Cutaneous manifestations in brucellosis

Brucellosis is a common worldwide zoonotic disease. Cutaneous manifestations are not specific and affect 1-14% of patients with brucellosis. Here, we describe 49-year-old female with fever and a diffuse maculopapular rash due to Brucella melitensis infection. Histopathology of skin biopsy revealed leukocytoclastic vasculitis; positive blood cultures for B. melitensis established the diagnosis of brucellosis. We provide a review of the relevant literature.     

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.     

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.     

Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations

A 37-year-old male presented with severe oral and genital mucosal ulcers, lichenoid eruption and twenty-nail dystrophy. Systemic examination was normal, except for anemia. On investigations, he was found to have persistently elevated peripheral eosinophilia, absolute eosinophil count >5000/mm³, bone marrow showing increased eosinophilic precursors, and infiltration by atypical cells. The serum vitamin B12 levels were grossly elevated, and Philadelphia chromosome study was negative. Thus, a diagnosis of chronic eosinophilic leukemia was made. The patient showed excellent response to imatinib mesylate. We are reporting a rare type of leukemia presenting with predominantly cutaneous manifestations.     

Cutaneous Manifestations of Mixed Connective Tissue Disease: Study from a Tertiary Care Hospital in Eastern India

Context:

Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe.

Aims:

This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe.

Settings and Design:

Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years.

Materials and Methods:

The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients.

Statistical Analysis Used:

SPSS (Version 17) and MedCalc (Version 11.6).

Results:

The Male: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynaud''s phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this disease

Conclusions:

Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease.     

Leukocytoclastic vasculitis: a window to systemic churg strauss syndrome

A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV) after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE), Anti nuclear antibody (ANA) positivity and negative antineutrophil cytoplasmic antibody (ANCA). Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT) were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS). This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.     

A CASE OF MULTIPLE MYELOMA PRESENTING AS A BULLOUS DERMATOSIS

Multiple myeloma is a malignant plasma cell proliferative disorder that produces a monoclonal immunoglobulin protein. The skin involvement and the development of bullous disease are rarely seen features in multiple myeloma. We present a 55-year-old man with a longstanding, large, tense bullous eruption and hypertrophic scars over his body accompanied recently with weight loss and fatique. He had no response to the previous treatments, which included oral glucocorticoids and dapsone. Histologic examination of the lesions revealed subepidermal bullae, while no immunoflourescence staining was observed. In a further detailed labarotory examination, multiple myeloma was detected. After the treatment of multiple myeloma with chemotherapy, the lesions regressed. Patients with longstanding, recurrent, unusual bullous eruption should be investigated for the development of multiple myeloma.     

Cutaneous manifestations of internal malignancy

Background:

Many malignancies affecting the internal organs display cutaneous manifestations which may be either specific (tumor metastases) or nonspecific lesions.

Aims:

The study is aimed at determining the frequency and significance of cutaneous manifestations among patients with internal malignancy.

Materials and Methods:

750 cases of proven internal malignancy, who attended a cancer chemotherapy center in South India, were studied. Specific infiltrates were confirmed by histopathology, fine needle aspiration cytology (FNAC) and marker studies.

Results:

Out of the 750 patients with internal malignancy, skin changes were seen in a total of 52 (6.93%) patients.

Conclusion:

Cutaneous metastases (specific lesions) were seen in 20 patients (2.66%): contiguous in 6 (0.8%), and non-contiguous in 14 (1.86%). Nonspecific skin changes were seen in 32 patients (4.26%). None of our patients presented with more than one type of skin lesions. Herpes zoster was the most common nonspecific lesion noticed in our patients, followed by generalized pruritus, multiple eruptive seborrheic keratoses, bullous disorder, erythroderma, flushing, purpura, pyoderma gangrenosum, insect bite allergy and lichenoid dermatitis.     

Hydroa Vacciniforme-Like Cutaneous T-cell Lymphoma

A 14-year-old Chinese girl had a 6-year history of recurrent lesions on her head, face, and limbs. Epstein-Barr virus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistry showed CD3+, CD4+, CD5+, CD8+, TIA-1+, GrB+, CD56-, and L26-. In situ hybridization staining for EBV-encoded small nonpolyadenylated RNA (EBER)-1 was positive. The patient showed significant improvement in clinical symptoms after being treated with acyclovir and IFN-α in this patient.     

Cerebriform Cutaneous Lesions in Pemphigus Vegetans

Pemphigus vegetans is an autoimmune bullous disorder characterized by vegetating lesions commonly over the flexures. A 42-year-old female patient came with pemphigus vegetans presenting with interesting cerebriform morphology of the cutaneous lesions over the flexures. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue is a well-known sign seen in pemphigus vegetans. Interestingly, we noticed the typical sulci and gyri pattern in the skin lesions of pemphigus vegetans over the flexures of the body. This clinical sign can be used as a clue in the diagnosis of pemphigus vegetans. Morphology and physical characteristics are important for the diagnosis of the disease. Clinical signs always give a clue to the probable or possible diagnosis in most of the dermatological conditions.     

Primary Cutaneous Nocardiosis

A 35-year-old male presented with multiple discharging sinuses and fluctuant swelling on right side of face which started after a vehicular accident three year back. The lesions did not respond to routine antibiotics treatment but went on increasing. Gram smear showed typical acid fast branching filaments suggestive of nocardiosis, which was confirmed by culture. Patient received co-trimoxazole for three and a half month. When seen again after three years, all the lesions had healed with puckered scarring. Patient had received co-trimoxazole for three and a half months with dramatic improvement.     

