Congenital cutaneous candidiasis: a rare and unpredictable disease

Congenital cutaneous candidiasis (CCC) is an extremely rare disorder that presents within the first 6 days of life. The manifestations ranges from diffuse skin eruption without any systemic symptoms to respiratory distress, hepatosplenomegaly, sepsis, and death. We report a neonate who presented with generalized skin eruptions at birth, characterized by erythematous macules and papules. The eruption involved head, face, neck, trunk, and extremities. Candida albicans was demonstrated on direct KOH smear, skin biopsy. The disease implies a congenital intrauterine infection and is different from neonatal candidiasis, which manifests as thrush or diaper dermatitis. The infection is acquired from the maternal genital tract in an ascending fashion. Clinical features, direct smear examination of specimen, and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period. Topical antifungal therapy is sufficient unless systemic candidiasis is present. Prognosis for congenital cutaneous candidiasis is good.     

Violaceous Maculopapular Rash in a Newborn: Congenital Rubella Syndrome

Congenital rubella syndrome involves a configuration of systemic and cutaneous manifestations in a neonate due to in utero infection caused by the rubella virus. The case of a preterm neonate with blueberry muffin lesions and classical as well as rare systemic features of congenital rubella syndrome is reported.     

Lymphoepithelioma-Like Carcinoma of the Skin: An Exceedingly Rare Primary Skin Tumor

Primary lymphoepithelioma-like carcinoma of the skin (LE-lCS) is a very rare cutaneous malignancy of uncertain origin. The neoplasm reveals typical morphological similarity to undifferentiated nasopharyngeal carcinoma (lymphoepithelioma). This case report presents a 47-year-old man with a 5 mm erythematous papule on dorsal nose of six months duration. The patient underwent complete surgical excision and is disease free 7 months later.     

Bilateral Systematized Epidermolytic Verrucous Epidermal Nevus: A Rare Entity

Verrucous epidermal nevi are congenital, noninflammatory cutaneous hamartomas composed of keratinocytes. They follow the lines of Blaschko and show hyperkeratosis without cellular atypia. The routine histology shows variable amount of hyperkeratosis, acanthosis and papillomatosis and rarely epidermolytic hyperkeratosis. We saw a 3-year-old boy with bilaterally symmetrical, systematized verrucous plaques along the lines of Blaschko extensively involving the trunk and extremities but sparing the face and palmoplantar skin. Histopathology showed features of epidermal nevi with prominent epidermolytic hyperkeratosis. We report here the case for the rarity of this entity.     

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.     

CUTANEOUS PANCREATIC METASTASIS: A CASE REPORT AND REVIEW OF LITERATURE

Pancreatic cancer is one of the most dangerous human cancers and will continue to be a major unsolved health problem as we enter the 21^st century. This is the case despite advances in imaging technology and surgical management. Indeed, 80% to 90% of pancreatic cancers are diagnosed either at the locally advanced or metastatic stage. Cutaneous metastases originating from pancreatic cancer are relatively rare. The most common site of cutaneous metastasis is the umbilicus, and this is known as the Sister Joseph''s nodule. Very few patients have been reported with cutaneous lesions disclosing a pancreatic carcinoma at sites other than the umbilical area. To the best of our knowledge, there have been no previous reports on cutaneous pancreatic metastasis in Egypt. This is a report on a patient with cutaneous pancreatic metastases at the neck and review of reported non-umbilical cutaneous metastases from pancreatic carcinoma in the literatures.     

Primary Cutaneous Diffuse Large B-Cell Lymphoma of the Upper Limb: A Fascinating Entity

Primary cutaneous lymphomas are defined as lymphoid neoplasms that present themselves clinically on the skin and do not have extra-cutaneous disease, when the diagnosis is made or even after 6 months of the diagnosis. Primary cutaneous lymphomas of B-cells are less frequent than lymphomas of T-cells. Primary B-cell lymphomas have a better prognosis than secondary B-cell lymphomas. Primary B-cell cutaneous lymphomas are classified into five types according to the World Health Organization and European Organization for Research and Treatment of Cancer classification. The primary diffuse large B-cell cutaneous lymphoma – leg type corresponds to approximately 5-10% of the B-cell cutaneous lymphomas. It is predominantly seen in elderly people and has a female preponderance. Skin lesions can be single, multiple, and even grouped. A 5-year survival rate ranges from 36 to 100% of the cases. The expression of Bcl-2, presence of multiple lesions, and involvement of both the upper limbs lead to a worse prognosis. Very few cases have been described in the literature.     

