Neusa Yuriko Sakai Valente

Phaeohyphomycotic Cyst Caused by Colletotrichum crassipes

ABSTRACT A case of phaeohyphomycosis is reported in a male renal transplant recipient with a nodular lesion in the right leg who was treated with immunosuppressing drugs. The lesion consisted of a purulent cyst with thick walls. The cyst was excised surgically, and the patient did not receive any antifungal therapy. One year later he remains well. Histological study of the lesion showed a granulomatous reaction of epithelioid and multinucleate giant cells, with a central area of necrosis and pus. Fontana-Masson staining demonstrated the presence of pigmented hyphal elements. The fungus Colletotrichum crassipes was grown in different cultures from the cyst. The in vitro inhibitory activities of eight antifungal drugs against the isolate were tested. Clotrimazole and UR-9825 were the most active drugs. This case represents the first known reported infection caused by this rare species.

Topical Application of Imiquimod as a Treatment for Chromoblastomycosis

Chromoblastomycosis is a subcutaneous mycosis that remains a therapeutic challenge, with no standard treatment and high rates of relapse. On the basis of our recent discoveries in mouse models, we tested the efficacy of topical applications of imiquimod to treat patients afflicted with this chronic fungal infection. We report results of treatment for the first 4 recipients of topical imiquimod, all of whom displayed a marked improvement of their lesions, both with and without concurrent oral antifungal therapy.

Cromoblastomicose: relato de 27 casos e revisão da literatura

BACKGROUND: Chromoblastomycosis is a subcutaneous mycosis that occurs mainly in rural workers although is being more commonly found among people working in other sectors. The fungus penetrates the skin after its inoculation and the most frequently isolated agent is the Fonsecaea pedrosoi. OBJECTIVES: This study aims at evaluating patients suffering from chromoblastomycosis admitted into the Department of Dermatology of the University Hospital of the Faculty of Medicine of Sao Paulo State during the ten-year period from 1997 to 2007. METHODS: It is a retrospective study and the medical report cards of 27 Brazilian patients diagnosed as suffering from Chromoblastomycosis from 1997 to 2007 at the Dermatology Department of the Medical School, University of Sao Paulo were reviewed. The following items were analyzed: previous therapeutic approaches; treatment implemented by the group; length of time between the appearing of the lesion and diagnosis; age; gender; profession; origin; site of lesions; isolated agents found in culture and histopathology. RESULTS: Twenty two patients were from the state of Sao Paulo whereas the others came from the states of Bahia and Rondonia. 37% of them were rural workers. Men were more frequently infected (85%). Lesions were more commonly found on the lower limbs (59.2%). In 52% of the cases the isolated agent was the dematiaceous fungus Fonsecaea. pedrosoi. Biopsies showed sclerotic bodies in 92.5% of the cases. CONCLUSION: Data found are in accordance with medical literature on the subject. The disease had been previously studied in our institution in 1983 by Cuce et al. This present study is the second retrospective one about the characteristics of patients suffering from chromoblastmycosis which has been published in indexed medical literature in the state of Sao Paulo.

Urticaria unresponsive to antihistaminic treatment: An open study of therapeutic options based on histopathologic features

Background: The non‐ or low‐sedating H1 receptor antagonists represent the basic therapy for urticaria. Objective: To test an alternative approach to patients unresponsive to conventional treatment. Materials and methods: A total of 22 patients with chronic urticaria unresponsive to conventional antihistamine treatment were enrolled for this study. They had uncontrolled urticaria even using multiple combinations of antihistamines on maximum doses and corticosteroids in short cycles (prednisone 20–40 mg, per os once a day, 3–7 days per month). Cutaneous biopsies of the urticaria lesions were taken. These findings were classified as: (I) a mixture of perivascular dermal inflammatory infiltrate composed of lymphocytes, monocytes and neutrophils and/or eosinophils; (II) inflammatory infiltrate composed chiefly of neutrophils; and (III) inflammatory infiltrate composed mainly of eosinophils. According to histology, the patients were submitted to one of the following therapeutic schemes: class A – antihistamine treatment plus dapsone; class B – colchicine or dapsone; class C – montelukast. Results: Four patients in class A, 08 in class B and seven in class C displayed complete control of urticaria after 12 weeks of treatment; one patient in class B and two in class C did not respond to treatment. Two years after discontinuation, 16 patients are still free of urticaria. Conclusions: This study suggests an alternative approach for treating unresponsive chronic urticaria.

