An unusual lesion on the nose: microvenular hemangioma.

Microvenular hemangioma (MVH) is an acquired, benign type of hemangioma that usually manifests itself as a solitary, slowly growing, red to violaceous, asymptomatic papule, plaque or nodule. It is typically located on the trunk or extremities of young adults. It can be difficult to differentiate MVH from other types of hemangioma and Kaposi sarcoma. Herein we report a case of MVH unusual for its location, age of onset, and morphologic features. A 62-year-old man complained of an asymptomatic, bluish-red discoloration on the tip of his nose that had been present for two years. Dermatologic examination showed a violaceous patch 2 × 2 cm in diameter with indistinct borders. Incisional biopsy revealed irregularly branched small or medium-sized vascular spaces lined with benign endothelial cells, positive for CD34 and negative for HHV-8. MVH is a rare lesion, and less than 70 cases have been published to date. A review of 40 reported cases revealed that 15% of MVH patients were over 40 years of age and only 3% of the cases showed macules or patches. A literature survey showed only two cases of MVH located on the facial region, one on the chin and the other on the cheek. Our case was unique for its location and interesting for other rarely encountered features. MVH should be considered in the differential diagnoses of vascular lesions on nasal skin.

Terra Firma-Forme Dermatosis Misdiagnosed as Nevoid Acanthosis Nigricans.

Terra firma-forme dermatosis (TFFD) is a clinical condition characterized by brown-gray, velvety, pigmented patches or plaques, resembling dirt on the skin. Nevoid acanthosis nigricans (NAN) is a rare and recently described form of acanthosis nigricans occurring during childhood or early adulthood. Herein we describe a patient with TFFD, initially misdiagnosed as NAN. The patient had developed hyperkeratotic and hyperpigmented plaques on and around the umbilicus during pregnancy. Though regular in bathing practices, she could not clear away the lesions and concerned marks for inesthetic appearance. Histopathological findings were compatible with NAN, and she was prescribed 10% urea lotion. On a dramatic healing after 3 weeks, a diagnosis of TFFD is considered and confirmed by the lesions getting wiped away by vigorous rubbing with alcohol pads. We discuss the key points of differentiating TFFD from NAN, and underline the importance of alcohol test for accurate diagnosis.

Comedones Induced by Vascular Laser Therapy.

A 21-year-old female presented with acne-like blackheads on brownish areas located on the cheek. She had been treated with neodymium-doped yttrium aluminium garnet (Nd-YAG) laser (1071 nm), 160 j/cm(2), three months ago for erythema and telangiectasia of her face. Afterwards, she developed atrophic, slightly depressed, hyperpigmented, 3-4 mm scars with superimposed tiny comedones within the treated areas. Topical treatment with tretinoin 0.05% cream on alternate days, and Sun Protection Factor (SPF) 50 sunscreen daily were commenced. After 2 months, comedones and hyperpigmentation mostly resolved but mild superficial atrophy persisted. According to our knowledge, this is the first case of atrophic scars studded with open comedones, developing shortly after laser therapy used for facial telangiectasia.

Blastic plasmacytoid dendritic cell neoplasm: skin and bone marrow infiltration of three cases and the review of the literature.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a distinct and rare neoplastic entity and was classified as a subgroup of acute myeloblastic leukemia by the WHO in 2008. The median survival of patients was 15.2 months in a large case series. Allogeneic or autologous bone marrow transplantation has been recommended by some reports because of the disease's poor prognosis. We present three patients who presented with both skin and bone marrow infiltration. A 57-year-old man, a 62-year-old woman, a 64-year-old man were admitted to our outpatient clinic because of skin lesions. All of the patient's had bone marrow infiltration with positivity of the CD4, CD56, and CD123 staining. Survival of the patient's were 42, 6 and 12 months, respectively. Two of the patients who presented as blastic form didn't respond to any chemotherapy. BPDCN is a difficult disease to diagnosis and manage. CD4, CD56, CD123, CD303, and T cell leukemia/lymphoma 1. Cutaneous lesions can present as isolated nodules, macules, and disseminated macules and nodules. Positivities are crucial to the diagnosis of the disease in histological examination. Bone marrow infiltration or disease relapse at presentation were related to poor prognosis. Complete immunocytochemical staining must be performed for all patients who have cutaneous lesions with or without blood count abnormalities. Bone marrow (allogeneic or autologous) transplantation should be considered at the first remission.

Hepatitis C and hepatitis B virus infections in the etiopathogenesis of pemphigus.

BACKGROUND: Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated. OBJECTIVES: To investigate the relationship between pemphigus and the hepatitis B and C virus infections. METHODS: This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases. RESULTS: The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections. CONCLUSIONS: This study does not support an association between pemphigus and hepatitis B and/or C virus infections.

