Successful treatment of generalized elastolytic giant cell granuloma with psoralen-ultraviolet A.

Elastolytic giant cell granuloma (EGCG) is an infrequent granulomatous skin disorder with variable response to different therapeutic regimens. Information on the benefit of phototherapy is very scarce as this therapy has seldom been tried in the affected patients. We present the results achieved in two female patients after undergoing psoralen-ultraviolet A (PUVA). Two 54-year-old otherwise healthy female patients received a course of PUVA after trying other alternatives. Complete clearance was achieved in the two patients with excellent tolerance and no adverse effects. We consider PUVA is a well-tolerated, safe, and effective treatment for patients with EGCG.

Multiple congenital familial smooth muscle hamartoma in two siblings.

Smooth muscle hamartoma (SMH) is a cutaneous malformation mainly composed of a disorganized proliferation of normal muscle fibers that arise from arrector pili. It usually presents as a single congenital lesion that frequently involves the back and the lower limbs. Unusual clinical presentations, such as atypical localizations, multiple disseminated lesions, and generalized forms have been rarely described. In 2001, Gualandri et al. reported the presence of multiple SMH in three members of the same family, namely two brothers and their mother. This is, as far as we know, the only familial case reported in the English literature. We herein describe a similar case affecting two siblings who presented with identical congenital lesions in the same location.

Coexistence of two types of clinical lesions in childhood-onset mastocytosis.

The vast majority of mastocytosis appear in childhood, urticaria pigmentosa (UP) and mastocytomas being the most common types. Terms such as "xanthelasmoid mastocytosis", "pseudoxanthomatous mastocytosis" or "nodular mastocytosis" have been introduced in the literature to describe the presence of yellowish papular or nodular lesions. We describe two children with cutaneous mastocytosis showing yellowish lesions in combination with other skin lesions. A 10-year-old girl presented with asymptomatic lesions in her vulva at birth, and developed brownish macules on her trunk years after. An eight-year-old boy presented with multiple yellowish papular lesions on his trunk, neck and limbs coexisting with a few clinically anetodermic lesions. No systemic involvement was found and the skin biopsy confirmed a cutaneous mastocytosis in both cases. The two patients are currently asymptomatic and are being periodically followed up. Mastocytoses may show a variety of clinical lesions, sometimes leading to misdiagnosis. Although there are previous reports, involvement of the mucosae and secondary anetoderma are not common findings in cutaneous mastocytoses. We consider that cutaneous manifestations of mastocytoses compose a clinical spectrum, thus explaining the coexistence of different clinical lesions and the development of uncommon presentations.

General features and treatment of notalgia paresthetica.

Notalgia paresthetica is a neurocutaneous disorder that most commonly manifests as pruritus and a brownish macula in the patient's upper back. Pain, burning, and paresthesias to a variable degree have also been reported. Although the physiopathology of notalgia paresthetica is still obscure, it is currently considered a sensory neuropathy caused by damage to the spinal nerves. To date, no definitive treatment has been described for this disorder. Alternatives, including physical therapies and topical, systemic, and intralesional drugs, have been tested with diverse results. A review of the current knowledge on notalgia paresthetica and its treatment is provided.

Contact allergy from vitamins in cosmetic products.

We report three new cases of allergic contact dermatitis due to vitamins in cosmetic creams. The first patient was diagnosed with worsening rosacea but had allergic contact dermatitis from alpha-tocopherol in a moisturizing cream. The second and third cases presented as acute eyelid dermatitis due to vitamin K in eyelid lifter creams. Repeated open application testing and patch tests with the actual products and individual components of the creams were useful in establishing the diagnosis.

Friction induced skin tags.

Skin tags are common benign neoplasm located predominantly in intertriginous skin. Generally of cosmetic concern, they can be easily treated with cryotherapy, electrodessication or snip-excision. Despite their high incidence data about their etiopathogenesis are scarce in the medical literature. We describe a patient who developed multiple skin tags arranged in a linear fashion suggesting an etiopathogenic role for friction.

Ulcerous lesions disclosing cutaneous infection with Fusarium solani.

Fusarium spp. are emergent opportunistic moulds capable of producing life-threatening diseases, usually in immunocompromised hosts. When they infect immunocompetent patients, they usually cause localized diseases. We describe here a case of cutaneous fusariosis in a patient with a medical history of diabetes mellitus and non-Hodgkin's lymphoma who presented with two painful lesions on her left leg. The diagnosis was made on the basis of histopathological findings and skin biopsy culture. The lesions resolved after treatment with oral itraconazole. Cutaneous fusariosis manifests clinically as lesions that can be grouped into a few clinical patterns, and should always be considered in the differential diagnosis of necrotic cutaneous lesions.

Allergic contact dermatitis from EMLA cream in a hemodialyzed patient.

The eutectic mixture EMLA cream has many medical uses, including pain relief for hemodialyzed patients prior to cannulation. Cutaneous side effects associated with its application have rarely been reported. A patient on hemodialysis developed an itchy eruption over his brachial arteriovenous fistula, where he was applying EMLA cream three times per week, prior to puncture for hemodialysis. Patch testing elicited a positive reaction to several allergens, including EMLA cream "as is" and prilocaine. The lesions resolved after topical treatment with corticosteroids.

[Generalized pustular psoriasis and cytolytic hepatitis associated with neutrophilic cholangitis]. / Psoriasis pustulosa generalizada y hepatitis citolítica asociada a colangitis neutrofílica.

Generalized pustular psoriasis (GPP) of the von Zumbusch type may be associated with liver disorders, usually resulting in cholestatic hepatitis with neutrophilic cholangitis. We describe the case of a woman who experienced a first episode of GPP, during which she presented with an increase in hepatic cytolysis parameters. An abdominal ultrasound and serological studies did not show any significant anomalies. A liver biopsy showed the presence of a neutrophilic infiltrate around the bile ducts of the portal space. In short, this fact suggests that neutrophilic cholangitis in the context of this disease may initially show patterns of analytical alterations other than the classically described cholestatic pattern.

[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.

Psoriasis pustulosa generalizada y hepatitis citolítica asociada a colangitis neutrofílica / Generalized pustular psoriasis and cytolytic hepatitis associated with neutrophilic cholangitis

La psoriasis pustulosa generalizada (PPG) de von Zumbusch puede asociarse a alteraciones hepáticas, resultando habitualmente en una hepatitis colestásica con colangitis neutrofílica. Describimos el caso de una mujer con un primer episodio de de PPG que presentó durante el mismo una elevación de los parámetros hepáticos de citolisis. La ecografía abdominal y los estudios serológicos no mostraron anomalías significativas. La biopsia hepática mostró la presencia de un infiltrado neutrofílico alrededor de los conductos biliares del espacio portal. En definitiva, este hecho sugiere que la colangitis neutrofílica en el contexto de esta enfermedad puede mostrar inicialmente otros patrones de alteraciones analíticas distintos del patrón colestásico clásicamente descrito

Generalized pustular psoriasis (GPP) of the von Zumbusch type may be associated with liver disorders, usually resulting in cholestatic hepatitis with neutrophilic cholangitis. We describe the case of a woman who experienced a first episode of GPP, during which she presented with an increase in hepatic cytolysis parameters. An abdominal ultrasound and serological studies did not show any significant anomalies. A liver biopsy showed the presence of a neutrophilic infiltrate around the bile ducts of the portal space. In short, this fact suggests that neutrophilic cholangitis in the context of this disease may initially show patterns of analytical alterations other than the classically described cholestatic pattern

Onicomicosis y balanitis en un niño de 5 años / Onychomycosis and balanitis in a 5-year-old boy

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