Glabellar red dots in frontal fibrosing alopecia: a further clinical sign of vellus follicle involvement

DEAR EDITOR, Frontal fibrosing alopecia (FFA) was first described by Kossard as a variant of lichen planopilaris (LPP) that affects mainly postmenopausal women. A progressive frontal or frontotemporal symmetric band of alopecia is the usual presentation, with eyebrows commonly involved. Reports of hair loss at other areas such as limbs and axillary and pubic regions with involvement of terminal, intermediate and vellus hair follicles in any stage of the hair cycle, from anagen to telogen, have expanded the spectrum of the disease, suggesting that FFA is, in fact, a generalized skin condition. Involvement of facial vellus hair follicles in FFA was first described by Donati et al. Unlike the noninflammatory hair loss resulting from vellus involvement at other body sites, affected facial vellus hair follicles were associated with skin surface changes, namely, facial papules. We describe three patients with FFA with a clinical feature that has not been previously reported: the presence of numerous follicular red dots (FRD) in the forehead. All patients were white women (age range 57–67 years) with disease duration ranging from 3 to 5 years. None of them was currently receiving any treatment for this condition. On examination, apart from clinical signs such as frontotemporal recession of the hairline, we noticed FRD that could be restricted to the glabellar zone as seen in two patients (Fig. 1a,c), but that could also be perceived more extensively in the forehead (one patient, Fig. 1b). In two subjects, FRD were associated with follicular keratosis (Fig. 1a). In order to correlate clinical and pathological features, punch biopsies from areas presenting the FRD pattern were obtained from two patients. Both specimens showed perifollicular lichenoid lymphocytic inflammatory infiltrate involving vellus hairs, histological features suggestive of vellus hair follicle involvement by LPP (Fig. 2). We also performed a retrospective evaluation of clinical images of 69 patients with FFA, which revealed three other female patients with the FRD pattern present in the glabellar region. FRD were first described by Tosti et al. as a trichoscopic feature of active discoid lupus erythematosus (DLE) of the scalp and its presence associated with better prognosis. It has been suggested that the overlying atrophic epidermis of DLE lesions would allow the visualization of the rich vascular net that naturally envelops the normal hair follicle. FRD in DLE lesions would therefore represent still viable hair follicles responsible for the better chance of hair regrowth reported in those patients. FRD have also been described in the eyebrows of patients with FFA. Even though no histopathological correlation was made by the

Two case reports of cutaneous adverse reactions following hepatitis B vaccine: lichen planus and granuloma annulare.

We report two cases of adverse cutaneous reactions following hepatitis B vaccination. The first case occurred 3 weeks after the first dose of hepatitis B vaccine in a 16‐year‐old white girl with the onset of lichen planus lesions on her thighs and abdomen. After the second dose a disseminated lichen planus developed within 2 weeks. The second case concerns to the development of papular and patch granuloma annulare in a 58‐year‐old white woman 2 months after the second dose of hepatitis B vaccine. To the best of our knowledge, only a few paediatric and adult cases of lichen planus as a complication of hepatitis B vaccination have been reported in medical literature so far. This is the second case of granuloma annulare following hepatitis B vaccine. Our report, similar to earlier papers, appears to support the onset of lichen planus and granuloma annulare as a possible rare complication of hepatitis B immunization.

Exuberant juvenile hyaline fibromatosis in two patients.

Abstract:  Juvenile hyaline fibromatosis and infantile systemic hyalinosis are rare autosomal recessive disorders of infancy and early childhood that are histologically characterized by deposition of hyaline material. The main clinical features are papulo‐nodular skin lesions, gingival hypertrophy, joint contractures, and bone abnormalities. However, infantile systemic hyalinosis has a more severe clinical presentation, including visceral involvement and premature death. Very recently, genetic studies identified mutations in the same gene in patients with both conditions, strongly suggesting that they belong to the same disease spectrum. We report two new nonrelated patients who met the criteria for the diagnosis of juvenile hyaline fibromatosis/infantile systemic hyalinosis. Clinical, histopathologic, immunohistochemical, and ultrastructural findings are presented, as well as an extensive review of the literature. Recent information regarding pathogenesis and treatment is discussed.