Hepatitis C and hepatitis B virus infections in the etiopathogenesis of pemphigus

BACKGROUND: Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated. OBJECTIVES: To investigate the relationship between pemphigus and the hepatitis B and C virus infections. METHODS: This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases. RESULTS: The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections. CONCLUSIONS: This study does not support an association between pemphigus and hepatitis B and/or C virus infections. .

Parotid tuberculosis associated with cutaneous tuberculosis on a medial epicanthus.

An 83-year-old woman presented with a 2-month history of a gradually enlarging, reddish, crusted papule on her left medial epicanthus. Her medical history did not reveal any systemic disease. She gave no personal history of tuberculosis or any systemic symptoms, such as night sweat, weight loss, and pulmonary abnormalities. Her husband had been treated for pulmonary tuberculosis 30 years ago. A dermatologic examination revealed a 2.5 x 1.2-cm nontender, erythematous plaque with fine, white adherent scales on the left medial epicanthus (figure 1A). All laboratory values were within the normal range. Results from a tuberculin skin test were initially negative. A skin biopsy was performed, and a pathological examination demonstrated multiple noncaseating granulomas with various diameters in the reticular dermis and an infiltrate of neutrophils and lymphocytes in the surrounding dermis (figure 2). Periodic acid-Schiff, Ziehl-Nilsen, gram, and giemsa stains were negative for any microorganism. Leishman-Donovan-like bodies were observed within the epitheloid histiocytes that formed the granulomas. The pathological diagnosis was granulomatous dermatitis. The patient was diagnosed with cutaneous leishmaniasis (CL) based on her clinical appearance and histopathological findings, although the parasite was not detected in the tissue specimens. Treatment with intralesional glucantime for 5 consecutive weeks did not improve her condition. By the end of the fifth week, the patient developed asymptomatic facial swelling and a 1.5 x 1.2-cm erythematous plaque in the left parotid area (figure 1B). An ultrasonographic examination demonstrated a 13 x 11 x 17-mm hypoechoic mass, which suggested pleomorphic adenoma. In addition, lymph nodes, the largest of which were 9 x 10 mm, were noted in the left cervical area. A skin biopsy from the erythematous plaque of the left parotid area demonstrated diffuse neutrophilic infiltration with formation of focal granulomas. Tuberculosis was suspected, and mycobacterium tuberculosis (MT) was isolated from the culture. A tuberculin skin test was performed again, which was positive (12 mm). The erythrocyte sedimentation rate was 35 mm/h, and all other laboratory tests were within normal limits. Pulmonary radiography and thoracic computerized tomography findings were normal. Fine needle aspiration biopsy and ultrasonographic examination of the parotid mass were performed, which revealed necrotic material with neutrophils and lymphocytes (figure 3). We treated the patient with a standard antituberculous regimen, comprising isoniazid 300 mg/d, rifampin 600 mg/d, ethambutol 1200 mg/d, and pyrazinamide 1500 mg/d. By the end of the second month of treatment, the patient improved considerably. There was a marked reduction in facial swelling, and the lesion on the left medial epicanthus regressed dramatically (Figure 4A and Figure 4B). No adverse effects of the medication occurred. An additional 7 months of therapy with isoniazid and rifampin was planned.

Variable clinical presentations of Classic Kaposi Sarcoma in Turkish patients.

BACKGROUND: Kaposi sarcoma (KS) is a vascular neoplasm with multicentric cutanenous and extracutaneous involvements, which was first described by Moriz Kaposi in 1872. Since then, different epidemiological clinical and histopathological variants of this neoplasm have been identified. Classic Kaposi sarcoma (CKS) is one of four main clinico-epidemiologiologic variants. characteristics of the disease. MATERIALS AND METHODS: Four Turkish inpatients with CKS were evaluated in the study. All medical history and clinical data were noted. A screening immunodeficiency workup were performed for all patients. HHV-8 immunofluorescence testing on the specimens and ELISA test for human immunodeficiency virus (HIV 1 and 2) were performed. Pulmonary X ray graphies and computurized tomography (CT) scan were applied. Stage of the tumor was determined, in each case, according to the classification system proposed by Brambilla et al in 2003. RESULTS: All patients are positive for HHV-8. They were all immunocompetent and negative for HIV1 and HIV2. The first patient was unusual for morphological presentation of several verrucoid lesions that was evaluated as verrucoid KS. He was considered stage IB CKS. The patient 2 was a young man and the course of KS seemed unexpectedly aggressive for CKS. His clinical appearence seemed us to be a patient with AIDSassociated KS. The patient was evaluated as stage IVB CKS. Our third patient had also prominent lymphedema associated with bluish discoloration on the toes and fingers, suggesting a diagnosis of peripheral vascular disorder. He was diagnosed as stage IIIB CKS. The fourth case was interesting for very extensive lesions involving big sized plaques and also the existence of mucosal lesion. The patient was diagnosed as stage IVB CKS. CONCLUSIONS: It seems that the reports of exceptional cases of KS are accumulating. Data from various cases should be collected and perhaps, novel clinical classifications should be considered.

