Anna Agusti-Mejias

Severe Refractory Atopic Dermatitis in an Adolescent Patient Successfully Treated with Ustekinumab

Received May 30, 2012, Revised July 11, 2012, Accepted for publication September 20, 2012 Corresponding author: Francesc Messeguer, Deparment of Dermatology, Instituto Valenciano de Oncologia, Mateo Pueyo nr.6, 5 CP:46800, Xativa-Valencia, Spain. Tel: 34-685-529-231, Fax: 34-961-114-342, E-mail: francescmb@comv.es This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Fig. 1. Patient lesions in January 2011, erythematous scaly plaques over 80% of her total body surface (SCORing atopic dermatitis [SCORAD]: 23). Severe Refractory Atopic Dermatitis in an Adolescent Patient Successfully Treated with Ustekinumab

Factores de riesgo para el desarrollo de metástasis cutáneas locorregionales como forma única de recaída en los pacientes con melanoma

BACKGROUND While locoregional cutaneous metastases (in transit and satellite) in melanoma have received little attention from researchers to date, they have pathogenic and prognostic features that distinguish them from other forms of locoregional recurrence. Identifying predictors of these metastases would be of great value for their prevention, early diagnosis, and treatment. The aim of this study was to identify the risk factors associated with locoregional cutaneous metastases as the first form of recurrence in the metastatic progression of melanoma. MATERIAL AND METHODS Between 2000 and 2010, we prospectively collected the data of 1327 patients diagnosed with stage I and II melanoma. During follow up, 112 patients (8.4%) developed metastases. Of these, 36 had exclusively locoregional cutaneous metastases. The clinical and histological characteristics of this subgroup were evaluated. RESULTS In the univariate analysis, significant predictors were patient age, primary tumor thickness, site, ulceration, mitotic index, and histological type. After multivariate analysis, the independent risk factors were tumor thickness (risk ratio [RR] 5.6; 95% CI: 2.7-11.5) and the location of the primary tumor on the lower limbs (RR 3.4; 95% CI: 1.0-11.5), on the head or neck (RR 4.8; 95% IC: 1.7-13.5), or in acral sites (RR 6.7; 95% IC: 2.2-20.8). CONCLUSION Patients who have melanomas with a Breslow thickness of more than 2mm located on the lower limbs, head, neck, or acral sites have a higher risk of developing locoregional cutaneous metastases. These findings could be useful in the design of future guidelines for the monitoring and management of melanoma.

Cutaneous involvement in multiple myeloma: report of two cases.

cerebral xanthomatosis nor dyslipoproteinemia. The xanthomas were removed by curettage and CO2-laser therapy. Consistent complex decongestive therapy (CDT) with manual lymphatic drainage, bandaging and compression garments were applied both preand postoperatively. Two years after commencing CDT, no further xanthomas have appeared. The association of papular xanthomas in chronic lymphedema is found especially in adolescents with primary lymphedema. There is no sex preference. The yellowish to red-brown papules with smooth or verruciform surface and a tendency to confluence develop some months to a few years after manifestation of the lymphedema. The histological pattern is characterized by subepidermal accumulation of foam cells and signs of lymph stasis. There is normolipidemia and no association with other diseases. Hunter et al. proposed that the development of xanthomas on lymphedema result from the phagocytosis of lipoproteins by tissue macrophages, because lipoproteins accumulate secondarily in the intercellular tissue clefts due to hydrostatic pressure or lymphatic obstruction. In patients with neither lipoprotein abnormalities nor associated systemic diseases, local dermal tissue abnormalities seem to play the major role in normolipemic xanthoma formation. Chronic lymphedema can selectively damage a cutaneous district and facilitate the onset of neoplasms, infections and immune disorders. The alteration of immunity is based on abnormal trafficking of immunocompetent cells through the lymphatic channels and altered signaling of neuromediators. This local immune imbalance may lead to an increased leakage of accumulated lipoproteins into the dermis. In terms of general pathology, the designation ‘‘lymphostatic xanthomatosis’’ defines a potential sequela of chronic primary lymphedema. Therefore, inappropriate or missing therapy of the lymphedema may be etiological for frequent relapses. The surgical therapeutic options consist of curettage, excision, electrodissection or laser removal. However, xanthomas can only be resolved permanently in combination with a consistent complex decongestive therapy.

