Dermatologica Sinica - Articles in Press

Rapidly progressing, painful, ulcerative changes in long-standing psoriasiform plaques in a 46-year-old man - Corrected Proof

2012-05-10

A 46-year-old man was admitted to a community hospital with fever and rapidly progressing, painful, ulcerative skin lesions. He was transferred to our hospital for further examination. According to his medical history, he had been diagnosed with psoriasis, but this was not confirmed by a skin biopsy. The generalized pruritic plaques on his trunk and extremities had waxed and waned for several years but had exacerbated during the previous year. Skin examination on admission showed diffuse redness with periorbital, paranasal, and perioral sparing with multiple coalescent annular plaques on the trunk and extremities. Some of these lesions were indurated, others were crusted. Ulcerative plaques were present on the dorsum of the patient's right hand and soles (Figures A and A). A few inguinal lymph nodes were palpable on each side. The histopathological examination of specimens obtained from his left elbow, left thigh, and an inguinal lymph node revealed dense infiltrations of atypical medium-sized lymphocytes with nuclear pleomorphism. Eosinophils were identified in the dermis () of the specimens from the elbow and thigh and in the perifollicular zones of the lymph node. Immunohistochemical staining showed that these infiltrates were positive for CD2, CD3, CD4, CD5, CD8, and CD30 and negative for CD10, CD20, CD21, CD23, Epithelial Membrane Antigen (EMA), and Anaplastic Lymphoma Kinase (ALK). In situ hybridization for Epstein-Barr virus-encoded RNA (EBER) produced negative findings. Other significant laboratory data included the following: white cell count, 23,410 cells/μL (normal: 3500–9100/μL); band form, 35% (normal: 0–3%); total Immunoglobulin E (IgE), 574 IU/mL (normal for adults: <87 IU/mL); LDH, 461 U/L (normal: 98–192 U/L); ferritin, 387.3 ng/mL (normal for men: 23.9–336.2 ng/mL). The results of the bone marrow biopsy were normal. Abdominal computed tomography (CT) and bone scan revealed the lack of involvement in the internal organs. Human T cell lymphotropic virus I (HTLV)-related lesions were suspected; therefore, serological tests were performed. Enzyme-linked immunosorbent assay (ELISA) screening test revealed positivity to anti-HTLV I/II, and reactivity to HTLV type I (HTLV-I) was confirmed by Western blot analysis.

Pulmonary Mycobacterium abscessus infection-induced erythema induratum - Corrected Proof

Chin-Yin Liu, Horng-Shin Lin, Yu-Ping Hsiao, Yuan-Ti Lee, Jen-Hung Yang — 2012-05-07

Abstract: Erythema induratum (EI) is clinically characterized by recurrent crops of tender nodules on the lower legs and lobular panniculitis with granulomatous inflammation and pathologically characterized by vasculitis and focal fat necrosis. Currently, many authors consider EI to be a multifactorial disorder with diverse causes, including Mycobacterium tuberculosis and hepatitis C infection. Here, we report a case of a 65-year-old female with a 1-year history of recurrent crops of tender nodules and plaques on her bilateral lower legs. In addition, she had suffered from a chronic cough with sputum for 1 year and had contact history with pulmonary nontuberculous mycobacterial infection from her husband. The histopathological findings of the skin biopsies were consistent with the diagnosis of EI. Chest computed tomography revealed multiple lymphadenopathy and two sets of sputum cultures showed M abscessus. After 2 months of anti-nontuberculous mycobacterial therapy with ciprofloxacin, the skin lesions resolved completely and there was no recurrence within the following year.

Hypopigmented large-cell acanthoma of the eyelid - Corrected Proof

A. Tulin Mansur, Ikbal E. Aydingoz, Ali Kupelioglu, Emel Dikicioglu Cetin — 2012-05-07

Abstract: Large-cell acanthoma is an asymptomatic, slightly keratotic, usually hyperpigmented epidermal lesion that is mostly seen in the sun-exposed skin of middle-aged or elderly people. It is characterized by extraordinarily large keratinocytes that can reach up to twice their normal size without a change in the nucleus/cytoplasm ratio. Here, we describe a chronic case of large-cell acanthoma that involved the whole upper eyelid of a 67-year-old man for at least 15 years. The lesion was almost depigmented and demonstrated altered consistency of the eyelid, causing lax and folded skin. The lesion responded to treatment with topical tretinoin and urea. This case verifies the benign character of large-cell acanthoma and demonstrates how it may have atypical presentations.

