Ayşe Tülin Mansur

Anticonvulsant hypersensitivity syndrome: clinical and laboratory features.

Background  Anticonvulsant hypersensitivity syndrome (AHS) is a severe adverse drug reaction with multiorgan involvement. Aromatic anticonvulsants are the most frequently involved drugs. This study was aimed to highlight the clinical and laboratory findings of AHS.

Pyoderma gangrenosum on the breast: A case presentation and review of the published work

We present a case of pyoderma gangrenosum localized on the breast, without a preceding surgical intervention and associated systemic disorder. The ulcer had rapidly developed and covered a large portion of the breast. The patient responded well to systemic steroids and salicylazosulfapyridine and the ulcer completely healed with scarring after 3 months of treatment. Pyoderma gangrenosum rarely involves the breasts. A published work survey disclosed only 31 reported cases up to date. In most of these cases the lesions were related to previous surgical interventions, probably as the result of a pathergy phenomenon. The main differential diagnoses were skin and soft tissue infections including necrotizing fasciitis, and malignant neoplasms. Negative initial wound cultures and the relative sparing of nipple/areola complex helped to eliminate these disorders. Though an unusual site for pyoderma gangrenosum, lesions on the breast showed the characteristic clinical features of the disease. The types of associated disorders were also similar to those of the cases with classical pyoderma gangrenosum. As most of the lesions healed with significant scarring, early recognition and treatment of pyoderma gangrenosum located on the breast is important to prevent serious physical and psychological morbidity.

Marie Unna hereditary hypotrichosis: A Turkish family with loss of eyebrows and a U2HR mutation†

We report a family with Marie Unna hereditary hypotrichosis (MUHH) from Turkey. MUHH is a distinct form of scalp and body hair loss characterized by the absence or scarcity of scalp hair, eyebrows, and eyelashes at birth. Coarse wiry hair begins to grow during childhood. Around puberty, progressive hair loss occurs in the affected patients. Recently, mutations were identified in U2HR, an inhibitory upstream open reading frame in the 5′‐untranslated region of the human hairless gene (HR) as the underlying cause of MUHH. We are presenting hair loss of eyebrows in a Turkish family comprising eight affected and seven unaffected individuals. The pedigree is compatible with autosomal dominant inheritance. Linkage and haplotype analyses confirmed linkage of this family to the MUHH locus at cytoband 8p21. By sequencing U2HR, we identified the mutation c.2T > C (M1T) in all affected family members. We concluded that there may be considerable clinical variations in MUHH, and that eyebrow loss is an important clue for accurate diagnosis.

Idiopathic CD4^+ T lymphocytopenia with epidermodysplasia verruciformis-like skin eruption, Nocardia farcinica brain abscesses and pulmonary tuberculosis : A case report with fatal outcome

Dear Editor, Idiopathic CD4 T lymphocytopenia (ICL) defined by the US Centers of Diseases Control and Prevention is characterized by decreased CD4 cell count (<300 cells ⁄lL or <20% of total T-cell count) on a minimum of two occasions at least 6 weeks apart, without laboratory evidence of HIV-1 or HIV-2, and the absence of any other immunodeficiency and therapy related with CD4 T-cell lymphocytopenia. Here we report a fatal case of ICL with pulmonary tuberculosis, disseminated epidermodysplasia verruciformis (EV)-like flat warts and Nocardia brain abscesses. A 19-year-old heterosexual man was admitted with itchy skin papules present since childhood. Medical history revealed a left cerebellar and a right premotor frontal abscess excised 2 months ago (Fig. 1a,b). Aerobic cultures of abscess content in sheep blood agar, chocolate agar and Löwenstein–Jensen yielded Nocardia (N) farcinica (Fig. 1c,d). The isolate was resistant to trimethoprim–sulphamethoxazole, but susceptible to amoxicillin–clavulanic acid and ciprofloxacin. The last two drugs were commenced at daily doses of 3 and 1.5 g, respectively. Past medical history was remarkable for pulmonary tuberculosis treated at the age of 10 years, and two occasions of purulent meningitis at the ages of 6 and 11 years. There was no consanguinity; he and his parents denied smoking, alcohol consumption and drug abuse. Dermatological examination revealed multiple, scaly and coalescing flat papules of 3–5 mm located on the face, neck, upper trunk, arms and dorsal aspects of the hands (Fig. 2a,b). Laboratory investigations including complete blood cell count, blood biochemistry, immunoglobulins, erythrocyte sedimentation rate, urinalysis, rheumatoid factor, hepatitis B surface antigen (HBsAg), antiHBsAg, anti-hepatitis C virus, HIV-1 RNA polymerase chain reaction, Toxoplasma immunoglobulin (Ig)G and IgM, rapid plasma reagin, mono-test, rubella IgM, Epstein–Barr Virus (EBV) viral capsid antigen (VCA) IgM, and antinuclear antibodies were within normal limits or negative. EBV VCA IgG test was positive and purified tuberculoprotein was anergic. Absolute cell counts and percentages of CD4 T cells in two measures performed at 6 weeks apart were 234 cells ⁄lL, 19.4% and 258 cells ⁄lL, 21% respectively. On these occasions, CD8 T cells were within normal limits and CD4 ⁄CD8 ratios were low (0.58 and 0.46). Anti-HIV-1 ⁄2 enzyme-linked immunosorbent assay test results were negative on seven occasions carried on intermittently. Chest X-ray was normal. Histological examination of papular lesions showed epidermal dysplasia (Fig. 3a,b). Ki-67 staining was positive on almost the whole epidermis (Fig. 3c). In the formalin-fixed, paraffin-embedded specimens of skin lesions, human papilloma virus (HPV)-16 DNA was detected by polymerase chain reaction. Skin lesions were treated with multiple sessions of cryotherapy with partial response. At the 6th month of treatment, investigations revealed cytomegalovirus IgM positivity, hepatosplenomegaly and cholelithiasis. Four months later, the patient returned with cough and expectoration. In the meantime, he had stopped antibiotherapy. Thorax computed tomography (CT) revealed multiple parenchymal nodular infiltrations in both lungs. Amoxicillin–clavulanic acid and ciprofloxacin were restarted. After 1 month of treatment, infiltrations regressed considerably. At the 20th month of follow up, while he was still on antibiotherapy, the patient

