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Background: Basal cell carcinoma (BCC) is the most common form of cancer among Caucasians and its incidence continues to rise. Surgical excision (SE) is considered standard treatment, though randomised trials with long-term follow-up are rare. We now report the long-term results of a randomised trial comparing surgical excision with Mohs' micrographic surgery (MMS) for facial BCC., Methods: 408 facial, high risk (diameter at least 1cm, H-zone location or aggressive histological subtype) primary BCCs (pBCCs) and 204 facial recurrent BCCs (rBCCs) were randomly allocated to treatment with either SE or MMS between 5th October 1999 and 27th February 2002. The primary outcome was recurrence of carcinoma. A modified intention to treat analysis was performed., Findings: For primary BCC, the 10-year cumulative probabilities of recurrence were 4.4% after MMS and 12.2% after SE (Log-rank test χ(2) 2.704, p=0.100). For recurrent BCC, cumulative 10-year recurrence probabilities were 3.9% and 13.5% for MMS and SE, respectively (Log-rank χ(2) 5.166, p=0.023). A substantial proportion of recurrences occurred after more than 5years post-treatment: 56% for pBCC and 14% for rBCC., Interpretation: Fewer recurrences occurred after treatment of high risk facial BCC with MMS compared to treatment with SE. The proportion of recurrences occurring more than 5years post-treatment was especially high for pBCC, stressing the need for long-term follow-up in patients with high risk facial pBCC., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: Although effective in superficial basal cell carcinoma (BCC), the treatment effect of photodynamic therapy (PDT) in nodular BCC (nBCC) is still questionable. The relation between tumor thickness and PDT failure is unclear., Objective: We sought to compare long-term effectiveness of fractionated 20% 5-aminolevulinic acid (ALA)-PDT with prior partial debulking versus surgical excision in nBCC. The effect of tumor thickness on ALA-PDT failure was analyzed., Methods: 173 primary, histologically proven nBCCs in 151 patients were randomized to fractionated ALA-PDT (n = 85) or surgical excision (n = 88). Two PDT illuminations were performed with a 1-hour interval. Follow-up was at least 5 years posttreatment. Clinical recurrences were confirmed histologically., Results: A total of 171 nBCCs were treated and had a median follow-up of 67 months (range 0-106). At 60 months, 23 tumors had recurred in the ALA-PDT group and 2 tumors in the surgical excision group. Cumulative recurrence probabilities 5 years posttreatment were 30.7% (95% confidence interval [CI] 21.5%-42.6%) for ALA-PDT and 2.3% (95% CI 0.6%-8.8%) for surgical excision (P < .0001). Two tumors in the ALA-PDT group recurred at 72 and 91 months posttreatment. Cumulative probability of recurrence-free survival post-PDT was 65.0% (95% CI 51%-76%) for nBCC measuring greater than 0.7 mm in thickness and 94.4% (95% CI 67%-99%, P = .018) for tumors less than or equal to 0.7 mm., Limitations: Tumor thickness on punch biopsy specimen might differ from the total lesion thickness., Conclusions: In nBCC, 5-year cumulative probability of recurrence after surgical excision is lower than after fractionated ALA-PDT with prior debulking. Although surgical excision remains the gold standard of treatment, PDT might be an alternative for inoperable patients with thin (≤0.7 mm) nBCC., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Superficial basal-cell carcinoma is most commonly treated with topical non-surgical treatments, such as photodynamic therapy or topical creams. Photodynamic therapy is considered the preferable treatment, although this has not been previously tested in a randomised control trial. We assessed the effectiveness of photodynamic therapy compared with imiquimod or fluorouracil in patients with superficial basal-cell carcinoma., Methods: In this single blind, non-inferiority, randomised controlled multicentre trial, we enrolled patients with a histologically proven superficial basal-cell carcinoma at seven hospitals in the Netherlands. Patients were randomly assigned to receive treatment with methylaminolevulinate photodynamic therapy (MAL-PDT; two sessions with an interval of 1 week), imiquimod cream (once daily, five times a week for 6 weeks), or fluorouracil cream (twice daily for 4 weeks). Follow-up was at 3 and 12 months post-treatment. Data were collected by one observer who was blinded to the assigned treatment. The primary outcome was the proportion of patients free of tumour at both 3 and 12 month follow up. A pre-specified non-inferiority margin of 10% was used and modified intention-to-treat analyses were done. This trial is registered as an International Standard Randomised controlled trial (ISRCTN 79701845)., Findings: 601 patients were randomised: 202 to receive MAL-PDT, 198 to receive imiquimod, and 201 to receive fluorouracil. A year after treatment, 52 of 196 patients treated with MAL-PDT, 31 of 189 treated with imiquimod, and 39 of 198 treated with fluorouracil had tumour residue or recurrence. The proportion of patients tumour-free at both 3 and 12 month follow-up was 72.8% (95% CI 66.8-79.4) for MAL-PDT, 83.4% (78.2-88.9) for imiquimod cream, and 80.1% (74.7-85.9) for fluorouracil cream. The difference between imiquimod and MAL-PDT was 10.6% (95% CI 1.5-19.5; p=0.021) and 7.3% (-1.9 to 16.5; p=0.120) between fluorouracil and MAL-PDT, and between fluorouracil and imiquimod was -3.3% (-11.6 to 5.0; p=0.435. For patients treated with MAL-PDT, moderate to severe pain and burning sensation were reported most often during the actual MAL-PDT session. For other local adverse reactions, local skin redness was most often reported as moderate or severe in all treatment groups. Patients treated with creams more often reported moderate to severe local swelling, erosion, crust formation, and itching of the skin than patients treated with MAL-PDT. In the MAL-PDT group no serious adverse events were reported. One patient treated with imiquimod and two patients treated with fluorouracil developed a local wound infection and needed additional treatment in the outpatient setting., Interpretation: Topical fluorouracil was non-inferior and imiquimod was superior to MAL-PDT for treatment of superficial basal-cell carcinoma. On the basis of these findings, imiquimod can be considered the preferred treatment, but all aspects affecting treatment choice should be weighted to select the best treatment for patients., Funding: Grant of the Netherlands Organization for Scientific Research ZONMW (08-82310-98-08626)., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: Generally basal cell carcinoma can be simply and curatively treated. However, large and long-standing tumours can be locally very destructive and in rare cases even metastasize. Hedgehog pathway inhibitors such as vismodegib constitute a new and promising therapy for metastatic or locally advanced basal cell carcinoma., Case Description: We describe a patient with metastasized basal cell carcinoma and basal cell nevus syndrome who, in the context of a study, was successfully treated with vismodegib. The main undesirable effects in our patient were muscle cramps, loss of taste, nausea and hair loss., Conclusion: Basal cell carcinoma is potentially a locally destructive skin tumour that only very rarely metastasizes. Hedgehog pathway inhibitors such as vismodegib can be administered in a selected group of patients with basal cell carcinoma.