Clinical characteristics of late-onset vitiligo in an Iranian population - Corrected Proof

Iraj Esfandiarpour, Saeedeh Farajzadeh — 2012-05-07

Abstract: Background: The age of onset of vitiligo is before 20 years in approximately in 50% of patients, and its incidence decreases with increasing age. In this study, our aim was to analyze the epidemiological and clinical factors of patients with late-onset vitiligo and compare these to patients with early-onset vitiligo.Methods: Eight hundred and twenty-five consecutive patients with vitiligo were examined from March 2003 through May 2006. The patients were divided into late-onset (disease onset > 50 years of age) and early-onset groups.Results: There were 54 (6.5%) patients diagnosed with late-onset vitiligo; 48.1% of these patients were female. Of those with early-onset vitiligo, 63.9% were female and 36.1% were male (p=0.02). Positive family history was present in 27.8% and 40.4% of the late- and early-onset groups, respectively. The most common first sites of involvement were the limbs in both groups. Generalized vitiligo (vitiligo vulgaris) was the most common presentation in both groups. Koebner phenomenon was detected in 79.6% of late- and 64.9% of early-onset vitiligo patients (p=0.02). Leukotrichia was present in 77.8% of late- and 52.7% of patients with early-onset vitiligo (p<0.001). Mucosal involvement was observed in 59.3% and 47% of late- and early-onset vitiligo patients, respectively (p=0.08). Associated diseases were seen in 18.5% of late- and in 23.1% of early-onset vitiligo patients.Conclusion: In patients with late-onset vitiligo, male gender, Koebner phenomenon, leukotrichia, and mucosal involvement were more common than in patients with early-onset vitiligo.

Anhidrotic ectodermal dysplasia—a case series in a medical center in southern Taiwan - Corrected Proof

Kuei-Chung Liu, Ching-Yuang Huang, Sheau-Chiou Chao — 2012-05-07

Abstract: Background: Anhidrotic ectodermal dysplasia (EDA) is a rare genodermatosis that causes developmental defects in ectoderm-derived structures. The clinical triad consists of hypodontia, hypotrichosis, and anhidrosis with other additional symptoms. The aim of this study is to summarize the clinical manifestations, family history, histopathological findings of the skin, and gene mutations in EDA patients who presented at a medical center in order to make the disease better known among medical personnel.Methods: A retrospective review of the medical charts and photographs of patients diagnosed with EDA at the Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University from June 1988 through May 2011 was performed. Information about the clinical manifestations, family history, histopathological findings of the skin, and genetic mutations was collected, and the initial presenting symptoms that lead to seeking medical services were determined.Results: One female patient and seven male patients were identified. The triad was present in all patients, in addition to other variable features. Five patients had a positive family history, while three of them were from the same family. Histopathological findings noted in the skin biopsies included the absence of sweat glands on the palmar skin. Genetic mutations were detected in six of seven patients in this study, but there was no genotype-phenotype correlation. The initial presentations most commonly noted were the absence of sweating with episodic hyperthermia since birth. Eczema since infancy was the main reason why these patients visited the dermatology department following the diagnosis of EDA.Conclusions: EDA is a rare genodermatosis, and it is invariably characterized by its clinical triad. Family history and genetic analysis help in the diagnosis. The dermatologist, pediatrician, and dentist are usually the medical personnel that these patients first visit, and therefore these individuals should be acquainted with this disease in order to provide appropriate care.

New-onset guttate psoriasis following intravesical immunotherapy of Bacillus Calmette–Guerin - Corrected Proof

Chih-Tsung Hung, Wei-Ming Wang, Chih-Wei Tsao, Chien-Ping Chiang — 2012-04-30

Abstract: Intravesical Bacillus Calmette–Guerin (BCG) is usually a well-tolerated treatment for urethelial carcinoma of the bladder. A 60-year-old male endured multiple erythematous white-scaled maculopapules over his trunk, back, and four extremities after 2 months of the 6-weekly intravesical instillation of BCG. Skin biopsy revealed parakeratotic hyperkeratosis, intracorneal neutrophilic Munro's abscess, and acanthosis. Guttate psoriasis was diagnosed, and he was treated successfully with topical steroid and narrow band UV-B phototherapy. We present the first Asian case of guttate psoriasis associated with intravesical BCG immunotherapy and review the literatures.