Systemic lupus erythematosus presenting as pyoderma gangrenosum in two cases.

Pyoderma gangrenosum (PG) is an uncommon, idiopathic, ulcerative neutrophilic dermatosis. It is characterized by painful nodular, bullous or pustular lesions that eventually ulcerate [1]. In many cases, PG is associated with a wide variety of diVerent disorders [2, 3] but SLE in association with PG is relatively uncommon. In all reports of these prior co-occurrences, SLE preceded the onset of PG; and most of these cases revealed positive lupus anticoagulant (LA) and anticardiolipin antibodies (ACA). Here, we report two cases of PG, one developing concurrent with and the other shortly after the onset of SLE, in the absence of ACA and LA.

Facial Candida folliculitis: possible role of sexual contact.

Candida albicans (C. albicans) organisms are common commensals of humans and are commonly found on skin, throughout the entire gastrointestinal tract, and in the female genital tract (Edwards Jr JE. Mandell, Dougles, and Bennett s Principles and Practice of Infectious Diseases, 6th edition, Elseviere Church. Liv., Philadelphia, 2005). Folliculitis due to Candida spp. is a very rare disease (Süss K et al., Mycoses. 1999; 42: 683–5). Herein we report a healthy man with facial Candida folliculitis (CF) due to C. albicans, and discuss the predisposing factors that may have played a role.

Correspondence: Retiform hemangioendothelioma presenting as a hyperhidrotic tumor

References 1 Lin RL, Janniger CK. Pyogenic granuloma. Cutis 2004; 74: 229–233. 2 Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases. Pediatr Dermatol 1991; 8: 267–276. 3 Holbe HC, Frosch PJ, Herbst RA. Surgical pearl: ligation of the base of pyogenic granuloma – an atraumatic, simple, and cost-effective procedure. J Am Acad Dermatol 2003; 49: 509–510. 4 Nishimura Y, Yamamoto Y, Kadota M. Ligation therapy for pyogenic granuloma. J Dermatol 2004; 31: 699–700.

Case of keratosis lichenoides chronica with atypical sarcoidal granulomatous inflammation.

Keratosis lichenoides chronica (KLC) is a rare chronic disease characterized by violaceous, papular and nodular lesions typically arranged in a linear and reticulate pattern. The etiology of KLC is unknown, but it may be associated with internal diseases such as hypothyroidism, glomerulonephritis and lymphoproliferative disorders. Herein, we describe the case of 44‐year‐old male patient with characteristic lesions of KLC on the trunk and extremities, present for 12 years. The clinical diagnosis was proven by histopathological examination on several occasions. In the years following the diagnosis of KLC, he developed bilateral hilar and multiple mediastinal, cervical and inguinal lymphadenopathies and hepatosplenomegaly. In 2000, diffuse interstitial and then reticulonodular pulmonary infiltrates associated with fever, weight loss, malaise and subcutaneous nodules developed. Biopsies taken from peripheral and mediastinal lymph nodes, pulmonary parenchyma, pleural tissue, bone marrow and skin showed non‐necrotizing granulomas, indicating a sarcoidal granulomatous reaction. Characteristic histopathological findings and the absence of atypical cells, clonality and a high proliferative index excluded lymphomas. Furthermore, detailed tests showed no evidence of an infectious granulomatous disease. As far as we know, this is the first reported case of KLC associated with a sarcoidal granulomatous reaction.