Erythema elevatum diutinum mimicking a vesiculobullous disease

A 68-year-old healthy woman came to our clinic with a nine-year history of a relapsing cutaneous eruption. Lesions began in the hands and slowly progressed to affect the legs. Clinical exam revealed numerous 5– 15 mm vesiculo-bullous lesions (Fig. 1), intermingled with 3–20 mm red to brownish firm papules and plaques on the extensor surface of the legs (Fig. 2). New skin lesions were associated with pruritus, while older lesions were asymptomatic. Lesions involuted spontaneously, leaving yellowish atrophic scars. General physical exam was otherwise normal. No systemic symptoms were noted except mild asthenia. Laboratory tests were all normal. Screening for internal malignancies with computed tomographic scan showed no abnormalities. Differential diagnosis included bullous disease and leukocytoclastic vasculitis. Two skin biopsies were performed, one from a vesicule (new lesion) from the right leg and one from an indurated papule (old lesion) from the left leg. The first biopsy revealed some intraepidermal spaces and edematous papillae (Fig. 3a) associated with a dense perivascular superficial and deep dermal infiltrate composed mostly of neutrophils and eosinophils, extensive leukocytoclastia, and focal fibrin necrosis of small vessels walls (Fig. 3b). The biopsy of the firm papule showed leukocytoclastia, marked dermal fibrosis, increased vascularity, and a scant infiltrate of neutrophils (Fig. 3c,d). On the basis of clinical appearance and histology, a diagnosis of erythema elevatum diutinum (EED) was made. The patient was treated with dapsone, 100 mg/d and hydroxychloroquine 200 mg/d, with great clinical improvement over the following 4–6 weeks. 1126 Figure 1 Numerous 5–15 mm vesiculobullous lesions in both legs mimicking a vesiculobullous disease

Dos casos de alergia de contacto a octocrileno en niños

varicella-zoster infection despite absence of viral genome. J Cut Pathol. 1996;23:576--81. 4. Freemer CS, Farmer ER, Corio RL, Altomonte VL, Wagner JE, Vogelsang GB, et al. Lichenoid chronic graft-vs-host disease occurring in a dermatomal distribution. Arch Dermatol. 1994;130:70--2. 5. Lee SW, Kim YC, Lee ES, Kang HY. Linear lichenoid graft versus host disease: an unusual configuration following Blaschko’s lines. J Dermatol. 2006;33:583--4. 6. Oiso N, Tatsumi Y, Rai S, Matsumura I, Kawada A. Superimposed linear graft-versus-host disease and secondary cutaneous involvement of anaplastic large cell lymphoma. Eur J Dermatol. 2011;21:636--8. 7. Kikuchi A, Okamoto S, Takahashi S, Asano S, Nishikawa T. Linear chronic cutaneous graft-versus-host disease. J Am Acad Dermatol. 1997;37:1004--6. 8. Cohen PR, Hymes SR. Linear and dermatomal cutaneous graftversus-host disease. South Med J. 1994;87:758--61. 9. Mun JH, Park HJ, Kim HS, Kim SH, Ko HC, Kim BS, et al. Lichen striatus occurring after allogenic peripheral blood stem cell transplantation in an adult with aplastic anemia. Ann Dermatol. 2012;24:87--9. 10. Sanli H, Anadolu R, Arat M, Ekmekci P, Birot A, Erdem C, et al. Dermatomal lichenoid graft-versus host disease within herpes zoster scars. Int J Dermatol. 2003;42:562--4. 11. Martires KJ, Baird K, Citrin DE, Hakim FT, Pavletic SZ, Cowen EW. Localization of sclerotic-type chronic graft-vshost disease to sites of skin injury: potential insight into the mechanism of isomorphic and isotopic responses. Arch Dermatol. 2011;147:1081--6.