Multiple hypertrophic purpuric to erythematous papuloplaques on four limbs of a 38-year-old man - Corrected Proof

Yung-Yi Lee, Jui-Hung Ko, Mei-Ching Lee, Jheng-Wei Lin — 2012-04-05

A 38-year-old healthy man presented with a 6-month history of mildly pruritic skin lesions on joints of four limbs. Initially, the itchy erythematous papules developed from extensor aspects of the joints, then the papules gradually progressed to violaceous plaques. He denied any trauma history or chronic scratching on the cutaneous lesions. On physical examination, there were multiple purpuric to erythematous hypertrophic, oval-shaped, and well-demarcated plaques with varying size on the extensor aspects of the left second and third metacarpophalangeal joints, left elbow, and bilateral knees (). The initial clinical diagnosis was lichen planus or lichen simplex chronicus. Skin biopsy revealed marked hyperkeratosis, hypergranulosis, moderate acanthosis, and thickened dermal vessels infiltrated and surrounded by many neutrophils, and scattered eosinophils and lymphocytes. Nuclear dusts and focal mild extravasation of red blood cells with some vessels surrounded with fibrin deposits were seen (). No bacilli are detected by Gram and Warthin-Starry stains.

Multiple erythematous nodules with a necrotic center in a patient with acute lymphoblastic leukemia - Corrected Proof

Yu-Wen Yeh, Wei-Ming Wang — 2012-03-19

A Taiwanese man 20 years of age presented with neutropenic fever, dyspnea, and generalized skin eruptions after receiving chemotherapy for the management of acute lymphoblastic leukemia (A). The chemotherapy regimen was given for 6 months but discontinued due to the profound pancytopenia, in which the absolute neutrophil count (ANC) was below 100/mm3. Multiple discrete painful skin eruptions started to develop 2 months later. The initial presentation of skin lesions was multiple tender erythematous papules to subcutaneous nodules carrying a necrotic center (B), in which bullae and crusted ulceration developed progressively (C and D). Skin biopsy showed numerous acute angle branching septate hyphae within dermal blood vessels resulting in vascular occlusion (A and B). Concurrently, chest radiography revealed reticulonodular opacities in the bilateral lung. The patient was treated with oral voriconazole 200 mg twice a day and intravenous micafungin 100 mg once a day for 3 weeks according to the results from micro-organic culture of both sputum and skin specimen. However, the general condition including neutropenia and dyspnea were not reversed. He eventually died due to sepsis with respiratory failure.

Mottled hyperpigmentation on the face and neck of a woman 35 years of age - Corrected Proof

Chih-Tsung Hung, Hong-Wei Gao, Bai-Yao Wu, Chien-Ping Chiang — 2012-03-14

A Taiwanese woman 35 years of age presented with asymptomatic, mottled hyper- and hypopigmented macules on her neck and face, which had presented in the last 2 years. Spotted macules were first noted on her neck and had slowly progressed to involve her face as time went by. Her medical history was unremarkable. She did not have photosensitivity or any other systemic or cutaneous diseases before the onset of the lesions. There were no family members with similar cutaneous presentations. Examination revealed diffuse hyperpigmentation with numerous and varying sizes of hypopigmented spots over her neck and face (). There were no apparent erythemas, blisters, telangiectasia, atrophy, or papular lesions. Her mental and developmental milestones were normal.

Dendritic cell neurofibroma with pseudorosettes: One case report and literature review - Corrected Proof

Hsin-Yin Huang, Yu-Hung Wu, Ya-Ju Hsieh — 2012-02-27

Abstract: Dendritic cell neurofibroma with pseudorosettes is a recently proposed, rare variant of neurofibroma. It has a distinctive histopathological finding that is composed of two types of cells that form a pseudorosette, which may be easily misdiagnosed as other neural tumor. Here, we report one typical case in order to raise awareness of this entity.

Pachyonychia congenita: report of two cases and mutation analysis - Corrected Proof

Jia-Ming Yeh, Ching-Yuang Huang, Sheau-Chiou Chao — 2012-02-17

Abstract: Pachyonychia congenita (PC) comprises a group of rare autosomal dominant genetic disorders that involve ectodermal dysplasia. It is characterized by hypertrophic nail dystrophy, focal palmoplantar keratoderma, follicular keratoses, and oral leukokeratosis. Historically, PC has been subdivided into two subtypes, PC-1 or PC-2, on the basis of clinical presentation. However, differential diagnosis based on clinical grounds, especially in young and/or not fully penetrant patients, can be difficult. In addition, clinical analysis of the large case series has shown that there is considerable phenotypic overlap between these two subtypes recently. Based on the advent of molecular genetics and the identification of the genes causing PC, more specific nomenclature has been adopted. Therefore, diagnosis at the molecular level is useful and important to confirm the clinical impression. In this report, we describe two typical cases of PC with mutation analysis revealed a small deletion (514_516delACC, Asn172del) and a point mutation (487 G > A, GAG → AAG, Glu163Lys) in the KRT6A gene.

