Calciphylaxis treated with sodium thiosulfate: report of two cases.

Although traditionally observed in patients with end-stage renal disease and secondary hyperparathyroidism, calciphylaxis has been reported in patients with normal renal and parathyroid function. There is no evidence-based therapy available. The use of sodium thiosulfate (STS) has been increasingly described. Herein we describe two patients who responded well to this treatment.

Subungual squamous cell carcinoma: a case series.

Subungual tumors are rare in general. Of all tumors, subungual squamous cell carcinoma (SSCC) is the most frequent one. Protean clinical presentations and the lack of awareness of the disease are responsible for an incorrect or delayed diagnosis and subsequent delayed treatment. We have reported here four patients with SSCC who were previously wrongly diagnosed with a benign process and treated unsuccessfully for years. We would like to highlight the need of a biopsy in chronic or recurrent nail lesions that fail to respond to a previous conservative treatment in order to rule out SSCC.

Porokeratoma: a different entity or a variant of verrucous (hyperkeratotic) porokeratosis?

Porokeratoma (acanthoma with features of porokeratosis) is a recently described entity with a distinct pattern of cornoid lamellation and clinically different from typical porokeratosis. We present a case of multiple porokeratomas on the buttock of a 78-year-old man with paraplegia secondary to poliomyelitis and ankylosing spondylarthritis. We briefly review the characteristics and controversies of this new entity.

Bebé colodión: su evolución; presentación de tres casos / Collodion baby: its evolution; report of three cases

El bebé colodión se caracteriza por la presencia de una membrana que cubre la totalidad de la piel del recién nacido. La mayoría de los niños que presenta esta membrana desarrolla ictiosis, siendo la eritrodermia ictiosiforme congénita no ampollar la forma más frecuente, seguida de la ictiosis lamelar y la ictiosis vulgar. No existen características clínicas ni histológicas que sirvan de guía para predecir el diagnóstico final. Estos niños presentan complicaciones debido a las alteraciones de la función de barrera de la piel, como deshidratación hipernatrémica, hipotermia e infecciones cutáneas y sistémicas. Sin embargo el pronóstico de este cuadro ha mejorado en los últimos años debido a los mejores cuidados intensivos neonatales. Presentamos tres casos de bebé colodión evaluados en el servicio de Dermatología del Complejo Hospitalario de Pontevedra (España) y describimos sus características clínicas, tratamientos, complicaciones, pruebas complementarias realizadas y diagnóstico final. A pesar de que el bebé colodión es un fenotipo infrecuente, destacamos la importancia de conocer su tratamiento, así como los diferentes procesos a los que puede dar lugar...

Collodion baby consists on a membrane covering the whole body surface of a newborn. Most children born as collodion baby develop ichthyosis, being the most frequent the non-bullous congenital ichthyosiform erythroderma, followed by lamellar ichthyosis and ichthyosis vulgaris. There are no distinctive clinical or histological features that can be used to predict the final diagnosis. These children have complications due to the impaired barrier function, such as hypernatraemic dehydration, hypothermia, or cutaneous and systemic infections. However the prognosis has dramatically improved over time with the development of neonatal intensive care. We present three cases of collodion baby seen at the Dermatology Service of the Pontevedra Hospital Complex (Spain) and describe their clinical characteristics, treatments, complications, diagnostic procedures and final diagnoses. Despite collodion baby is an uncommon entity, we highlight the importance of knowing its treatment and the different processes that may arise from it...

Hypochondroplasia and Acanthosis nigricans: a new syndrome due to the p.Lys650Thr mutation in the fibroblast growth factor receptor 3 gene?

