Hyaline cell-rich chondroid syringoma of the finger

A mixed tumor is a neoplasm that has microscopic features of both epithelial and mesenchymal differentiation. Such mixed tumors are known as pleomorphic adenomas in the salivary glands, and their cutaneous counterparts are called chondroid syringomas. These tumors commonly occur in the head and neck region of middle-aged men. Hyaline cell-rich chondroid syringoma is a rare benign variant of chondroid syringoma composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. Although very few cases have been reported in literature, it is important to be aware of this entity so as to avoid misdiagnosis on histopathological examination. In this report we present a case of hyaline cell-rich chondroid syringoma occurring in the finger.     

GENERALIZED ERUPTIVE SYRINGOMAS

Generalized eruptive syringoma is a rare clinical presentation of a benign adnexal tumor that derives from the intraepidermal portion of the eccrine sweat ducts. It presents as successive crops of small flesh-colored papules on the anterior body surfaces. It generally occurs in the peripubertal period. Treatment of this benign condition is cosmetic only. A case of a 28-year-old female with an eight-year history of eruptive syringoma is presented.     

Chondroid Syringoma of the Forearm: A Case Report of a Rare Localization

Chondroid syringoma (CS) is an uncommon benign adnexal tumor of the skin with eccrine and apocrine origin, which usually involves the head and neck region. The presentation of CS in other areas of the body is rare. A 45-year-old male patient presented to the dermatology clinic with a chief complaint of a painless, slow-growing mass on his left forearm, which gradually developed over the course of 2 years. A solitary, firm, purple, mobile, non-tender nodule was located in the distal part of left dorsal forearm, which was 1.8 cm in diameter. The tumor was surgically excised and sent for the histopathological evaluation. Results of biopsy and hematoxylin and eosin staining confirmed the diagnosis of CS and showed no evidence of malignancy. Although CS is an uncommon tumor in uppr limb region, it should be considered as one of the differential diagnoses, when dealing with tumors of this area.     

DETECTION OF R576 INTERLEUKIN-4 RECEPTOR AN ALLELE GENE, SERUM INTERLEUKIN-4, AND EOSINOPHILIC CATIONIC PROTEIN IN ATOPIC DERMATITIS PATIENTS

Atopic dermatitis (AD) is a chronic pruritic skin disease. It results from a complex interplay between strong genetic and environmental factors. The aim of this work was to study some biochemical markers of the dermatosis. This included detection of R576 interleukin-4 receptor alpha allele gene. Twenty five patients with AD and 25 controls participated in this study.     

Radiation Port Cutaneous Metastases: Reports of Two Patients Whose Recurrent Visceral Cancers Presented as Skin Lesions at the Site of Previous Radiation and Literature Review

Radiation therapy is associated with a variety of complications, including the development of primary skin cancers in the radiated region. However, it is rare for patients with visceral cancers who are treated with radiation therapy to subsequently develop cutaneous metastasis within the radiation port. We describe two patients with internal malignancies who developed cutaneous metastases within their radiation ports following radiotherapy. In addition, we used PubMed to perform an extensive literature review and identify additional reports of cutaneous metastasis within a radiation port. We excluded patients who developed melanoma or primary skin cancers in the radiation port. We also excluded patients with non-solid organ malignancies. Herein, we summarize the characteristics of 23 additional patients who experienced radiation port cutaneous metastases and explore possible mechanisms for the occurrence of radiation port cutaneous metastases.     

Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.     

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.     

