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.

Granuloma annulare photoinduced by paroxetine.

Granuloma annulare (GA) is a benign granulomatous skin disease with several clinical manifestations and characteristic histological findings. GA located in photoexposed areas is a rare finding and its association to a drug-induced systemic photosensitivity is even less common. To the best of our knowledge, only one case of systemic drug photosensitivity manifesting as a GA has been reported. We describe a patient with systemic photosensitivity to paroxetine with clinical and histological manifestations of GA, which was confirmed by the photobiological study. The phototest revealed a reduction of the minimal erythematous dose for UVB while taking the paroxetine and its normalization after its withdrawal, which was accompanied by the clinical resolution of the skin eruption. The manifestation of systemic drug photosensitivity as a GA like in our case is exceptional.

Prevalence of milia and palatal and gingival cysts in Spanish newborns.

We performed a descriptive survey of 1,000 consecutive newborns cared for in the first 3 days of life in the health area of Ferrol (in northwest Spain) to assess the prevalence and most-frequent locations of oral cysts and milia and to study the influence of different maternal and neonatal parameters in the development of these lesions. Prevalence of palatal, gingival, and cutaneous cysts was 53.7%, 13.4%, and 16.6%, respectively. Milia were predominantly located on the cheeks, chin, and forehead. We found a frequent association between palatal and gingival cysts but not between oral cysts and milia. Neonatal factors such as sex, weight at birth, and gestational age can significantly influence the development of palatal and gingival cysts.

Letter: Cutaneous mastocytosis with systemic involvement mimicking clinical and dermatoscopically multiple melanocytic nevi.

Mastocytosis can sometimes resemble other skin conditions, especially pigmented ones, not only clinically but also dermatoscopically. We report the case of a woman with the diagnosis of cutaneous mastocytosis mimicking multiple melanocytic nevi. Melanocytic stimulation can be induced by high levels of stem cell factor. The progressive increase in the number of pigmented lesions in a patient should lead us to perform a biopsy to search for mastocytosis.

Birt-Hogg-Dubé syndrome in a patient with localized fibrofolliculomas and a novel mutation in the FLCN gene.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) is characterized by skin fibrofolliculomas (FF), multiple lung cysts, spontaneous pneumothorax, and renal cancer. Cutaneous lesions are usually distributed over the face, neck, and upper trunk. The presence of FF confined to a circumscribed region of the skin has rarely been reported. CASE REPORT: A 64-year-old woman presented with a 20-year history of asymptomatic skin lesions located on the neck. Multiple skin-colored papules with a clinical plaque-like appearance were confined to the right side of the neck. Histopathological findings were typical for FF, and BHDS was suspected. The novel heterozygous mutation p.Val126SerfsX4 was identified in exon 5 of the FLCN gene. Colonoscopy, abdominal ultrasound, and abdominal thoracic scan revealed no associated pathologies, except for benign renal and hepatic cysts. DISCUSSION: To date, only two cases of localized FF in BHDS have been reported. Mutation analysis was not performed, but the authors considered the lesions to represent a localized variant of BHDS and speculated that this unusual form of the disease may be associated with a lack of visceral involvement as no signs of systemic disease were detected. CONCLUSIONS: We identified the novel germline mutation p.Vall26SerfsX4 as responsible for this aspect of the patient's phenotype, which suggests that alterations in the FLCN gene are also responsible for localized forms of BHDS. Moreover, the localized distribution of skin lesions may be related to a less severe form of the disease.

Disseminated eruptive clear cell acanthoma with spontaneous regression: further evidence of an inflammatory origin?

Clear cell acanthoma (CCA) is a benign epidermal lesion with distinctive clinicopathological features. Multiple disseminated eruptive CCA is an infrequent clinical variant that has been rarely reported. It is characterized by the presence of more than 30 lesions from 1 to 10 mm in diameter that appear progressively over the years. We report the case of a 65-year-old woman with multiple disseminated eruptive CCA affecting her lower extremities. In contrast to previous reports, most of the lesions appeared in a short period of time (less than a month) and, what is more interesting is that some of them have regressed spontaneously leaving residual hyperpigmentation. At present, the histogenesis and etiology of CCA remain unknown. Accumulating data suggest a reactive origin associated with a variety of different inflammatory conditions. The case presented in this report further substantiates that CCA is indeed a reactive epidermal reaction pattern with an inflammatory etiology.