Variable clinical presentations of secondary delusional infestation: an experience of six cases from a psychodermatology clinic.

OBJECTIVE: Delusional Infestation (DI) is a relatively rare condition with a fixed belief of being infested with living organisms, despite a lack of medical evidence of such infestation. Although it seems to be a psychiatric disease, patients commonly are admitted to dermatology clinics because of skin findings. Psychiatrists can underestimate its prevalance, whereas dermatologists can miss the diagnosis. It should be managed as a psychodermatological disease. Our aim in the study was to evaluate six patients with different clinical presentations of DI and to emphasize some clinical features. METHOD: All patients were internalized in the psychodermatology clinic for this study. Medical history and clinical data from dermatologic and psychiatric examinations were noted; Mini International Neuropsychiatric Interview (MINI-Plus) and laboratory investigations including blood and urine analyses, microscopic analysis of so-called pathogens, and skin biopsy if needed, were performed. The diagnosis was made based on detailed history, dermatologic and psychiatric examinations, and laboratory investigations. RESULTS: All patients had symptoms of itching, burning, or crawling sensations dermatologically and thus were admitted to dermatology clinic. They were all considered secondary DI to another medical condition or to psychiatric illness. Vitamin B12 deficiency, diabetes, and hypothyroidism were the underlying medical conditions. Related psychiatric illnesses were trichotillomania and schizoaffective disorder, schizophrenia, shared pychotic disorder, and brief psychotic disorder. Two patients had delusions of inanimate materials; four patients had partial and complete remissions; and two patients have dropped out. CONCLUSION: Each patient had different clinical characteristics creating diagnostic challenges. All complaints were related to the infestation of the skin. The presence of different psychiatric comorbidities is remarkable. It seems that both psychiatrists and dermatologists can face diagnostic and therapeutic challenges of this complex disease in clinical settings, particularly if there are unusual clinical features of DI. Therefore, both psychiatrists and dermatologists should be well aware of DI.

Waardenburg Syndrome type 1: A case report.

Waardenburg Syndrome (WS) is a rare hereditary disorder that is characterized by the clinical manifestations of oculocutaneous anomalies of pigmentation, congenital deafness, dystopia canthorum, and broad nasal root. It demonstrates both genetically and clinically heterogenous characteristics. In this article, we report an 11-month-old boy with WS1, one of four clinicat types of WS. He exhibited white forelock, hypopigmented macules and patches, heterochromia irides, and dystopia canthorum.

Do eating disorders accompany metabolic syndrome in psoriasis patients? Results of a preliminary study.

BACKGROUND: Metabolic syndrome (MBS) has been reported as a frequent comorbidity in psoriatic patients. The main pathogenesis is considered to be inflammation in this association. MBS has been investigated in eating disorders as well. While psoriasis has some psychiatric comorbidities, the link between psoriasis, MBS, and eating disorders (EDs) is unknown. METHODS: The study was designed as a cross-sectional, randomized, and controlled trial. A total of 100 patients with psoriasis were included in the study. Sociodemographic data, clinical subtype of psoriasis, Psoriasis Area and Severity Index (PASI) scores, and associated diseases were registered for each patient. The criteria for diagnosis of MBS developed by the International Diabetes Foundation (IDF) was used. These are central obesity (waist circumference ≥94 cm in men or ≥80 cm in women), plus two of the following: elevated triglycerides (≥150 mg/dL), reduced high-desity lipoprotein cholesterol (>40 mg/dL for men; >50 mg/dL for women), elevated blood pressure (≥130 mmHg systolic or ≥85 mmHg diastolic), and elevated fasting blood glucose (≥100 mg/dL). Additionally, the Eating Attitude Test (EAT), Beck Depression Inventory (BDI), and Beck Anxiety Inventory (BAI), and psychiatric interview were performed for all patients. RESULTS: There were 45 female and 55 male patients, aged between 18 and 85 years old (median 41.12 ± 16.01). MBS was present in 31% of the patients with psoriasis. There was no correlation between the severity of psoriasis and MBS. EAT scores were ≥30 in 7/100 patients. Four out of 31 patients with MBS (12.9%) had ED and 3/69 patients were without MBS (4.3%). Mean ED scores were compared statistically and the difference was significant (EAT = 17.9 ± 9.558 and 11.5 ± 7.204, P < 0.001). CONCLUSION: Defining risk factors leading to comorbidities is important in psoriasis. EDs seem to have an impact on the development of MBS in psoriasis. Establishment and treatment of EDs in patients with psoriasis may prevent the onset of MBS and other comorbidities due to MBS.