Strengths and Limitations of Evidence-Based Dermatology

The need for understanding and reflecting on evidence-based dermatology (EBD) has never been greater given the exponential growth of new external evidence to inform clinical practice. Like any other branch of medicine, dermatologists need to acquire new skills in constructing answerable questions, efficiently searching electronic bibliographic databases, and critically appraising different types of studies. Secondary summaries of evidence in the form of systematic reviews (SR), that is, reviews that are conducted in a systematic, unbiased and explicit manner, reside at the top of the evidence hierarchy, because they are less prone to bias than traditional expert reviews. In addition to providing summaries of the best external evidence, systematic reviews and randomized controlled trials (RCTs) are also powerful ways of identifying research gaps and ultimately setting the agenda of future clinical research in dermatology. But like any paradigm, EBD can have its limitations. Wrong application, misuse and overuse of EBD can have serious consequences. For example, mindless pooling together of data from dissimilar studies in a meta-analysis may render it a form of reductionism that does not make any sense. Similarly, even highly protocolised study designs such as SRs and RCTs are still susceptible to some degree of dishonesty and bias. Over-reliance on randomized controlled trials (RCT) may be inappropriate, as RCTs are not a good source for picking up rare but important adverse effects such as lupus syndrome with minocycline. A common criticism leveled against SRs is that these frequently conclude that there is lack of sufficient evidence to inform current clinical practice, but arguably, such a perception is grounded more on the interpretation of the SRs than anything else. The apparent absence of evidence should not paralyze the dermatologist to adopt a state of therapeutic nihilism. Poor primary data and an SR based on evidence that is not up-to-date are also limitations that can only improve with better primary studies and updated reviews such as those done by the Cochrane Collaboration. Most dermatologists are interested in integrating the best external evidence with the care of individual patients and have been practicing good EBD without realizing it.     

Treatment of Refractory Chronic Urticaria

Chronic spontaneous urticaria is a distressing disease encountered frequently in clinical practice. The current mainstay of therapy is the use of second-generation, non-sedating antihistamines. However, in patients who do not respond satisfactorily to these agents, a variety of other drugs are used. This article examines the available literature for frequently used agents including systemic corticosteroids, leukotriene receptor antagonists, dapsone, sulfasalazine, hydroxychloroquine, H2 antagonists, methotrexate, cyclosporine A, omalizumab, autologous serum therapy, and mycophenolate mofetil, with an additional focus on publications in Indian literature.     

Solitary Pilar Leiomyoma of the Nasal Dorsum: Case Report and Literature Review

Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.     

Multiple Carboxylase Deficiency (Late Onset) Due to Deficiency of Biotinidase

Biotinidase is a ubiquitous mammalian cell enzyme occurring in liver, serum and kidney. It cleaves biotin from biocytin, which is a cofactor for biotin dependent enzymes, namely the human carboxylases. Biotinidase deficiency is associated with a wide spectrum of neurological, dermatological, immunological and ophthalmological abnormalities. This is a case of a 3-year-old boy presenting with delayed developmental milestones, tachypnea, progressively increasing ataxia, alopecia and dermatitis, all which dramatically responded to high doses of biotin.     

Folliculitis et perifolliculitis capitis abscedens et suffodiens controlled with a combination therapy: systemic antibiosis (metronidazole plus clindamycin), dermatosurgical approach, and high-dose isotretinoin

Folliculitis et perifolliculitis capitis abscedens et suffodiens is a rare disease of unknown etiology. It is a suppurative process that involves the scalp, eventually resulting in extensive scarring and irreversible alopecia. The condition is also known as 'acne necrotica miliaris' or 'Proprionibacterium' folliculitis. Most often the disease affects men of African-American or African-Caribbean descent between 20 and 40 years of age. The clinical picture is determined by fluctuating painful fistule-forming conglomerates of abscesses in the region of the occipital scalp. The cause of scalp folliculitis is not well understood. It is generally considered to be an inflammatory reaction to components of the hair follicle, particularly the micro-organisms. These include: bacteria (especially Propionibacterium acnes, but in severe cases, also Staphylococcus aureus), Yeasts (Malassezia species) and mites (Demodex folliculorum). The initial histopathologic finding is an exclusively neutrophilic infiltration followed by a granulomatous infiltrate. The treatment of the disease is usually difficult and often disappointing. Successful treatment with isotretinoin 1 mg/kg body mass could be achieved only after regular systematic administration in the course of 3-4 months. Here we describe a patient with eruptive purulent form of the disease, which has been controlled with combination therapy: systemic antibiosis with metronidazole and clindamycin, dermatosurgical removal of single nodular formations, and isotretinoin 1 mg/kg body mass for 3-5 months.     

