Anastasiya Atanasova Chokoeva

Tinea profunda of the genital area. Successful treatment of a rare skin disease

We present a rare case of 36‐year‐old female patient, who developed a severe Majocchi granuloma in the pubis after waxing, with isolated causative agent Trichophyton mentagrophytes. The condition was initially misdiagnosed as a bacterial folliculitis and treated unsuccessfully with topical corticosteroids and antibiotics. After the adequate diagnose was confirmed by mycological examination, followed by histopathological verification, complete remission of the symptoms was achieved at the 4th week after initiating 6 weeks course of antifungal therapy with Terbinafine 250 mg/daily dose, while good therapeutic response was observed even in the 10th day. The etiopathogenesis of the disease, as well as its current treatment options are considered, in respect to the rare occurrence of this condition in the pubic area and its frequent misdiagnosis.

Plexiform Neurofibroma: A Case Report

AbstractPlexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds.We report a rare case of a 30-year-old man who presented with a progressive facial deformity that began in early childhood. Skin examination also revealed multiple neurofibromas and café-au-lait macules on the trunk and arms. Histopathological examination on biopsy samples showed overgrowth of peripheral nerve components and connective tissue. Two diagnostic criteria for NF-1 (plexiform variant) were met, the patient did not accept to undergo genetic testing. Craniofacial MRI confirmed the presence of a deforming mass arising from the left side of his face giving homolateral eye dislocation.Surgery is the mainstay of the treatment. However, the patient expressed the preference to avoid surgery and chose to undergo clinical follow-up every 6 months.Diagnosis of plexiform neurofibromas is usually made clinically, especially if classical hallmarks of NF-1 are present. Therapy is surgical, aiming at resecting deforming masses and cancerous tissue when malignant transformation occurs.

Melanoma in a Chinese dragon tattoo

A 42-year-old white man presented to our clinic in October, 2015, with itching in the nasolabial fold and around the eyebrows, with accompaying erythema and desquamation consistent with seborrhoeic dermatitis. We noted multiple naevi on whole body dermatological examination, as well as a multicoloured tattoo of a Chinese dragon covering almost his entire back, arms, and chest. On closer inspection he had one clinically dysplastic pigmented naevus, sited within a scale of the dragon’s tail (fi gure). He reported having the tattoo done 1 year previously, but the naevus had been present at least 5 years earlier, and the tattoo artist had purposefully worked around the existing lesion. The patient had type II skin with blonde hair and blue eyes, and had previously worked in the French Foreign Legion during which time he had had prolonged sun exposure. He reported no medical history apart from multiple painful sunburns in childhood, took no medication, and family history for dermatological disease was negative. We did total surgical excision with 1 cm surgical margins. Histological examination confi rmed the diagnosis of superfi cial spreading melanoma with tumour thickness 0·26 mm, and the tumour was classifi ed as IA according to the American Joint Committee on Cancer (AJCC) staging system. Additional screening tests were within the normal range. We have given sun care education and planned follow-up in line with the AJCC recommendations for melanoma stage IA. The patient remains well with no signs of further disease. Many skin lesions have been reported in association with tattoos, both benign and malignant. The pathogenesis of malignant transformation in tattoos is unknown, although hypotheses include the toxic eff ects of the pigments and their part in causing oxidative stress in response to ultraviolet light, chronic infl ammatory response, trauma as a trigger for malignant changes in existing lesions, and seeding of melanoma cells from existing melanomas during the tattoo process. In our case the patient’s existing pigmented lesion had not been aff ected by the needle during the tattoo procedure. Although not considered a risk factor for malignant transformation, physicians should examine tattoos carefully, because they might disguise a malignant lesion.

Relapsing advanced metatypical basal cell carcinomas (MTBCC) of the face: Surgical modalities

SummaryMetatypical basal cell carcinoma (MTBCC) represents a high-risk type of cutaneous tumour. We report about three different patients with relapsing advanced large MTBCC: one of the scalp and two of the cheek region. Such patients required in most of the cases a complex surgical approach to achieve a stable and complete remission.In the first presented patient a combination of flaps and grafts has been performed. We describe tailored surgical approaches. By this contrivance it is possible to treat even elderly patients with exposed bone after complete excision effectively and safe. Interdisciplinary team work is for the benefit of these patients.ZusammenfassungDas metatypische Basalzellkarzinom (MTBCC) stellt einen Hochrisikotyp der Hauttumoren dar. Wir berichten über drei unterschiedliche Patienten mit rezidivierenden, fortgeschrittenen großen MTBCC: ein Tumor am Kapillitium und zwei an der Wange. Diese Patienten benötigen in der Regel ein komplexes chirurgisches Herangehen, um eine stabile und komplette Remission zu erreichen. Wir stellen individualisierte chirurgische Verfahren vor, die herangezogen werden können. Auf diese Weise wird es möglich, auch für ältere Patienten mit exponierten Knochen nach R0-Resektion eine wirksame und sichere Therapie zu ermöglichen. Eine interdisziplinäre Zusammenarbeit dient dem Wohl der Patienten.