Intra-arterial angiolymphoid hyperplasia with eosinophilia of the temporal artery: A case report and review of the literature - Corrected Proof

Hao-Tien Hsiao, Yu-Hung Wu — 2012-02-17

Abstract: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a relatively common benign vascular disorder of uncertain etiology and has many synonyms, such as pseudopyogenic granuloma, atypical pyogenic granuloma, epithelioid hemangioma, and histiocytoid hemangioma. It usually develops on the head and neck, manifesting as reddish-to-dark purpuric papules or nodules. In this article, we report two patients who had uncommon intra-arterial ALHE that occurred in the temporal artery. In both cases, the ALHE presented as skin-colored subcutaneous nodules over the forehead, mimicking temporal arteritis. Histopathologically, intravascular epithelioid endothelial cell proliferation occurred, with lymphocyte and eosinophil infiltration in the stroma. One patient also showed typical findings of ALHE in the adjacent soft tissue. We know of seven similar cases that have previously been reported in the literature to date.

A case of cutaneous clear cell sarcoma determined by clinicopathological and cytogenetic analysis - Corrected Proof

Yung-Ting Chuang, Tze-Yi Lin, Chih-Jung Hsu — 2012-02-17

Abstract: Cutaneous clear cell sarcoma (CCS) is a rare soft tissue malignancy that typically manifests in the distal extremities of young adults. Although it shows melanocytic differentiation, CCS is clearly pathologically and genetically distinct from malignant melanoma. Here, we report the case of a 43-year-old male who had an asymptomatic, deep-seated, slowly enlarging, firm mass over the right heel for 30 years that recently and rapidly progressed with tenderness. We arranged for the total excision of the tumor. Pathological and cytogenetic analysis of the biopsied specimen showed that it was a clear cell sarcoma. Computed tomography and positron emission tomography scans showed no signs of metastasis, and no other abnormal hypermetabolic lesions were detected. Wide excision with split-thickness skin graft and a sentinel lymph node biopsy were performed. Because of positive findings in the sentinel lymph nodes, the patient was transferred to the plastic surgery department for further radical popliteal and inguinal lymph node dissection. The patient has received regular outpatient follow-up care in our hospital for the past 8 months with no evidence of recurrence.

Multiple erythematous erosive papules and nodules on the perianal area of an 84-year-old bedridden woman - Corrected Proof

Yung-Yi Lee, Jui-Hung Ko, Yi-Chin Shih — 2011-11-14

An 84-year-old woman presented with a 6-month history of multiple painful cutaneous papulonodules on the buttocks and perianal area. She had underlying dementia and was bedridden. She had urine incontinence and encopresis for>10 years. The patient initially developed papules around the anus. She did not undergo any systemic or topical treatment, and the papules gradually enlarged to papulonodules after 6 months. Her family did not report any history of topical skin contact. Physical examination showed many flat-topped erosive erythematous papules and nodules on the perianal region (). The rapid plasma reagin and Treponema pallidum hemagglutination tests yielded negative results. Bacterial culture did not yield any microbial growth. A skin biopsy showed psoriasiform acanthosis with a moderate infiltrate of lymphoplasma cells and neutrophils in the papillary and upper dermis. Koilocytic changes and fungal hyphae were not observed in the epidermis ().

Multiple brownish warty papulonodules on left dorsal hand - Corrected Proof

Che-Yi Chou, Shih-Wei Yeh — 2011-11-11

A 56-year-old nonobese man presented with progressive thickening and darkening of the left dorsal hand for 3 months. The patient denied any history of systemic disease, such as diabetes mellitus or other endocrinopathies, and there was no family history of malignancy. He reported no systemic symptoms such as weight loss, general malaise or melena, with the exception of a 1-month history of low back pain. Physical examination revealed multiple brownish warty papules and nodules over the left dorsal hand (). An incisional biopsy of the plaque was performed and histopathological examination demonstrated pronounced hyperkeratosis, acanthosis, and papillomatosis ().