Mal de Meleda with Congenital Cataract: A Novel Case Report

Mal de meleda (MdM), a rare autosomal recessive genodermatosis is characterized by erythema and hyperkeratosis of the palms and soles with a sharp demarcation and that progress with age (progrediens) and extend to the dorsal aspects of the hands and feet (transgrediens). It has been associated with various conditions albeit rarely with congenial cataract. Ocular lens and the skin have the same embryological origins. We hereby present this novel case report of Mal de meleda in association with congenital posterior subcapsular cataract which to the best of our knowledge has not been reported from India before.     

Basal Cell Carcinoma and Syringocystadenoma Papilliferum Arising in Nevus Sebaceous on Face—A Rare Entity

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprising of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual to develop two different types of tumor, benign and malignant, to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 27-year-old male with two neoplastic proliferations including a syringocystadenoma papilliferum a benign tumor and basal cell carcinoma a malignant tumor arising in a long-standing nevus sebaceus on the face. Neoplastic changes are common in nevus sebaceous present on scalp but our case is unique due to the presence of two different types of neoplasm in a nevus sebaceous which was present on face.     

ELEPHANTIASIS OF THE EXTERNAL GENITALIA: A SEQUEL TO CUTANEOUS TUBERCULOSIS

Tuberculosis continues to be an important public health problem and cutaneous tuberculosis constitutes a minor proportion of extra pulmonary manifestations of tuberculosis. Elephantiasis of the external genitalia, as a sequel to cutaneous tuberculosis, in a 40-year-old diabetic lady is being reported for its rarity. The patient also had lesions of healed scrofuloderma of 27 years’ duration, in both axillae, with residual pedunculated nodules.     

Topical Steroid-Damaged Skin

Topical steroids, commonly used for a wide range of skin disorders, are associated with side effects both systemic and cutaneous. This article aims at bringing awareness among practitioners, about the cutaneous side effects of easily available, over the counter, topical steroids. This makes it important for us as dermatologists to weigh the usefulness of topical steroids versus their side effects, and to make an informed decision regarding their use in each individual based on other factors such as age, site involved and type of skin disorder.     

Bilateral Multisegmental Zosteriform Leiomyoma Cutis: A Rare Entity

Cutaneous leiomyomas are benign tumors derived from the smooth muscles of the skin. They are firm, skin-colored to brownish, frequently painful papules and nodules presenting either as a solitary tumor or multiple clustered lesions. Uncommon patterns of multiple leiomyoma cutis include linear, zosteriform, or dermatomal-like arrangement of lesions. We saw a 32-year-old man who presented with clusters of skin colored to reddish brown, occasionally painful papules and nodules distributed segmentally over C6 to T8 dermatomes on left side and T11 to L1 dermatomes on right side. Histopathology confirmed the diagnosis of cutaneous leiomyoma. The case is reported here for the occurrence of this benign cutaneous neoplasm in an extremely rare bilateral multisegmental distribution.     

Approach to Cutaneous Lymphoid Infiltrates: When to Consider Lymphoma?

Cutaneous lymphoid infiltrates (CLIs) are common in routine dermatopathology. However, differentiating a reactive CLI from a malignant lymphocytic infiltrate is often a significant challenge since many inflammatory dermatoses can clinically and/or histopathologically mimic cutaneous lymphomas, coined pseudolymphomas. We conducted a literature review from 1966 to July 1, 2015, at PubMed.gov using the search terms: Cutaneous lymphoma, cutaneous pseudolymphoma, cutaneous lymphoid hyperplasia, simulants/mimics/imitators of cutaneous lymphomas, and cutaneous lymphoid infiltrates. The diagnostic approach to CLIs and the most common differential imitators of lymphoma is discussed herein based on six predominant morphologic and immunophenotypic, histopathologic patterns: (1) Superficial dermal T-cell infiltrates (2) superficial and deep dermal perivascular and/or nodular natural killer/T-cell infiltrates (3) pan-dermal diffuse T-cell infiltrates (4) panniculitic T-cell infiltrates (5) small cell predominant B-cell infiltrates, and (6) large-cell predominant B-cell infiltrates. Since no single histopathological feature is sufficient to discern between a benign and a malignant CLI, the overall balance of clinical, histopathological, immunophenotypic, and molecular features should be considered carefully to establish a diagnosis. Despite advances in ancillary studies such as immunohistochemistry and molecular clonality, these studies often display specificity and sensitivity limitations. Therefore, proper clinicopathological correlation still remains the gold standard for the precise diagnosis of CLIs.     