Extrinsic apoptotic pathways, anti‐TNF‐alpha therapy and cancer progression: more and more clinical data support this critical relationship?

Anastasiya Atanasova Chokoeva*, Georgi Tchernev†, Torello Lotti‡§¶ & Uwe Wollina** *OnkodermaPoliclinic for Dermatology and Dermatologic Surgery, Gen Skobelev 26, Sofia, Bulgaria, †Policlinic for Dermatology and Venereology, Medical Faculty, University Hospital Lozenetz, Saint Kliment Ohridski University, Sofia, Bulgaria, ‡University of Rome “G. Marconi,” Rome, §Institute of Dermatology LifeCronos, Florence, Italy, ¶World Health Academy Foundation, Zurich (CH), •• and **Department of Dermatology and Allergology, Hospital Dresden-Friedrichstadt, Academic Teaching Hospital, Technical University of Dresden, Dresden, Germany

Disseminated superficial actinic porokeratosis (DSAP): significant improvement after local administration of calcipotriol/betamethasone gel?

SummaryPorokeratosis is defined as a disorder of keratinization characterized by one or more atrophic patches surrounded by a clinically and histologically distinctive hyperkeratotic ridgelike border called the cornoid lamella. Lesions are most commonly located on the sun-exposed sides of trunk and extremities, while exclusive facial involvement has been also reported. Despite that the exact risk of cutaneous malignancy developing in porokeratosis is unknown, it is confirmed by series of case reports, including squamous cell carcinoma, basal cell carcinoma and even melanoma. Although no definite cure exists, a number of treatment modalities, from topical medications to laser and light devices, have been reported with variable success. Although promising, reports of disseminated superficial actinic porokeratosis treated with vitamin D3 analogs are rare. No satisfactory enough effect is obtained also with calcipotriol monotherapy.We report a case of disseminated superficial actinic porokeratosis in a 80-year-old female patient, successfully treated with combination of topical calcipotriol/betamethasone gel. A significant improvement was noticed after 3 weeks; almost full resolution of the clinical symptoms without appearance of fresh lesions was achieved in the second month. To the best of our knowledge, this is the first report of successful treatment with full resolution in a short interval, by simultaneous application of vitamin D3 analog (calcipotriol) and corticosteroid (betamethasone), combined in a one commercial product as a gel for treatment of keratinocyte proliferation’s disorder such as psoriasis vulgaris. This promising new entity seems to be more effective, because of the simultaneous synergetic effects of the substations, namely the normalization of the proliferation of the keratinocytes by calcipotriol on one hand, and the reducing of the inflammation by the corticosteroid on another. Sun protection stays always mandatory, as well as the regular clinical observation, regarding the risk of malignant transformation.ZusammenfassungDie Porokeratose wird als Keratinisierungsstörung mit einer oder mehreren atrophen Läsionen mit klinisch und histologisch charakteristischer hyperkeratotischer Kornoidlamelle definiert. Die Herde finden sich meistens auf sonnenexponierter Haut an Rumpf und Extremitäten. Es wurde auch schon über eine ausschließliche Gesichtsbeteiligung berichtet. Das exakte Risiko der Entartung ist nicht bekannt, doch wurde in Fallserien über Plattenepithel-, Basalzellkarzinome und sogar Melanome berichtet. Obwohl keine komplette Abheilung möglich ist, existieren verschiedene Behandlungsoptionen – von topischer Therapie über Laser und Phototherapie mit variablem Therapieerfolg. Obwohl vielversprechend, sind Berichte zur Behandlung der disseminierten superfiziellen aktinischen Porokeratose mit Vitamin D3-Analoga spärlich. Mit einer Calcipotriol-Monotherapie werden jedoch keine zufriedenstellenden Resultate erzielt.Wir berichten über eine 80-jährige Patientin mit einer disseminierten superfiziellen aktinischen Porokeratose, die unter einer topischen kombinierten Therapie mit Calcipotriol/Betamethason-Gel eine deutliche Besserung zeigte. Eine erste signifikante Besserung war nach 3 Wochen zu sehen, gefolgt von einer fast vollständigen Abheilung im zweiten Behandlungsmonat, ohne dass neue Herde aufschossen. Soweit uns bekannt, ist dies der erste Bericht einer erfolgreichen Therapie mit (nahezu) vollständiger Abheilung nach kurzer simultaner Anwendung von Vitamin D3 Analogon (Calcipotriol) und Kortikosteroid (Betamethason) in Gelform. Das kommerzielle Kombinationspräparat ist für die Therapie hyperproliferativer Erkrankungen wie der Psoriasis zugelassen. Die Kombination der beiden Wirkstoffe scheint effektiver zu sein, da Synergien durch die Normalisierung der Hyperproliferation der Keratinozyten sowie die Reduktion der Entzündung auftreten. Sonnenschutz ist erforderlich, aber auch eine regelmäßige Nachsorge, wenn man das Risiko der neoplastischen Entartung bedenkt.