Melanoma maligno cutâneo primário: estudo retrospectivo de 1963 a 1997 no Hospital do Servidor Público Estadual de São Paulo

OBJECTIVES: A retrospective study on the Primary Cutaneous Malignant Melanoma in the Hospital do Servidor Publico Estadual de Sao Paulo (HSPE-SP) analyzing its distribution according to age, sex, race and cutaneous site. METHODOLOGY: We studied 222 patients with Primary Cutaneous Malignant Melanoma as diagnosed at Hospital do Servidor Publico Estadual de Sao Paulo, Brazil between the period from 1963 to 1997. A retrospective study was performed. Data were expressed as inance of caucasians (98.19%) over afro-americans (1.81) and of the female sex (69.36%) over the male sex (30.63%) was found. The predominant age on the occasion of the diagnosis was between 50 and 60 years for the women (25.32%) and between 60 and 69 years for the men (22.52%). The most frequent site of the cancer in men was the back region (29.41%) and in the lower members in the women (38.31%). The most frequent level of the tumor invasion (Clark) was IV (39.77%), and the average of tumor thickeness (Breslow) was < 0,75mm (28.4%). A 5 years survival was observed in 73.3% of the patients. CONCLUSIONS: At our Hospital the incidence of Primary Cutaneous Malignant Melanoma has shown an increase in recent years; these results are compatible with the most recent international surveys.OBJECTIVES A retrospective study on the Primary Cutaneous Malignant Melanoma in the Hospital do Servidor Público Estadual de São Paulo (HSPE-SP) analyzing its distribution according to age, sex, race and cutaneous site. METHODOLOGY We studied 222 patients with Primary Cutaneous Malignant Melanoma as diagnosed at Hospital do Servidor Público Estadual de São Paulo, Brazil between the period from 1963 to 1997. A retrospective study was performed. Data were expressed as inance of caucasians (98.19%) over afro-americans (1.81) and of the female sex (69.36%) over the male sex (30.63%) was found. The predominant age on the occasion of the diagnosis was between 50 and 60 years for the women (25.32%) and between 60 and 69 years for the men (22.52%). The most frequent site of the cancer in men was the back region (29.41%) and in the lower members in the women (38.31%). The most frequent level of the tumor invasion (Clark) was IV (39.77%), and the average of tumor thickeness (Breslow) was < 0.75 mm (28.4%). A 5 years survival was observed in 73.3% of the patients. CONCLUSIONS At our Hospital the incidence of Primary Cutaneous Malignant Melanoma has shown an increase in recent years; these results are compatible with the most recent international surveys.

Clinical and dermoscopic features of lichen planus pigmentosus in 37 patients with frontal fibrosing alopecia.