A giant benign clear cell hidradenoma on the anterior trunk.

Clear cell hidradenoma (CCH) is an uncommon variant of benign cutaneous adnexal tumors. These tumors are clinically asymptomatic, solitary dermal nodules. They occur most frequently on the scalp, face abdomen and extremities. Growth is slow and malignant change is rare. 45-year-old woman presented with a nodule which had begun 4 years ago as a small nodular asymptomatic lesion and had a central ulceration and a minimal hemorrhagic discharge on her anterior abdomen wall. On dermatologic examination there was a 6.5×5×4 cm non-tender, soft reddish purple nodule, with lobular appearance and ulceration. In the laboratory investigations, all hematologic and biochemical tests were normal. A computed tomography (CT) scan demonstrated a cystic tumor with lobulated contour with contrast enhancement. The lesion was excised totally. In histopathological examination, the tumor was composed of biphasic smaller dark polygonal cells and larger clear cells and coarse nuclear chromatine. There were duct like structures. Immunohistochemical investigation was done for the suspicion of malignancy. Cytoplasm of clear cells and of duct like structures showed PAS-positive and d-PAS resistant staining. There was a positive reaction to epithelial membrane antigen and carcinoembryonic antigen. The mitotic index in Ki 67 examination was low. All these findings confirmed the diagnosis of benign CCH.

Lupus erythematosus and lichen planus overlap syndrome:a case report with a rapid response to topical corticosteroid therapy.

Lupus erythematosus (LE) and lichen planus (LP) may occur as an overlap syndrome. We report the clinical characteristics of a young man with lesions diagnosed as LE and LP by histopathological and direct immunoflurosence examinations. We achieved remarkable clinical response from the treatment with topical corticosteroids and no recurrence was seen in a 6 months of follow up time. We found this case interesting because of the rapid improvement with corticosteroid and discussed if there is a real overlap or a coexistence according to the literature.

Case Report of Bullous Pemphigoid following Fundus Fluorescein Angiography.

PURPOSE: To report a first case of bullous pemphigoid (BP) following intravenous fluorescein for fundus angiography. Clinical Features: A 70-year-old male patient was admitted to the intensive care unit with BP and sepsis. He reported a history of fundus fluorescein angiography with a pre-diagnosis of senile macular degeneration 2 months prior to presentation. At that time, fluorescein extravasated at the antecubital region. Following the procedure, pruritus and erythema began at the wrists bilaterally, and quickly spread to the entire body. The patient also reported a history of allergy to human albumin solution (Plamasteril(R); Abbott) 15 years before, during bypass surgery. On dermatologic examination, erythematous patches were present on the scalp, chest and anogenital region. Vesicles and bullous lesions were present on upper and lower extremities. On day 2 of hospitalization, tense bullae appeared on the upper and lower extremities. The patient was treated with oral methylprednisolone 48 mg (Prednol(R); Mustafa Nevzat), topical clobetasol dipropionate 0.05% cream (Dermovate(R); Glaxo SmithKline), and topical 4% urea lotion (Excipial Lipo(R); Orva) for presumptive bullous pemphigoid. Skin punch biopsy provided tissue for histopathology, direct immunofluorescence examination, and salt extraction, which were all consistent with BP. After 1 month, the patient was transferred to the intensive care unit with sepsis secondary to urinary tract infection; he died 2 weeks later from sepsis and cardiac failure. CONCLUSIONS: To our knowledge, this is the first reported case of BP following fundus fluorescein angiography in a patient with known human albumin solution allergy. Consideration should be made to avoid fluorescein angiography, change administration route, or premedicate with antihistamines in patients with known human albumin solution allergy. The association between fundus fluorescein angiography and BP should be further investigated.

Ross syndrome: Unilateral hyperhidrosis, Adie's tonic pupils and diffuse areflexia.

Ross syndrome is a rare disorder first described in 1958 with partial autonomic dysfunction. It has three basic components including unilateral or bilateral segmental anhidrosis, Adie's tonic pupils and areflexia or hyporeflexia of deep tendon reflexes. The most disturbing symptom in the patients is segmental compensatory hyperhidrosis and often the hypohidrosis or anhidrosis is not even noticed. While the pathogenesis of Ross syndrome is unclear, degenerative changes or damage to the peripheral autonomic nerve system or dorsal root ganglia have been suggested as possible causes. About 50 cases have been reported, usually by neurologists and ophthalmologists, and less often by dermatologists. We present a 26-year-old patient who displayed the classic triad of this syndrome, emphasizing that the presenting complaint may be hyperhidrosis and that multidisciplinary evaluation in neurology and ophthalmology is essential.