Cutaneous drug hypersensitivity: immunological and genetic perspective

Drug hypersensitivity is an unpredictable, immunologically mediated adverse reaction, clustered in a genetically predisposed individual. The role of "hapten concept" in immune sensitization has recently been contested by the "pharmacological interaction" hypothesis. After completion of the "human genome project" and with the availability of high-resolution genotyping, genetic susceptibility to hypersensitivity for certain drugs has been proved beyond doubt though the trend is ethnicity and phenotype dependent. Application of this newly acquired knowledge may reduce or abolish the morbidity and mortality associated with cutaneous drug hypersensitivity.     

Cryoglobulinemia in a child

Cryoglobulinemia is a rare condition. We report a case of cryoglobulinemia in 1.5-year-old boy, who presented with bluish black discoloration of hands and feet. Serum was positive for cryoglobulins. The serum cryoglobulin level was 70 μg/ml (normal <20 μg). Serum protein electrophoresis revealed the presence of M-spike. Serum immunofixation and assessment of clonality could not be done as these specialized investigations are not available here. Investigations did not reveal any systemic involvement. He was treated with anticoagulants, broad-spectrum antibiotics, and systemic steroids with which he recovered completely.     

Systemic Sclerosis: Current Concepts in Pathogenesis and Therapeutic Aspects of Dermatological Manifestations

Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud''s phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient''s quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature.     

Churg-strauss syndrome without respiratory symptoms in a child

Churg-Strauss Syndrome (CSS) is rare in children. It consists of a small- and medium-sized vessel vasculitis, with skin and peripheral nerve involvement. It is characterized by eosinophilia, extravascular necrotizing granuloma, and eosinophilic infiltration of multiple organs particularly the lungs, but may also involve the gastrointestinal tract, the heart, and the kidneys. The condition is usually associated with a preceding history of asthma or allergic sinusitis. It has rarely been reported in children, where most of the cases had pre-existing asthma, allergic rhinitis, or atopic disease. We report a 10-year-old Arab girl proven to have CSS, with no history of asthma or allergic rhinitis, who presented with tender cutaneous nodules of lower extremities, foot drop, and peripheral eosinophilia, without any clinical respiratory symptoms or signs.     

A retrospective analysis of histopathology of 64 cases of lepra reactions

Background:

Lepra reactions are not always diagnosable under the microscope. We analyzed skin histopathology in 64 cases of lepra reaction.

Aim:

To make detailed observations on histopathologic features of type 1 and type 2 lepra reaction (erythema nodosum leprosum, ENL).

Materials and Methods:

In this retrospective study, we included 64 patients diagnosed during a 3-year period as lepra reaction based on clinico-pathological co-relation.

Results:

Out of the 64 patients, 22 were of type 1 reaction and 42 of ENL. The most consistent finding in type 1 reaction was papillary dermal edema (86%) followed by pyknosis of lymphocytes (77%) and intercellular edema within granuloma (73%). Surprisingly, folliculotropism of lymphocytes was seen in 55% and subcutaneous infiltration in 36%. In ENL, the most common finding was presence of neutrophils within the granuloma (100%), followed by leukocytoclasia (81%), papillary dermal edema (81%), and neutrophilic panniculitis (69%). Fibrin in the vessel wall or/and granulomas was noted in only 38% while fibrin thrombi in the vessel walls were seen in only 12% of cases.

Conclusion:

Infiltration of macrophage granulomas by neutrophils is a reliable sign of ENL. Classical signs of vasculitis are not always present in ENL. Folliculotropism and lymphocytic panniculitis were frequent in type 1 reactions while neutrophilic panniculitis was common with ENL.     

Digital Gangrene Associated with Anticentromere Antibodies

Anticentromere antibodies have been associated with peripheral vascular occlusive disease, but then it is mostly accompanied by sclerodactyly in the context of a connective tissue disorder. We report a case of digital gangrene in a 75 year old lady with no other associations except positive anticentromere antibodies.     

Pseudolymphomatous Folliculitis: A Distinctive Cutaneous Lymphoid Hyperplasia

Pseudolymphomatous folliculitis (PLF) was first described in 1986 as a distinct variant of pseudolymphoma, characterized by a dense lymphoid infiltrate and accompanied by hyperplastic hair follicles. Here in we report a case of PLF presenting as an erythematous plaque with pustules and satellite lesions on forehead in an otherwise healthy adult male patient.     

Diffuse Cutaneous Mastocytosis with Bullous Lesions and Pulmonary Involvement: A Rare Case

Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes. However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.     

Simulation-Based Cutaneous Surgical-Skill Training on a Chicken-Skin Bench Model in a Medical Undergraduate Program

Background:

Because of ethical and medico-legal aspects involved in the training of cutaneous surgical skills on living patients, human cadavers and living animals, it is necessary the search for alternative and effective forms of training simulation.

Aims:

To propose and describe an alternative methodology for teaching and learning the principles of cutaneous surgery in a medical undergraduate program by using a chicken-skin bench model.

Materials and Methods:

One instructor for every four students, teaching materials on cutaneous surgical skills, chicken trunks, wings, or thighs, a rigid platform support, needled threads, needle holders, surgical blades with scalpel handles, rat-tooth tweezers, scissors, and marking pens were necessary for training simulation.

Results:

A proposal for simulation-based training on incision, suture, biopsy, and on reconstruction techniques using a chicken-skin bench model distributed in several sessions and with increasing levels of difficultywas structured. Both feedback and objective evaluations always directed to individual students were also outlined.

Conclusion:

The teaching of a methodology for the principles of cutaneous surgery using a chicken-skin bench model versatile, portable, easy to assemble, and inexpensive is an alternative and complementary option to the armamentarium of methods based on other bench models described.