Annular lichen planus-like keratosis: clues to pre-existing porokeratosis

Lichen planus-like keratosis (LPLK), or benign lichenoid keratosis, is usually a solitary, scaling, hyperkeratotic, papular lesion, and is generally considered as an involuting epidermal inflammatory process that leaves a pigmented lesion behind. It was first described in 1966 by Shapiro and Ackerman, since which time its clinical, histologic and even dermoscopic variants have been defined. Herein, we report an annular LPLK, displaying unusual clinical and histopathologic features.

Development of multiple non-melanoma skin carcinomas in a patient with non-bullous congenital ichthyosiform erythroderma

Dear Editor, Non-bullous congenital ichthyosiform erythroderma (NBCIE; Online Mendelian Inheritance in Man no. 242100) is an autosomal recessive congenital keratinization disorder. In this paper, we report a male patient with NBCIE who developed multiple non-melanoma skin carcinomas (NMSC) in both sunexposed and non-sun-exposed areas. The patient was a 55-year-old man, presenting with an axillary tumor, which has grown gradually over the past 6 months. His medical history revealed the presence of redness and scaling, which had covered most of his body since early childhood, but no history of bullous lesions. Two basal cell carcinomas (BCC) had been excised from the malar region and dorsum of his right hand 1 year ago. His medical history was remarkable for chronic bronchitis. The patient’s medical history was not remarkable for hidradenitis suppurativa chronica or recurrent infection on the left axilla. He was born to consanguineous parents (who were first cousins), and his skin type was II. Two of his eight siblings had a similar skin disease. He had been exposed to intense sunlight since childhood due to farm life. Dermatological examination revealed widespread erythema and thin, white scales covering his body, in addition to scarring alopecia, ectropion, hypoplastic gingiva, nail deformities and palmoplantar hyperkeratosis (Fig. 1a–d). A large, fluctuant mass of 7 cm · 8 cm, exhibiting an ulcer with purulent secretion, was present in the left axilla (Fig. 1e). A hyperkeratotic, erosive nodule of 1 cm · 1 cm was noticed on the dorsum of the left hand. Regional lymphadenopathy was not present. Laboratory examinations revealed no abnormalities, other than a moderately elevated erythrocyte sedimentation rate and increased C-reactive protein. Chest X-rays showed pleural calcifications on the right side. A computed tomography scan of the thorax demonstrated calcific thickening of the pleura, as well as pretracheal and pericarinal lymphadenopathies. Magnetic resonance imaging of the left axillary region revealed a mass of 80 mm · 70 mm · 75 mm with sharp borders. There was no sign of invasion in the adjacent bones and muscles. Abdominal ultrasonography was normal. As the tuberculin purified protein derivative test was negative, and there was no acid-fast bacillus in the sputum, pneumopleural changes were considered to be inactive tuberculosis. The histopathological analysis of the erythrodermic skin showed compact hyperkeratosis, focal parakeratosis, hypergranulosis and acanthosis, compatible with NBCIE (Fig. 2a). The axillary tumor was excised, together with neighboring muscular tissue and regional lymph nodes, which revealed infiltration of atypical and typical squamous cells, extending from epidermis to dermis, with involvement of an intratumoral lymph node (Fig. 2b,c). The undifferentiated cell rate was 75%, and grade 3 squamous cell carcinoma (SCC) was diagnosed according to the Broders classification. No perineural invasion was detected. Microscopic findings of the hand nodule were consistent with superficial invasive SCC. The polymerase chain reaction analysis of the erythrodermic skin and the axillary tumor failed to demonstrate human papilloma virus DNA. Acitretin 25 mg ⁄day was commenced for prophylaxis of additional NMSC. Two months later, local tumor recurrence was observed, accompanied by erythema, edema and immobilization of the left arm. Assessment with 20 mCi technetium-99m methylene diphosphonate bone scintigraphy showed increased activity, which was consistent with metastasis on the left shoulder, proximal humerus and left hemithorax. At this stage,