Nevus lentiginoso atípico. Estudio clínico-patológico de 14 casos

BACKGROUND Atypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. MATERIAL AND METHODS We reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. RESULTS Four (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. CONCLUSIONS Atypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer.

CARTA AL EDITORSíndrome del cabello anágeno suelto. Utilidad diagnóstica del tricogramaLoose anagen hair syndrome. Diagnostic use of the trichogram

dad clı́nica, fenotipos infantil tardı́a clásica y juvenil. Catepsina D (CTS): CLN10 forma congénita. Posteriormente se harı́a el estudio genético. La forma CLN3 (juvenil o enfermedad de Batten) tiene presentación tı́pica con ceguera progresiva, y como screening, podrı́an buscarse linfocitos vacuolados y luego realizar genética CLN3, más común 1kb deleción. Si las determinaciones anteriores fueran negativas y persiste alto grado de sospecha de CLN, serı́a obligatoria la ME y si fuera positiva hacer genética orientada según ME de las formas menos habituales: CLN5 (forma finlandesa), CLN6, CLN7 (forma turca), CLN8 (forma turca y epilepsia del norte con retardo mental) y CLN4 gen todavı́a no identificado (forma del adulto o enfermedad de Kufs). Queremos destacar las ventajas que a nuestro juicio aporta la biopsia de piel, que permite: el estudio de la actividad enzimática (PTT1, TPP1, CTSD) y ME en las CLN; el estudio de otras enfermedades lisosomales y mitocondriales; y almacenar fibroblastos, tejido vivo, para futuros estudios en caso de no llegar a un diagnóstico.

Síndrome del cabello anágeno suelto. Utilidad diagnóstica del tricograma

dad clı́nica, fenotipos infantil tardı́a clásica y juvenil. Catepsina D (CTS): CLN10 forma congénita. Posteriormente se harı́a el estudio genético. La forma CLN3 (juvenil o enfermedad de Batten) tiene presentación tı́pica con ceguera progresiva, y como screening, podrı́an buscarse linfocitos vacuolados y luego realizar genética CLN3, más común 1kb deleción. Si las determinaciones anteriores fueran negativas y persiste alto grado de sospecha de CLN, serı́a obligatoria la ME y si fuera positiva hacer genética orientada según ME de las formas menos habituales: CLN5 (forma finlandesa), CLN6, CLN7 (forma turca), CLN8 (forma turca y epilepsia del norte con retardo mental) y CLN4 gen todavı́a no identificado (forma del adulto o enfermedad de Kufs). Queremos destacar las ventajas que a nuestro juicio aporta la biopsia de piel, que permite: el estudio de la actividad enzimática (PTT1, TPP1, CTSD) y ME en las CLN; el estudio de otras enfermedades lisosomales y mitocondriales; y almacenar fibroblastos, tejido vivo, para futuros estudios en caso de no llegar a un diagnóstico.

Coalescence of multiple piloleiomyomas in an indurated hemifacial plaque

1 Willemze R, Jaffe ES, Burg G, et al. WHO–EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768–3785. 2 Choi YL, Park JH, Namkung JH, et al. Extranodal NK/T-cell lymphoma with cutaneous involvement: ‘‘nasal’’ vs. ‘‘nasal-type’’ subgroups – a retrospective study of 18 patients. Br J Dermatol 2009; 160: 333–337. 3 Lee MW. Characteristics of cutaneous lymphomas in Korea. Clin Exp Dermatol 2003; 28: 639–646. 4 Hasserjian RP, Harris NL. NK-cell lymphomas and leukemias: a spectrum of tumors with variable manifestations and immunophenotype. Am J Clin Pathol 2007; 127: 860–868. 5 Siu LL, Chan JK, Kwong YL. Natural killer cell malignancies: clinicopathologic and molecular features. Histol Histopathol 2002; 17: 539–554. 6 Wu X, Li P, Zhao J, et al. A clinical study of 115 patients with extranodal natural killer/T cell lymphoma, nasal type. Clin Oncol (R Coll Radiol) 2008; 20: 619– 625. 7 Iwatsuki K, Xu Z, Ohtsuka M, Kaneko F. Cutaneous lymphoproliferative disorders associated with Epstein–Barr virus infection: a clinical overview. J Dermatol Sci 2000; 22: 181–195.