Background: The type of treatment for a basal cell carcinoma (BCC) depends on the histologic subtype. Histologic examination is usually performed on incisional biopsy specimens. In primary BCC, the histologic subtype is correctly identified with a punch biopsy in 80.7% of cases. In recurrent BCC, correct identification is more difficult because of discontinuous growth caused by scar formation. Because an aggressive histologic subtype has a significantly higher risk for recurrence in these tumors, the histologic subtype is at least as important in recurrent BCC as it is in primary BCC., Objective: To investigate the correlation between histologic findings on punch biopsy specimens and subsequent excision specimens in recurrent BCC. Furthermore, we sought to clarify how often an aggressive histologic subtype was missed, based on the punch biopsy specimen., Methods: We compared the histologic subtype in a punch biopsy specimen with the subsequent excision specimen in recurrent BCC. All BCCs were coded and judged randomly by the same dermatopathologist., Results: In 24 of 73 investigated BCCs (32.9%), the histologic subtype of the initial biopsy did not match with the histologic subtype of the subsequent excision. Of the 37 excised BCCs with an aggressive histologic subtype, 7 (19%) were missed by the initial punch biopsy., Limitations: Intraobserver variation may have affected the results of this study., Conclusions: Discriminating tumors with any aggressive growth is relevant for treatment. However, in recurrent BCC, the histology of the biopsy specimen does not always correlate with the histology of the definitive excision. This may have important therapeutic implications., (Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

We present a 49-year-old man seen at the dermatology outpatient department with a 3-year history of painful swollen digits of hands and feet. On enquiry he reported dysuria. On examination we saw extensive swelling of the digits, keratosis of the nails, and some psoriasiform skin lesions on the soles of the feet. The differential diagnosis included acrodermatitis continua suppurativa, reactive arthritis and psoriatic arthritis. Radiographic imaging revealed the presence of arthritis. Testing proved negative for rheumatoid factor and positive for HLA-B27 making spondyloarthropathy the most likely diagnosis, either in the form of reactive arthritis or psoriatic arthritis. The patient was treated with combination therapy of doxycycline, methotrexate and folic acid. Because of insufficient response to therapy, the methotrexate dose was raised and eventually etanercept was added. During the last visit to the outpatient clinic, the patient still showed insufficient response to therapy.