BACKGROUND: Hypochondroplasia (HCH) is a skeletal dysplasia inherited in an autosomal dominant manner due, in most cases, to mutations in the fibroblast growth factor receptor 3 (FGFR3). Acanthosis nigricans (AN) is a velvety and papillomatous pigmented hyperkeratosis of the skin, which has been recognized in some genetic disorders more severe than HCH involving the FGFR3 gene. OBJECTIVE AND DESIGN: After initial study of the proband, who had been consulted for short stature and who also presented AN, the study was extended to the patient's mother and to 12 additional family members. METHODS: Clinical, biochemical and radiological studies were performed on the family. In addition, exons 11 and 13 of FGFR3 were analyzed. RESULTS: The proband and ten relatives presented HCH plus AN and the analysis of FGFR3 showed the p.Lys650Thr mutation. The members with normal phenotypes were non-carriers of the mutation. CONCLUSION: This is the first report of a large pedigree with the clinical phenotype of HCH plus AN due to a FGFR3 mutation, p.Lys650Thr. This finding demonstrates the coexistence of both conditions due to the same mutation and it might represent a true complex, which should be further established by searching for AN in mild HCH patients or for HCH in patients with AN.

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.

Progressive nodular histiocytoma associated with thrombocytopenia with absent radii (TAR syndrome) and angiofibromas.

A 50-year-old woman was admitted due to a long-standing history of cutaneous lesions, which were gradually increasing in number and size, located on the trunk and extremities. Histological studies confirmed the initial clinical diagnosis of histiocytomas. Moreover, the patient had numerous smooth erythematous papules on her chin and around her nose, which were diagnosed histologically as angiofibromas. The patient had congenital phocomelia. Analytical and imaging studies revealed the presence of bilateral phocomelia due to absent radii and thrombocytopenia (TAR syndrome). Multiple histiocytomas in a normolipaemic patient bring up several differential diagnoses. Slow progressive evolution without spontaneous resolution and a scattered distribution on the trunk and extremities suggest the diagnosis of progressive nodular histiocytoma. To our knowledge progressive nodular histiocytoma has not been reported previously associated either with TAR syndrome or with angiofibromas. These entities are uncommon, thus their association may not be due to chance.

[Comparative study of the treatment of psoriasis plaques with PUVA baths and narrow-band UVA (311 nm)]. / Estudio comparativo del tratamiento de la psoriasis en placas con baño de PUVA y UVB de banda estrecha (311 nm).

INTRODUCTION: Studies carried out to date comparing treatment with PUVA baths and narrow-band UVB (NBUVB) in psoriasis show that better results are obtained with NBUVB. Certain features of the protocols may interfere with the results. MATERIAL AND METHODS: Prospective study in a psoriasis unit, with treatment assigned according to availability. Of 167 patients with psoriasis plaques, 32 received PUVA baths (photosensitizer 8-methoxypsoralen at a concentration of 2.6 mg/l) and 135 received NBUVB radiation. A clearing rate of over 70 % was considered a good response. The data were described and compared between the two groups, including multivariate analysis techniques, in order to statistically control the effects of gender, number of sessions necessary for success, minimum phototoxic dose and minimum erythema dose. RESULTS: A good response was obtained in 87.5 % of the cases with PUVA baths (95 % CI: 71.0-96.5) and in 87.4 % of the cases with NBUVB (95 % CI: 80.6-92.5). No significant differences were found in the success and abandonment percentages. Among the patients who responded to the treatment, no differences were found in the number of sessions or in the cumulative dose. Gender, cumulative dose or minimum phototoxic dose and minimum erythema dose values were not associated with the response to the treatments either. CONCLUSIONS: With psoriasis plaques, similar response percentages can be expected with both treatments, using the proposed protocols. The response is regardless of gender, cumulative dose and minimum phototoxic dose and minimum erythema dose values.

[De Sanctis-Cacchione syndrome]. / Síndrome de De Sanctis-Cacchione.

We present a male patient with photosensitivity since the earliest months of his life, and pigmented macules in exposed areas, some showing clinical atypia, which increased in number over time. Molecular biology studies detected an alteration in DNA repair ability, so xeroderma pigmentosum was diagnosed. Shortly after birth, low weight, microcephaly and psychomotor retardation had been observed, but the cause was not established. The patient progressively showed neurological disorders that included perceptive deafness, hyporeflexia and areflexia, as well as choreoathetotic movements. Therefore, we felt that the patient's symptoms fit De Sanctis-Cacchione syndrome.