Cutaneous Polymer-Coating Embolism After Endovascular Procedures: Report of Two Cases and a Literature Review.

ABSTRACT: Different hydrophilic and hydrophobic polymers are used as lubricious coatings to reduce vascular traumas in minimally invasive percutaneous procedures. Although they are usually very safe, there is still a risk of serious complications in patients undergoing such procedures, mostly derived from the devices' coating detachment and systemic embolization. The lungs are the most common organ involved, followed by the central nervous system. Yet, cutaneous embolization is unusual, and only 19 cases are available in the literature. Most commonly, they present as asymptomatic retiform purpura on the lower legs, which tends to involve spontaneously. Correct clinical diagnosis is not suspected in most cases, being cholesterol emboly or vasculitis the preferred options. Time interval since surgical procedure and appearance of lesions vary widely but they generally start in the first few days. Histopathological identification of the embolus as bluish, amorphous intraluminal material in dermal vessels is diagnostic, but vasculitic signs are not present. We report 2 cases of skin lesions as the main manifestation of polymer embolization after endovascular surgical procedures. In both cases, biopsy allowed identification of embolized foreign material and lesions resolved without specific treatment.

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.

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.

Patch testing with formaldehyde and formaldehyde-releasers: multicentre study in Spain (2005-2009).

BACKGROUND: Formaldehyde and formaldehyde-releasers are common causes of allergic contact dermatitis. OBJECTIVES: To determine the frequency of sensitization to formaldehyde and seven formaldehyde-releasers. To establish and characterize groups of patients according to the results of patch testing. MATERIALS AND METHODS: We performed a 5-year retrospective study, in six Spanish hospitals, of patients with positive patch test reactions to formaldehyde or any of seven formaldehyde-releasers. RESULTS: The most frequent allergens were formaldehyde (1.72%), imidazolidinyl urea (1.05%), quaternium-15 (0.88%), and diazolidinyl urea (0.79%). Patients with sensitization to only formaldehyde had a higher frequency of occupational dermatitis (25%) than patients with sensitization to only formaldehyde-releasers (9.5%). The most common sites of dermatitis were the hands (31.7%) in patients with sensitization to only formaldehyde and the face and legs (31.3% and 24.6%) in patients with sensitization to only formaldehyde-releasers. We found a subgroup of 25 patients who were sensitized to both imidazolidinyl urea and diazolidinyl urea, and only 6 of these (24%) were also sensitized to formaldehyde. CONCLUSIONS: The inclusion of imidazolidinyl urea and diazolidinyl urea in the baseline series of the Spanish Contact Dermatitis and Skin Allergy Research Group (GEIDAC) should enable better classification of patients allergic to formaldehyde, and could aid in their management.

Clinicopathological and immnuohistochemical findings in a series of folliculosebaceous cystic hamartoma.

BACKGROUND: Folliculo-sebaceous cystic hamartoma (FSCH) is an uncommon skin condition presenting as a slow-growing papulo-nodular lesion, in or around the nose. Most cases are not clinically suspected and only histopathological examination allows the diagnosis. Pathological features include a dermal-located infundibulo-cystic structure with sebaceous glands radiating around, a stromal component encircling the epithelial structures, with clefts between the lesional epithelial and stromal parts, as well as between this and the adjacent dermis. RESULTS: We report eight patients with the diagnosis of FSCH (5 females and 3 males), with ages ranging from 35 to 77 years. Most cases (5 out of 8) were located in or around the nose and sizes were comprised between 0.6 and 1.2 cm. Lesions had grown for long periods of time, up to ten years in one case. Immunohistochemistry showed staining for p63 in the epithelial component of all lesions, while CD10 was only present in some sebocytes. CD34 and Factor XIIIa positive cells were present in the lesional stroma. Staining for androgen and alpha-estrogen receptors was also usually noticed. CONCLUSIONS: FCSH is a hamartomatous skin lesion, clinically indistinct but with well-defined histopathological features. Immunohistochemistry shows a profile very close to normal sebaceous glands.

Contact allergic dermatitis from melamine formaldehyde resins in a patient with a negative patch-test reaction to formaldehyde.

Melamine paper is a basic material used in the furniture industry for home and office interiors. Contact allergic dermatitis from melamine formaldehyde resins (MFRs) should be considered in patients who work on melamine paper impregnation lines. We report a case of a 28-year-old female plywood worker who developed eczema on the dorsal side of her hands and wrists after 2 years of working on the melamine paper impregnation line. She had a relevant positive patch-test reaction to MFR, with a negative reaction to formaldehyde. Contact dermatitis due to MFR is not common, and it is usually related to products that are not fully cured or to close contact with intermediate products on the assembly line. Formaldehyde release from MFR can explain most of the positive responses. To our knowledge, this is the first report of MFR contact allergic dermatitis in a worker on a melamine paper impregnation line.

