Manifestations of Multiple Myeloma

Multiple myeloma (MM) is a proliferative disorder of plasma cells which produce abnormal immunoglobulin proteins. Skin involvement is rarely found in this disorder. They are either specific or nonspecific lesions. We report four such interesting patients who presented to us initially with common dermatoses such as leukocytoclastic vasculitis, pyoderma gangrenosum, and vesiculobullous disorders and were subsequently diagnosed to have MM. There were no skeletal involvements or renal function abnormality at the time of presentation. Unusual presentation, nonresponsiveness to conventional therapy, and abnormal blood parameters prompted us to suspect some underlying systemic conditions which were later confirmed to be MM after serum immunoelectrophoresis for M-band and bone marrow biopsy.     

Biostatistics Series Module 6: Correlation and Linear Regression

Correlation and linear regression are the most commonly used techniques for quantifying the association between two numeric variables. Correlation quantifies the strength of the linear relationship between paired variables, expressing this as a correlation coefficient. If both variables x and y are normally distributed, we calculate Pearson''s correlation coefficient (r). If normality assumption is not met for one or both variables in a correlation analysis, a rank correlation coefficient, such as Spearman''s rho (ρ) may be calculated. A hypothesis test of correlation tests whether the linear relationship between the two variables holds in the underlying population, in which case it returns a P < 0.05. A 95% confidence interval of the correlation coefficient can also be calculated for an idea of the correlation in the population. The value r² denotes the proportion of the variability of the dependent variable y that can be attributed to its linear relation with the independent variable x and is called the coefficient of determination. Linear regression is a technique that attempts to link two correlated variables x and y in the form of a mathematical equation (y = a + bx), such that given the value of one variable the other may be predicted. In general, the method of least squares is applied to obtain the equation of the regression line. Correlation and linear regression analysis are based on certain assumptions pertaining to the data sets. If these assumptions are not met, misleading conclusions may be drawn. The first assumption is that of linear relationship between the two variables. A scatter plot is essential before embarking on any correlation-regression analysis to show that this is indeed the case. Outliers or clustering within data sets can distort the correlation coefficient value. Finally, it is vital to remember that though strong correlation can be a pointer toward causation, the two are not synonymous.     

Pseudoxanthoma elasticum with periumbilical perforation in a nullipara

Pseudoxanthoma elasticum (PXE) is an inherited multisystem disorder that primarily affects the skin and is characterized by progressive calcification and degeneration of the elastic fibers. PXE has recently been found to be caused by mutations in the ATP-binding cassette transporter C6 (ABCC6) or the multidrug resistance-associated protein 6 (MRP6) genes. Perforating PXE is a rare presentation that is usually seen in the periumbilical area in obese multiparous black women; it has distinct clinical and histopathological features and there may or may not be systemic manifestations. We report an unusual case of PXE in a nulliparous woman, with perforation in the periumbilical area and without any systemic involvement.     

NORMOLIPEMIC TUBEROUS XANTHOMAS

Xanthomas are often a manifestation of underlying lipid abnormalities. A 50-year-old male presented to our hospital with the lesions of multiple tuberous xanthomas all over the body. Routine investigations and systemic examination were normal. Lipid profile was within normal range and serum protein electrophoresis showed normal pattern. Histopathology from a nodular lesion showed collection of foamy macrophages in the dermis. We present a case of normolipemic tuberous xanthomas, which is an uncommon occurrence.     

ADVERSE CUTANEOUS DRUG REACTION

In everyday clinical practice, almost all physicians come across many instances of suspected adverse cutaneous drug reactions (ACDR) in different forms. Although such cutaneous reactions are common, comprehensive information regarding their incidence, severity and ultimate health effects are often not available as many cases go unreported. It is also a fact that in the present world, almost everyday a new drug enters market; therefore, a chance of a new drug reaction manifesting somewhere in some form in any corner of world is unknown or unreported. Although many a times, presentation is too trivial and benign, the early identification of the condition and identifying the culprit drug and omit it at earliest holds the keystone in management and prevention of a more severe drug rash. Therefore, not only the dermatologists, but all practicing physicians should be familiar with these conditions to diagnose them early and to be prepared to handle them adequately. However, we all know it is most challenging and practically difficult when patient is on multiple medicines because of myriad clinical symptoms, poorly understood multiple mechanisms of drug-host interaction, relative paucity of laboratory testing that is available for any definitive and confirmatory drug-specific testing. Therefore, in practice, the diagnosis of ACDR is purely based on clinical judgment. In this discussion, we will be primarily focusing on pathomechanism and approach to reach a diagnosis, which is the vital pillar to manage any case of ACDR.