Histiocytoid Sweet syndrome: Report of two cases and review of the literature - Corrected Proof

Ching-Fu Huang, Bai-Yao Wu, Fang-Yih Liaw, Wei-Ming Wang, Chien-Ping Chiang — 2011-11-10

Abstract: Histiocytoid Sweet syndrome (SS) is a rare inflammatory disease that has recently been described as a variant of classic SS. Histopathologically, histiocytoid SS is characterized by papillary dermal edema with infiltration of histiocyte-like cells into the upper dermis. These microscopic features may be similar to those of leukemia cutis, which involves infiltration of malignant blasts into the dermis. However, the treatment and clinical prognosis of these two conditions are quite different. Here, we report the case of two Taiwanese patients with histiocytoid SS. Interestingly, one of the patients had a history of myelodysplastic syndrome and was initially considered to have ongoing leukemic transformation with concomitant leukemia cutis. Finally, the patient was diagnosed with histiocytoid SS based on histological findings, and both patients were successfully treated with low-dose oral corticosteroids.

Transient zinc deficiency syndrome in a breast-fed infant due to decreased zinc in breast milk (type II hypozincemia of infancy): A case report and review of the literature - Corrected Proof

Wei-Li Yang, Chao-Kai Hsu, Sheau-Chiou Chao, Ching-Yuang Huang, Julia Yu-Yun Lee — 2011-10-31

Abstract: Type II hypozincemia of infancy is a rare, hereditary zinc deficiency occurring in infants while exclusively on breast feeding. It is caused by defective transfer of zinc into breast milk. Only a few dozen cases have been reported. A 6-month-old, full-term, breast-fed female infant presented with a 3-week history of erythematous to dusky red papules and annular plaques over the perioral and diaper area as well as the digits. The eruption was accompanied by poor appetite and irritable crying. Serum zinc was low (4.896 μmol/L, normal = 10.71−18.36 μmol/L) in the patient but was normal in the mother. Interestingly, the zinc level in the breast milk was very low (2.142 μmol/L; normal postpartum zinc = 18.36 μmol/L at 6 months). Histopathology of a skin biopsy specimen showed spongiotic psoriasiform dermatitis with pallor of superficial keratinocytes, consistent with deficiency disease. With oral zinc sulfate supplement, her skin lesions improved significantly within 4 days. Type II hypozincemia needs to be differentiated from the classical hereditary acrodermatitis enteropathica, which typically develops symptoms after weaning because of poor intestinal absorption of zinc in the affected infants. Mutations in zinc transporter genes have been detected in SLC39A4 (Zip4) and SLC30A2 (ZnT2), respectively, in classical acrodermatitis enteropathica and type II hypozincemia. No mutation was found in these two genes in the present pedigree. Therefore, the genetic defect in our patient might involve other zinc transporter genes.

Pigmented epithelioid melanocytoma: Report of a case and review of 173 cases in the literature - Corrected Proof

Pai-Shan Cheng, Shih-Sung Chuang, Tseng-Tong Kuo, Feng-Jie Lai — 2011-10-28

Abstract: Pigmented epithelioid melanocytoma (PEM), or animal-type melanoma, is an unusual variant of melanoma which has been reported to have indolent behavior and a relatively good prognosis. We report a 12-year-old girl with PEM on the third finger web of her right hand. Histopathologically, it was composed of heavily pigmented dermal epithelioid and spindled melanocytic tumor cells. A sentinel lymph node biopsy was negative, and no recurrence was noted 1 year later. We reviewed 173 previously published cases of PEM or so-called animal-type melanoma in the literature. Among the 173 cases and our case, extremities were the most common sites of occurrence (52/129, 40.3%), and most of the depth of invasions were Clark level IV and V [76/114 (66.7%) and 33/114 (28.9%), respectively]. Lymph nodes metastasis was noted in 39/89 (43.8%) of the cases being investigated. Only two cases died of the disease with visceral metastasis. Thus, a more advanced level of invasion and the presence of lymph node metastasis did not imply a definitely malignant clinical course, because spreading beyond lymph nodes was rare (5/174, 2.9%). However, long-term follow-up with more cases and further research are needed to fully delineate the true biological nature of this pigmented melanocytic tumor.

