Is it just a psoriasiform dermatitis?

Bazex syndrome (BS) is a rare paraneoplastic syndrome most frequently associated with squamous cell carcinomas of the upper aerodigestive tractand other tumours. Characteristically, cutaneous lesions precede the diagnosis of malignancy. We report a 72-year-old patient with 1-year history of acral dermatitis. The diagnosis of BS was based on the presence of psoriasiform acral dermatitis and the evidence of two simultaneous tumors (prostate adenocarcinoma and undifferentiated carcinoma ofthe submandibular gland). It is important to have this syndrome in mind since cutaneous features usually precede an underlying neoplasm.

Kaposi sarcoma following postmastectomy lymphedema.

Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.

Elastolytic Actinic Giant Cell Granuloma.

Actinic granuloma and annular elastolytic giant cell granuloma are different terms used to define skin lesions characterized by elastolysis, elastophagocytosis, and multinucleated giant cell infiltrate. The clinical appearance varies from papules to annular plaques. Although elastolytic actinic giant cell granuloma shares some clinical features with granuloma annulare, they can be differentiated by histopathologic findings. The disease is initiated by an immune response triggered by different factors that alter the elastic tissue. The course tends to be chronic, with variable response to treatments, although spontaneous remission may occur. Diabetes mellitus is the systemic disease most frequently associated with this condition.

Tolerability and effectiveness of liquid nitrogen spray cryotherapy with very short freeze times in the treatment of xanthelasma palpebrarum.

Xanthelasma are cholesterol-filled, soft, yellow plaques that usually appear on the medial aspects of the eyelids bilaterally. They are always benign lesions so therapy is usually undertaken only for cosmetic reasons. Surgical excision, chemical peeling with tricholoroacetic acid, and laser ablation are commonly used treatments. Liquid nitrogen cryotherapy is a potentially effective but rarely used treatment due to the risk of intense eyelid swelling. We report on our experience with four of our patients, and propose an explanation for the effectiveness of gentle liquid nitrogen spray cryotherapy in xanthelasma. We consider that gentle liquid nitrogen cryotherapy should be used in the treatment of xanthelasma due to the ease of application and low risk of adverse effects.

Annular elastolytic giant cell granuloma associated to late-onset X-linked dominant protoporphyria.

X-linked dominant protoporphyria (XLDPP) is a genetic disorder that affects the synthesis of the heme group due to an increase in delta-aminolaevulinate synthase 2 (ALAS2) enzyme activity. Moreover, annular elastolytic giant-cell granuloma (AEGCG) is a rare reactive granulomatous dermatosis, usually associated with actinic damage. An 86-year-old man presented with edematous-erythematous lesions in photoexposed areas of the face and on the dorsum of both hands. Protoporphyrin levels in serum and feces were significantly elevated and a heterozygous frameshift mutation in the exon 11 of the ALAS2 gene: c.1706-1709del (p.Glu569GlyfsX24) was identified. Concomitantly, we observed an annular plaque with raised borders on the back of his right hand, clinically and histologically compatible with a diagnosis of AEGCG. Skin lesions disappeared only upon use of a physical sunscreen. We report two rare photodermatoses in an elderly patient and discuss the significance of dermal elastic fiber damage induced by the XLDPP as a main triggering factor of AEGCG.

Elastolytic giant cell granuloma: clinic-pathologic review of twenty cases.

BACKGROUND: O'Brien described four histopathological patterns of actinic granuloma (AG). Since then, only single cases and a few series have been reported in the literature, most corresponding to cases of the giant cell type. METHODS: We reviewed all the cases diagnosed as AG or elastolytic giant cell granuloma (EGCG) in our department from 1988 until 2010. The biopsies were classified into the four patterns previously described. RESULTS: Giant cell pattern was found to be the most frequent (70% of the cases). In four cases, the biopsies showed more than one histopathologic pattern. All the lesions were located on sun-exposed areas or were related to chronic heat exposure. Diabetes mellitus was associated in 40 % of the cases. CONCLUSIONS: The giant cell pattern of EGCG is the most frequent. Some cases may share histopathologic features of more than one variant and thus, we consider they may be categorized as mixed patterns. Diabetes mellitus is the most common associated disease and should always be ruled out.

Eritema flagelado por setas Shiitake / Flagellate erythema induced by Shiitake mushroom

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Validation of the Spanish Acne Severity Scale (Escala de Gravedad del Acné Española--EGAE).

BACKGROUND: Several acne grading systems have been described, but consensus is lacking on which shows superiority. A standardized system would facilitate therapeutic decisions and the analysis of clinical trial data. OBJECTIVE: To assess the feasibility, reliability, validity and sensitivity to change of the Spanish Acne Severity Scale (EGAE). MATERIALS & METHODS: A Spanish, multicentre, prospective, observational study was performed in patients with facial, back or chest acne assessed using EGAE, Leeds Revised Acne Grading system (LRAG) and lesion count. Clinicians answered 4 questions regarding EGAE use and time employed. Patients were evaluated at baseline and after 5±1 weeks. Four additional blinded observers, all dermatologists, evaluated patients' pictures using EGAE and LRAG. RESULTS: In total, 349 acne locations were assessed in 328 patients. Of the dermatologists, 95.6% (CI: 92.9-97.5%) reported that EGAE was easy to use, and 75% used it in <3 minutes. Interobserver reliability of the EGAE scale was shown by a Kendall's W of 0.773 (p<0.001). EGAE and LRAG scales showed a high correlation (Spearman's correlation>0.85; p<0.001). EGAE mean score in treatment-compliant patients was significantly lower at follow-up than at baseline (2.14 vs. 1.57, p<0.001, Cohen's d=0.35).The pre-post-treatment difference in EGAE mean score in non-compliant patients was not significant (1.44 vs. 1.32, p<0.102) and Cohen's d was lower (0.19) than in compliant patients. CONCLUSION: The use of EGAE to evaluate acne grade in daily clinical dermatological practice in Spanish centres has shown feasibility, high interobserver reliability, concurrent validity and sensitivity to detect treatment effects.

Vasculitis acral y pancitopenia como síndrome paraneoplásico asociado a carcinoma epidermoide de pulmón / Acral vasculitis and pancytopenia as a paraneoplastic syndrome associated with epidermoid lung cancer

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