Clinico-Mycological Study of Dermatophyte Toenail Onychomycosis in New Delhi,India

Introduction:

There is a constant need to define the epidemiological and mycological characteristics of onychomycosis (OM) for optimal management strategies.

Objectives:

To define the epidemiological and mycological characteristics of patients with dermatophyte toenail OM in a tertiary care hospital.

Materials and Methods:

Hundred consecutive patients of KOH and culture-positive dermatophyte toenail OM were subjected to detailed history, clinical examination and investigations.

Results:

Maximum number of patients (40%) belonged to 31-45 years age group and there was a male preponderance (M:F = 6.7:1). The mean duration of disease was 54 months. Thirty-three patients had fingernail involvement in addition to the toenail OM and 37% had co-existent cutaneous dermatophyte infection. Discoloration was the most common symptom (98%). Ninety-four (94%) patients had distal lateral subungual onychomycosis (DSLO) while two had superficial onychomycosis (SO) and only one had proximal superficial onychomycosis (PSO). Trichophyton interdigitale was the most common etiological agent (61%) followed by Trichophyton rubrum and Trichophyton verrucosum.

Conclusions:

Toenail OM is more common in males. DSLO was the most common clinical variant and T. interdigitale the most common etiological fungus responsible for toenail OM in our region. The importance of early diagnosis and treatment is highlighted as long-standing toenail OM predisposes to fingernail onychomycosis and recurrent tinea pedis.     

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.     

Solitary cylindroma with malignant transformation

A 59-year-old woman presented with a history of rapidly progressive recurrent tumor of 6.5 cm diameter of the scalp. Histopathological examination revealed a case of malignant cylindroma. There has been no recurrence or metastases and the patient is disease free at the end of 15 months postoperatively. Malignant transformation occurs less often in solitary form of cylindroma, but once transformed, tumors behave aggressively with extensive local infiltrative growth or metastases. The case is reported to document a rare case of malignant cylindroma arising in a patient with solitary cylindroma on the parieto-temporal region.     

Topical Therapy in Atopic Dermatitis in Children

Atopic dermatitis (AD) is a common, chronic childhood skin disorder caused by complex genetic, immunological, and environmental interactions. It significantly impairs quality of life for both child and family. Treatment is complex and must be tailored to the individual taking into account personal, social, and emotional factors, as well as disease severity. This review covers the management of AD in children with topical treatments, focusing on: education and empowerment of patients and caregivers, avoidance of trigger factors, repair and maintenance of the skin barrier by correct use of emollients, control of inflammation with topical corticosteroids and calcineurin inhibitors, minimizing infection, and the use of bandages and body suits.     

A Report of 10 Individuals with Weathering Nodules and Review of the Literature

Weathering nodules are a benign skin condition that usually present as papules on the helices of patients with significant prior sun exposure. They are easily recognized clinically and blanch upon application of pressure to the adjacent helical rim: a positive blanch sign. We describe the clinical presentation of weathering nodules in 10 patients, nine men and one woman, aging from 38 to 70 (median 59), and their associated risk factors. Eight patients had a history of actinic keratosis, three had a history of nonmelanoma skin cancer, and all patients had increased Sun exposure through outdoor activities. Weathering nodules are rarely mentioned in the literature and may be confused with other cutaneous disorders. Therefore, it is paramount for clinicians to become familiar with weathering nodules and include them in the differential diagnosis of ear nodules. Appropriate diagnosis will help avoid unnecessary biopsies while reassuring the patient that the lesions are benign.