DEAR EDITOR, The coexistence of frontal fibrosing alopecia (FFA) and lichen planus pigmentosus (LPPigm), an uncommon macular variant of lichen planus, was first reported by Dlova in 2013 in South African patients. Herein, we report 37 patients with such an association, describe the clinical and dermoscopic features of facial LPPigm and compare our findings with previously published data. In total, 37 patients aged 34–79 years were included [36 women (30 postmenopausal, six premenopausal) and one man]; 33 had skin phototype IV or higher (hispanics and people of African descent) and three had skin phototype III. All patients presented with frontotemporal hair loss; three (8%) had the recently described pseudo ‘fringe sign’. Facial papules were noted in 16 (43%), eyebrow involvement in 36 (97%) and body hair loss in 27 (73%). All patients presented with facial hyperpigmentation, which we classified as diffuse in 25 (67%), reticulated in eight (22%) and comprised of multiple pigmented macules in four (11%) (Fig. 1a–c). In 14 (38%), neck involvement was also prominent. In 19 patients, LPPigm preceded FFA presentation, in five it followed diagnosis of FFA, in one patient FFA and LPPigm were diagnosed simultaneously and in 12 patients precise disease onset was unable to be confirmed. Punch biopsies were performed in 14 patients, confirming a clinical diagnosis of LPPigm, which showed an interface dermatitis pattern and/or pigment incontinence, occasionally involving hair follicles and eccrine glands (Fig. 1d–f). Epidermal atrophy and discrete perivascular lymphocytic infiltrate were commonly observed. Dermoscopy and photographic documentation of facial lesions were performed in all cases using either Fotofinder Dermoscope (nonpolarized; Teachscreen Software, Bad Birnbach, Germany), Fotofinder Handyscope (Teachscreen Software) or DermLite Foto II Pro (3Gen, San Juan Capistrano, CA, U.S.A.) attached to a Canon EOS T3i camera (polarized; Canon, Tokyo, Japan). The photographs were analysed by all authors and four distinct patterns of pigmentation were identified: (i) pseudonetwork (n = 24); (ii) dotted pattern (n = 8); (iii) speckled blue–grey dots (n = 13); and (iv) blue–grey dots arranged in circles (n = 7) (Fig. 1g–k), with 14 (38%) patients showing more then one pattern. Additionally, rhomboidal structures were noted in five patients with a pseudonetwork pattern and asymmetry of follicular openings in one (Fig. 1l, m). There was no correlation between the clinical type of pigmentation and one specific dermoscopic pigmentation pattern. Vascular alterations were assessed in 25 cases, and 12 presented focal erythema or telangiectasias and three diffuse erythema (Fig. 1g–i). Twenty-four patients presented complete or partial loss of facial vellus hair. In accordance with the initial description and later reports, coexistence of FFA and LPPigm in our series was observed mostly in dark-skinned patients (89%). Most of our patients were postmenopausal; only 17% were premenopausal. This differs from the series of Dlova, which included 64% premenopausal patients, but is similar to the recently published large series of patients with FFA. In the same study by Dlova, LPPigm preceded hair loss in all patients and was considered as a herald sign of FFA. A clear history of LPPigm preceding FFA was present in only 19 (51%) of our patients. However, considering that early signs of FFA and LPPigm can go unnoticed in patients, and a possible recall bias, we consider assessment of the predictive status of LPPigm as a harbinger of FFA to be hampered in the present study. The blue–grey hue observed through dermoscopy can be justified by the depth of the pigment present in the dermis, and has been described in lesions of lichen planus pigmentosus inversus. Speckled blue–grey dots and the pseudonetwork patterns possibly correlate with a more prominent interfollicular interface dermatitis; the former resulting from scattered foci of inflammation with melanophages, and the latter from a broader damage to the epidermal basal cell layer. Demonstration of eccrine and follicular involvement in biopsy specimens supports that the dotted pattern and circles result from damage to those structures, respectively. Blue–grey or brown perifollicular pigmentation in facial macules resembling LPPigm have been recently reported in patients with FFA and might correspond to the circles observed in our patients. Loss of facial vellus hairs also results from hair follicle involvement and has been previously described in FFA. Vascular changes might be found in the context of an inflammatory process and epidermal atrophy might have facilitated their visualization by dermoscopy. Clinically, the differential diagnosis of LPPigm should be established with a heterogeneous group of disorders causing facial hyperpigmentation, such as exogenous ochronosis, melasma, Riehl’s melanosis and ashy dermatosis. Hydroxychloroquine-induced hyperpigmentation should also be

Elejalde syndrome: report of a case and review of the literature.

Abstract:  Elejalde syndrome is a rare autosomal recessive condition, with only 10 reported cases through 2001. It is characterized by silvery hair, pigment abnormalities, and profound central nervous system dysfunction. The differential diagnosis includes Griscelli and Chediak‐Higashi syndromes, which present with silvery hair, pigment abnormalities, central nervous system alterations, and severe immunologic dysfunction. We report a 6‐year‐old girl with Elejalde syndrome and review Elejalde, Griscelli, and Chediak‐Higashi syndromes.