Lymphomatoid contact dermatitis induced by gold earrings.

Metallic gold has long been regarded as a relatively safe material when in contact with the skin and mucosal membranes, with only sporadic reports of allergic contact dermatitis or stomatitis. This is probably due to its stability and low tendency to ionization. Lymphomatoid reactions have been described in cutaneous responses to several products. We report a patient who developed bilateral nodules on the earlobes at the sites of piercing earrings. Histologically, these lesions presented a lymphomatoid appearance, with a T-cell diffuse infiltrate. The patch test demonstrated a strong reaction to 1% gold sodium thiosulfate.

[Acrokeratosis paraneoplastica with bullous lesions associated with esophageal squamous cell carcinoma]. / Acroqueratosis paraneoplásica con lesiones ampollosas asociada a carcinoma epidermoide esofágico.

Acrokeratosis paraneoplastica (Bazex syndrome) is characterized by an acral eruption with a psoriasiform appearance, which usually presents simultaneously with an underlying neoplasm. We describe the case of a 64-year-old male who presented with a two-month history of pruritic, flaky, erythematous lesions on the palms, backs of the fingers and toes and pinnae, accompanied by bullous lesions. The patient also reported progressive dysphagia in the last six months, and general wasting with a loss of 15 kg. The digestive endoscopy revealed a squamous cell carcinoma in the proximal esophagus. The histopathological study of the bullous lesions showed the presence of a subepidermal bulla, and direct immunofluorescence revealed granular deposits of IgG, IgA and C3 in the basal membrane of the healthy perilesional skin. These clinical/pathological findings support the existence of an immunological pathogenic mechanism related to this entity.

[Porphyria cutanea tarda in a dialyzed female patient]. / Porfiria cutánea tarda en una paciente hemodializada.

Dermatological disorders are a frequent finding in patients with renal insufficiency. Porphyria cutanea tarda, pseudoporphyria, phototoxicity or concurrent bullous diseases may cause the appearance of lesions with liquid content in patients in dialysis. We describe the case of a female patient in dialysis who developed bullous lesions. The clinical and laboratory findings were initially compatible with the suspicion of pseudoporphyria, but the finding of some frankly elevated levels of porphyrins in serum, urine and feces confirmed the diagnosis of porphyria cutanea tarda. Porphyria cutanea tarda and pseudoporphyria present with common clinical manifestations. However, the levels of porphyrins in plasma, urine and feces in pseudoporphyria are normal or slightly elevated.

Multinucleate cell angiohistiocytoma. Report of five cases.

BACKGROUND: Multinucleate cell angiohistiocytoma is an infrequent and most likely non-neoplastic disorder usually seen in acral regions in elderly women. It presents clinically as asymptomatic red-to-brown tumors, with a tendency to confluence. It must be distinguished from other diverse cutaneous lesions, notably dermatofibroma, Kaposi sarcoma, and angiofibroma. METHODS: We report the clinical, histopathological, and immunohistochemical findings of five patients, all women aged between 51 and 78 years. All except the first presented lesions on both of the lower limbs. None of the patients developed spontaneous resolution of the lesions and one was successfully treated by cryosurgery. COMMENTS: Multinucleate cells are characteristic, but neither exclusive nor pathognomonic, of multinucleate cell angiohistiocytoma, since they can also appear in other inflammatory, neoplastic, or reactive processes. The presence of these cells and vascular proliferation in dermis media are the principal histopathological findings in this infrequent entity. In immunohistochemical studies, the multinucleate cells are often positive for vimentin and factor XIIIa.

Annular follicular lichen planus.

Annular lesions can be found in cases of lichen planus (LP). We present a case with follicular (histopathologic) and annular (clinical) features. To our knowledge, this is the first case of annular follicular LP reported in the literature.

Ulerythema ophryogenes in Cornelia de Lange syndrome.

A 17-year-old woman with Cornelia de Lange syndrome had asymptomatic skin lesions since the age of 4 years. These were multiple, follicular, horny papules, present on both cheeks, and surrounded by erythematous skin. Similar lesions were present on the external aspect of the arms, but amidst skin of normal coloration. Keratosis pilaris atrophicans faciei (ulerythema ophryogenes) has been described as a cutaneous marker for several congenital syndromes. To our knowledge, its possible association with Cornelia de Lange syndrome has never been reported.