A 5-day-old girl presented with thrombocytopenia, leukopenia, anemia and crusted purpura on the skin. The diagnosis Langerhans' cell histiocytosis (LCH) was suspected on clinical grounds and subsequently confirmed by histopathological examination of a skin biopsy. Cytological examination of a bone marrow aspirate revealed numerous histiocytes, which is suspect for bone-marrow infiltration by LCH. LCH is a condition in which a clonal population of Langerhans' cells accumulates in various tissues, causing tissue damage and/or dysfunction. The prognosis of this disease depends on the age of the patient, the extent of the disease and the presence of vital organ failure. In case of organ dysfunction, systemic chemotherapy is indicated. Although very rare, LCH can be a life-threatening disease. Early diagnosis can improve chances of survival. We briefly discuss diagnostic procedures and treatment.

Primary cutaneous follicle center lymphoma (PCFCL) is a neoplasm with differentiation of centrocytes and centroblasts presenting in the skin. At the time of initial manifestation, extracutaneous involvement is absent. PCFCL is considered as an indolent variant of primary cutaneous B-cell lymphomas since dissemination to extracutaneous sites is rare and the prognosis is favorable. Here we describe a 30-year-old man who was diagnosed with a cutaneous FCL and did not show extracutaneous affection at the time of occurrence. Six months later, however, he developed a diffuse large B-cell non-Hodgkin lymphoma localized in several lymph nodes of the neck that most likely reflects the occurrence of a second primary tumor in the same patient.

Background: Skin cancer incidence rates have been increasing for decades and this increase is expected to continue. Surgical excision (SE) is the treatment of first choice for nodular basal cell carcinoma (nBCC). Photodynamic therapy (PDT) has proven to be an effective treatment for superficial basal cell carcinoma. Its long-term efficacy in nBCC has not yet been established., Objectives: Prospectively compare the efficacy of 5-aminolaevulinic acid (ALA)-PDT and SE in terms of failure rates with long-term follow-up. Determinants of failure in the study population, such as the effect of tumour depth, were analysed retrospectively., Methods: A randomized controlled trial in 173 primary nBCCs in 149 patients. Primary nBCCs were randomly assigned either to PDT (n = 85) or to SE (n = 88). Tumours treated with PDT were illuminated twice on the same day, 4 h after application of ALA cream, 3 weeks after debulking. SE was performed under local anaesthesia with a 3-mm margin, followed by histological examination. An intention-to-treat analysis was performed., Results: In total, 171 primary nBCCs in 149 patients were treated. A 3-year interim analysis revealed that the cumulative incidence of failure was 2.3% for SE and 30.3% for PDT (P < 0.001). Tumour depth and other analysed determinants of failure were not significantly related to treatment failure., Conclusions: SE proved to be significantly more effective than treatment with fractionated illumination ALA-PDT. Therefore, in the treatment of primary nBCC, SE is preferred over PDT following this treatment regimen.

A 53-year-old woman with blistering on the hands was diagnosed with porphyria cutanea tarda (PCT). At that time, she was treated with interferon for liver metastasis of a pancreas tumor since 7 years. Although apparently rare, PCT has been described as possible cutaneous manifestation both of hepatic tumors and interferon treatment. Further, PCT can also be exacerbated by hepatitis C virus infection, alcohol, estrogens, HFE gene mutations predisposing for hereditary hemochromatosis, hexachlorobenzene, and 2,3,7,8-tetrachlorodibenzo-p-dioxin. Therefore, it is important to keep in mind that the manifestation of PCT may be associated with distinct precipitating factors, which are briefly discussed here.

Bullous congenital ichthyosiform erythroderma (BCIE), also known as epidermolytic hyperkeratosis (EHK, OMIM 113800) is characterized by erythroderma and blistering at birth, leading to generalized hyperkeratosis of varying severity in adulthood. Clinically, BCIE can be divided into two groups: BCIE with or without palmoplantar involvement, associated with mutations in keratin 1 or keratin 10, respectively. Here we report a newborn with generalized erythema, blistering and erosions at the time of birth. No hyperkeratosis was seen on the palms and soles. The lack of palmoplantar involvement suggested that keratin 10 could be involved. DNA analysis showed a known mutation in the keratin 10 gene.