Vemurafenib-associated neutrophilic panniculitis: An emergent adverse effect of variable severity.

Vemurafenib is a selective BRAF kinase inhibitor recently proven to improve rates of overall and progression-free survival in patients with BRAF-V600-mutant metastatic melanoma. The most common adverse effects of this targeted therapy are arthralgia, fatigue, and cutaneous lesions, including alopecia, photosensitivity, pruritus, hand-foot skin reactions, squamous cell carcinomas, keratoacanthomas, warty dyskeratomas and verrucous keratosis. Less frequently, cases of panniculitis of varying severity have been reported in patients receiving vemurafenib. In this report, we describe a patient who developed asymptomatic nodules on her legs, with complete, spontaneous resolution, while on vemurafenib therapy. A causal relationship was considered likely because of the timing of occurrence and the absence of other potential causes after extensive assessment. Vemurafenib therapy was continued at full dosage and no recurrences were observed. We believe that management of lobular panniculitis associated with selective BRAF inhibitors should vary according to the clinical presentation, degree of systemic involvement, and presence of joint inflammation. Physicians should be aware of this emergent side effect. Treatment discontinuation should be considered on a case-by-case basis because the condition may resolve spontaneously.

Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature.

Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy with characteristic histological findings. It was described in 2000, and 9 cases have since been described. Two women of 83 and 74 years consulted for long-standing telangiectasias. In case 1, they affected the limbs and trunk and in case 2 were located on the legs. Biopsies of these lesions showed dilated vascular structures whose walls were thickened due to deposition of eosinophilic hyaline material. The affected vessels were located in the superficial dermis in case 1, and in case 2 the reticular dermis was also affected. CCV is a microangiopathy of unknown etiology. Clinically it is indistinguishable from generalized essential telangiectasia and differs in its histology. CCV may be underdiagnosed, and some nonbiopsied cases of generalized essential telangiectasia may really be CCV. We contribute 2 new cases of this entity to help establish its clinical and epidemiological characteristics and make its etiology better known.

Recurrence rates of primary basal cell carcinoma in facial risk areas treated with curettage and electrodesiccation.

BACKGROUND: The incidence of basal cell carcinoma (BCC) is increasing. Curettage and electrodesiccation (CE) are not recommended for BCC treatment at medium- and high-risk facial sites. Surgical excision has been proposed as the treatment of choice. OBJECTIVE: We sought to evaluate the cumulative recurrence rate (RR) of primary BCC in facial areas of medium and high risk after CE. METHODS: This nonrandomized, clinical trial enrolled 257 patients with primary BCC located in medium- and high-risk facial areas, and treated with 4 or 5 cycles of CE by a single operator from a section specializing in BCC CE in a tertiary teaching hospital in Oviedo, Spain. Exclusion criteria for study entry included: recurrent BCC, fibrosing BCC, ill-defined BCC, and BCC larger than 10 mm in diameter (high-risk facial sites) or larger than 15 mm in diameter (medium-risk sites); BCC smaller than 4 mm; and nonbiopsy-proven BCC. BCCs included in the study were from the nose, and paranasal and nasal-labial fold (n = 105); eyelids and canthi (n = 48); perioral areas (n = 12); ears (n = 11); forehead and temples (n = 48); periauricular areas (n = 14); and malar areas and cheeks (n = 19). The primary outcome was recurrence of carcinoma, which was clinically evaluated by at least two observers in consensus. Data were analyzed using both a life table method and Kaplan-Meier analysis. The statistical analysis included best- and worst-case scenarios (which means that all cases lost to follow-up were considered as recurrences). RESULTS: The 5-year cumulative non-RR in the best-case scenario was 98.80% (SE 0.70, 95% confidence interval 97.40%-100%); thus, a 5-year cumulative RR of 1.20% was found after CE in our medium- and high-risk BCCs of the face (best case). The 5-year cumulative non-RR in the worst-case scenario was 79.40% (95% confidence interval 78.90%-79.90%); thus, a 5-year cumulative RR of 20.60%. LIMITATIONS: Retrospective design with a relatively small number of patients lost to follow-up is a study limitation. CONCLUSION: High 5-year cure rates can be obtained after CE of primary, nonfibrosing BCCs of medium- and high-risk areas of the face performed in a specialized section.

[Cutaneous semiology: a historical perspective of the evolution of basic terminology in Spain]. / Semiología cutánea: perspectiva histórica de la evolución de la terminología básica en España.