Dermoscopic features of discoid lupus erythematosus - Corrected Proof

Tsung-Ming Tsai, Kuo-Chia Yang, Tsung-Hua Tsai — 2011-10-28

An otherwise healthy 38-year-old man presented with skin lesions over his scalp and face of several months’ duration. The lesions were exacerbated after sun exposure. Physical examination showed many erythematous to brownish alopecia patches located on his scalp (A) and atrophic plaques on his face (B). Laboratory studies including hematologic tests, erythrocyte sedimentation rate, anti-nuclear antibody, and routine urine tests were within normal limits. Anti-dsDNA was 10 KIU/L (normal limit <10 KIU/L).

Concomitant occurrence of acneiform eruption, alopecia areata, and urticaria during adalimumab treatment in a patient with pustulosis palmoplantaris: Case report and literature review - Corrected Proof

Guan-Yi He, Tsen-Fang Tsai — 2011-10-26

Abstract: Adalimumab is a fully human immunoglobulin G1 monoclonal antibody against tumor necrosis factor (TNF)-α that is increasingly used for the treatment of many autoimmune diseases. However, it has also been reported that adalimumab can induce many adverse cutaneous reactions, including paradoxical psoriasiform eruptions. We describe a patient with pustulosis palmoplantaris who developed four cutaneous adverse reactions, including eczematous lesions, acneiform eruption, alopecia areata, and urticaria during adalimumab treatment. A common histopathological finding in these acneiform and urticarial lesions was the presence of eosinophilic infiltrates. Some authors assume that cross-regulation between TNF-α and interferon-α may contribute to development of a clinical spectrum of cutaneous reactions in predisposed individuals undergoing anti-TNF therapy. The use of different biologics, including adalimumab, etanercept, and ustekinumab, did not seem to improve pustulosis palmoplantaris disease activity in our patient.

Porocarcinoma in situ showing follicular differentiation: a case report - Corrected Proof

Chia-Lan Ou, Chien-Jui Cheng, Kuo-Hsien Wang — 2011-10-24

Abstract: Poroid neoplasm is a skin appendage tumor that has both benign and malignant counterparts. It has traditionally been regarded as of eccrine origin and has four types: intraepidermal poroma (hidroacanthoma simplex), poroma, dermal duct tumor, and poroid hidradenoma. Here we describe the case of a 64-year-old woman who had a verrucous, erythematous to brownish tumor on her left buttock for many years. Histopathology revealed an intra-epidermal poroid tumor with both benign and malignant parts. The benign part had intra-epidermal nests of poroid cells, which were smaller, monomorphic and sharply marginated from adjacent keratinocytes. The malignant part showed similar cell types, but had a higher nuclear/cytoplasmic ratio, pleomorphism, and prominent mitoses. Ductal structures were noted in neoplastic cells and an epithelial membrane antigen stain was strongly positive. Interestingly, peripheral palisading and primitive follicular germ formation were also observed in the neoplasm, which suggests follicular differentiation. We made a final diagnosis of porocarcinoma in situ with follicular differentiation, which may support the folliculosebaceous–apocrine unit theory, but a tumor with such a combination has not been described before.

Congenital erythropoietic porphyria - Corrected Proof

Wen-Hao Lee, Wei-Chun Tai, Po-Yuan Wu — 2011-10-24

Abstract: Congenital erythropoietic porphyria (CEP), or “Günther disease”, is a rare variant of porphyria. It is an autosomal recessive disease caused by deficient uroporphyrinogen III synthase (URO-III-synthase), the fourth enzyme in the heme biosynthetic pathway. We herein report a case of a man with the typical clinical presentations of hyper- and hypo-pigmentation and blister formation over sun-exposed areas, mutilation of the fingers, dark-purple urine, and erythrodontia with pinkish fluorescence under a Wood’s lamp. The diagnosis was confirmed by decreased activity of URO-III-synthase in red blood cells (RBC) and a porphyrin profile compatible with CEP.

Good therapeutic outcome of radiotherapy in large facial skin cancers - Corrected Proof

2011-10-24

Reports of treating large facial skin cancers by radiotherapy are scant. Complete resolution of a huge exophytic angiosarcoma of the central face could be achieved by radiotherapy with relapse-free survival of 5 years. In this report, we describe two cases of large squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) treated by radiotherapy with good clinical and cosmetic results. Although these tumors might be amenable to conventional surgery or Mohs micrographic surgery followed by reconstruction surgery, the cosmetic and functional outcomes may not be satisfactory.