Sinusitis destructiva masiva secundaria a consumo inhalado de cocaína

Se trata de unamujer de 24 anos con historia de abuso cronico de cocaina inhalada desde los 14 anos de edad e infecciones nasosinusales recidivantes asociadas al consumo de esta droga. En los ultimos 4 anos, la sinusitismaxilar se habia cronificado, generando una destruccion tisular rapidamente progresiva, sin cese del consumo de cocaina, que presento como resultado una destruccion total de las estructuras blandas y oseas centrofaciales, incluyendo tabique nasal y nariz, con erosion parcial del paladar duro (fig. 1). En el diagnostico diferencial de los procesos destructivos nasosinusales que afectan a la linea media hay que descartar: linfoma nasal, granuloma letal de la linea media, sifilis terciaria y vasculitis sistemicas tipo granulomatosis de Wegener. En este caso se confirmo que la sinusitis destructiva era secundaria a la inhalacion cronica de cocaina. El consumo de cocaina en polvo via oral o nasal se asocia a necrosis osteocartilaginosa, ulceraciones en la pared faringea, perforacion del paladar duro, perforacion del septo nasal, enfermedad periodontal e, incluso, necrosis a distancia (helix, pulpejos de los dedos). El efecto vasoconstrictor de la cocaina causa isquemia local en los huesos centrofaciales y tejidosblandos. Recientemente sehadescritoelpapeldel levamisol, unagenteantihelminticoe inmunomodulador, usadocon frecuencia para cortar esta droga y que contamina la cocaina en polvo: el levamisol actua como un toxico vascular y puede generar una vasculitis de pequeno vaso con trombosis y necrosis tisular local y a distancia. En nuestra paciente, las infecciones nasosinusales recidivantes contribuyeron al proceso destructivo final, y fallecio dos meses despues de tomar esta imagen por progresion de la enfermedad que llego a producir una afectacion intracraneal letal.Se trata de una mujer de 24 años con historia de abuso crónico de cocaı́na inhalada desde los 14 años de edad e infecciones nasosinusales recidivantes asociadas al consumo de esta droga. En los últimos 4 años, la sinusitis maxilar se habı́a cronificado, generando una destrucción tisular rápidamente progresiva, sin cese del consumo de cocaı́na, que presentó como resultado una destrucción total de las estructuras blandas y óseas centrofaciales, incluyendo tabique nasal y nariz, con erosión parcial del paladar duro (fig. 1). En el diagnóstico diferencial de los procesos destructivos nasosinusales que afectan a la lı́nea media hay que descartar: linfoma nasal, granuloma letal de la lı́nea media, sı́filis terciaria y vasculitis sistémicas tipo granulomatosis de Wegener. En este caso se confirmó que la sinusitis destructiva era secundaria a la inhalación crónica de cocaı́na. El consumo de cocaı́na en polvo vı́a oral o nasal se asocia a necrosis osteocartilaginosa, ulceraciones en la pared farı́ngea, perforación del paladar duro, perforación del septo nasal, enfermedad periodontal e, incluso, necrosis a distancia (hélix, pulpejos de los dedos). El efecto vasoconstrictor de la cocaı́na causa isquemia local en los huesos centrofaciales y tejidos blandos. Recientemente se ha descrito el papel del levamisol, un agente antihelmı́ntico e inmunomodulador, usado con frecuencia para cortar esta droga y que contamina la cocaı́na en polvo: el levamisol actúa como un tóxico vascular y puede generar una vasculitis de pequeño vaso con trombosis y necrosis tisular local y a distancia. En nuestra paciente, las infecciones nasosinusales recidivantes contribuyeron al proceso destructivo final, y falleció dos meses después de tomar esta imagen por progresión de la enfermedad que llegó a producir una afectación intracraneal letal.