There are still numerous incompatibilities in the definitions of basic dermatological terms. In Spain, the problem is aggravated by the coexistence and conflict between two different semantic models, imported from other countries (France and the USA). These two models really have different "units": one model can be called "essentialist" and the other "nominalist". These models are not original to Spain; rather, each one shows the predominance or influence of an outside culture. Through a historical and evolutional study, we were able to verify that the origin of these semantic models goes back to Joseph Plenck and Robert Willan (essentialist model) and to Ferdinand Hebra (nominalist model), respectively. An analysis of these models from a historical perspective may help in understanding the origin of the current conflicts in the everyday use of Spanish dermatological semiology, and may therefore help resolve them.

[Acquired flat angiomas in the form of a port-wine stain]. / Angiomas planos adquiridos en mancha de vino de Oporto.

The development of flat angiomas in the form of a "port-wine stain" is an infrequent event in adults. We describe two cases of acquired flat angioma in the form of a "port-wine stain", which began in the second decade of life and grew slowly and steadily until reaching a large size. In the first case, it affected the front and back of the trunk, and in the second patient, the neck and left shoulder. Histopathology showed ectatic vessels in the papillary and reticular dermis, with no proliferation of endothelial cells.

[Scleromyxedema: ultrastructural study]. / Escleromixedema: estudio ultraestructural.

Scleromyxedema is a type of mucinosis that presents with some well defined clinical and histopathological characteristics. We describe the findings observed with transmission electron microscopy in a case that we recently studied. The patient was a 56-year-old female with a localized eruption of lichenoid papules on the face, upper trunk and limbs. It was accompanied by IgG lambda gammopathy. Optical microscopy showed the findings typical of scleromyxedema. Electron microscopy revealed the existence of a large number of fibroblasts with high activity levels in the synthesis and release of collagen fibers and a mucoid substance.

Nevus of Ota associated with ipsilateral deafness.

Nevus of Ota is a benign pigmentary disorder that involves the skin innervated by the first and second branches of the trigeminal nerve. It is a dermal melanocytosis frequent in Oriental persons but uncommon in white persons. We report a case of nevus of Ota in a white woman emphasizing the wide extension of the pigmentation and its association with ipsilateral sensorineural hypoacusia.

Dermoscopic features of plaque psoriasis and lichen planus: new observations.

BACKGROUND: Dermoscopic features of common inflammatory dermatoses are not well studied and previous reports on this topic are limited to the search of vascular features (capillaroscopy). OBJECTIVE: Dermoscopic features of psoriasis and lichen planus (LP) are investigated to determine both vascular and nonvascular features of these two dermatoses. PATIENTS AND METHODS: Dermoscopic images of 25 patients with LP and 20 patients with plaque psoriasis (PP) were evaluated. Findings were statistically analyzed including the reproducibility of scoring (Cohen's kappa statistics). RESULTS: Our observations clearly showed that the evaluation of both vascular and nonvascular findings improved the surface microscopy of these diseases. A vascular feature (homogeneous red globules) was the most significant dermoscopic finding in the PP dermoscopic pattern (20/20, 100%; p < 0.001). A nonvascular feature (whitish striae) was the most significant dermoscopic feature in the LP pattern (23/25, 92%; p < 0.001). Dermoscopic features of LP also included gray-blue dots, comedo, milium-like cysts, and vascular structures (red lines). The intraobserver and interobserver reproducibility of scoring was very high (k value, 0.9). CONCLUSION: Our study shows for the first time that the efficacy of surface microscopy of common inflammatory dermatoses may be improved by investigating both vascular and nonvascular findings and its practicability in daily practice by using the dermoscope. Although dermoscopy does not provide clear additional help in the clinical differentiation between typical PP and LP, it could be helpful in patients with darker skin and for teaching settings.

Metastatic eccrine porocarcinoma: a 5.6-year follow-up study of a patient treated with a combined therapeutic protocol.

BACKGROUND: Metastatic eccrine porocarcinoma (EP) is an uncommon, malignant, and potentially lethal neoplasm that arises from the eccrine sweat glands. OBJECTIVE: To present the 5.6-year follow-up study of a male patient with metastatic EP, highlighting the widespread cutaneous involvement and the response to the treatment carried out. METHODS: We describe the evolution of the tumor and the combined therapy carried out and review the treatments employed in previously reported cases, comparing them with ours. RESULTS: The patient developed multiple cutaneous and regional lymph node metastases 15 months after surgical excision of the primary tumor. He was treated with prophylactic lymphadenectomy, radiotherapy, and oral isotretinoin, subsequently substituted by tegafur. We have not found evidence of distant metastases after a 5.6-year follow-up. CONCLUSIONS: The optimum treatment for metastatic EP is not standardized, and the results obtained to date were generally poor. In this context, we consider it of interest to highlight the response of our patient to the therapeutic regime employed, which may be advantageous in future cases of this rare tumor.