The role of cytokines in the pathomechanism of cutaneous disorders

Cytokines are produced transiently by cutaneous cells in response to immune stimuli. They are non-immunoglobulin proteins and glycoproteins. Cytokines, which are signaling molecules, act by sending downstream signals to various cells through a number of signal transduction pathways and by binding to cytokine receptors on the surface of cells. Cytokines regulate immunity and inflammation and thus play an important role in the pathogenesis of various cutaneous disorders.     

A clinical and histopathological profile of patients with cutaneous tuberculosis

Extrapulmonary tuberculosis constitutes about 10% of all cases of tuberculosis, and cutaneous tuberculosis makes up only a small proportion of these cases. Despite prevention programs, tuberculosis is still progressing endemically in developing countries. Commonest clinical variant of cutaneous tuberculosis in our study was lupus vulgaris seen in 55% patients followed by scrufuloderma seen in 25% patients followed by orificial tuberculosis, tuberculosis verrucosa cutis, papulonecrotic tuberculid, and erythema induratum seen in 5% each. The commonest site of involvement was limbs seen in 50% patients followed by neck seen in 25% patients, face in 15%, and trunk in 10% patients. Maximum percentage of patients (55%) had duration of cutaneous tuberculosis between 6-12 months followed by 35% between 13-24 months, 5% had duration of cutaneous tuberculosis less than 6 months, and the rest 5% had duration more than 24 months. The commonest histopathological feature in our study was tuberculoid granuloma with epitheloid cell and Langhans giant cells seen in 70% patients, hyperkeratosis was seen in 15% patients and AFB bacilli were seen in 5% patients.     

Immune deposits in cutaneous lesions of Wegener's granulomatosis: predictor of an active disease

A retrospective analysis was conducted of eight cases of Wegener's granulomatosis (WG), who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA) status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3)-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO)-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3) or absence (n=1) of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.     

Extrinsic apoptotic pathways: a new potential "target" for more sufficient therapy in a case of cutaneous anaplastic large cd30+ alk-T-cell lymphoma

The primary cutaneous T-cell lymphomas (CTCL) represent a clonal T-lymphocyte proliferation infiltrating the skin. CD30⁺T-cell lymphomas present clinically as nodules with a diameter between 1 and 15 cm, mostly in elderly patients. The role of the CD30 molecule in patients suffering from T-cell lymphomas is not completely clear yet. The signal transduction pathway which includes CD30 seems to play a key role in tumor progression. In certain forms of T-cellular lymphomas, the interaction between CD30/CD30-ligand is able to provoke apoptosis of the “tumor lymphocytes”. The modern conceptions of the pathogenesis of T-cell lymphomas include disorders in the pathways involved in programmed cellular death and disregulation in the expression of certain of its regulatory molecules. We are presenting an unusual case of a female patient with a primary cutaneous form of CD30⁺/ALK⁻ anaplastic large T-cell lymphoma. Upon the introduction of systemic PUVA, (psoralen plus ultraviolet light radiation) combined with beam therapy, a complete remission could be noticed. Eight months later, we observed a local recurrence, which was overcome by CHOP chemotherapy (Cyclophosphamide, Hydroxydaunorubicin (Doxorubicin), Vincristin (Oncovin®), Predniso(lo)n). Six months later, new cutaneous lesions had been noticed again. A new therapeutic hope for the patients with anaplastic large CTCL is actually based on the influence of the activity of the different apoptotic pathways. Death ligands, including tumor necrosis factor (TNF)-α, CD95L/FasL, and TRAIL, mediate also some important safeguard mechanisms against tumor growth in patients with CD30⁺ cutaneous anaplastic large T-cell lymphomas and critically contribute to lymphocyte homeostasis.     

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.     

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.