Penile Melanosis Associated with Lichen Sclerosus et Atrophicus: First Description in the Medical Literature

We present a 74-year-old male patient with 3-years history of visible discoloration of the glans penis, without subjective complaints. Histopathological examination after incision biopsy revealed a moderate increase in the number of melanocytes in the basal layer with irregular distribution, without melanocytic nests, melanophages in the superficial dermis, and subepidermal sclerosus. No cytologic atypia of melanocytes was detectable. The diagnosis of melanosis of the genitalia in association with lichen sclerosus was made. The importance of the presented cases implicated the unique clinical manifestation of penile melanosis, associated with lichen sclerosus of the penis in one hand, the essential differentiation between malignant melanoma via careful histological examination for diagnosis confirmation in other, in order to optimize the therapeutic behavior.

Primary Solitary Melanoma of the Lymphatic Nodes Or a Single Metastasis of Unknown Melanoma: Do We Need a New Staging System?

BACKGROUND: Malignant melanoma is a disease which has a cutaneous origin in 90% of the patients, but in rare cases, it could be discovered as secondary deposits with unknown primary site. Metastatic Malignant Melanoma occurs without a primary site in about 3% of all melanoma patients, and it could be divided into two main groups: metastatic lymph nodes’ involvement or non-lymph nodes disease. The lack of unified classification and staging system, provided by AJCC (2009), as well as the lack for curtain diagnostic and therapeutic protocol, prompt us to raise the question what is the right way to precede in cases of metastasis of the lymph nodes, without evidence of a primary tumour? CASE REPORT: We report a case of 67-years- old woman who presented in the dermatology clinic after a surgical removal of an enlarged lymph node in her left femoral area, verified histologically as a metastasis of melanoma. After a diagnostic refinement in the clinic, the diagnosis of metastasis of malignant melanoma was confirmed by histology revision. We use the presented case to create for the first time in the world literature a novel stereotype of thinking, which is also followed by a stereotype of clinical behaviour – gentle to the patient, but providing a certain amount of security and satisfaction for the medical staff. CONCLUSION: The affection of a single lymph node in the absence of a primary tumour should not automatically lead to the conclusion that it is a single metastasis, but rather a primary melanoma of the lymph nodes, in cases of a negative PET scan, for example. In these cases, the measuring of the tumour thickness should guide the further therapeutic behaviour and determine the approach.

Ventral Abdominal Hernia

A 63-year-old Caucasian female patient presented with redness of the both foot and lower legs, as well as edema of the left lower leg, accompanied by subjective complaints of burning. Fever was not reported. Well-circumscribed oval shaped tumor formation was revealed also on the abdominal wall, with hyperpigmented and depigmented areas on its ulcerated surface, measuring approximately 10/10cm in diameter, with soft-elastic texture on palpation. The lesion occurred in 2011, according to the patient’s history. No subjective complaints were reported in association. The performed ultrasonography revealed intestinal loops in the hernial sac, without incarceration. The diagnosis of ventral abdominal hernia without mechanical ileus was made. The patient was referred for planned surgical procedure, because of her refusal on this stage. The clinical manifestation of the tumor formation on the abdominal wall, required wide spectrum of differential diagnosis, including aneurysm of the abdominal aorta, abdominal tumor, subcutaneous tumor or metastasis or hernia. In the presented cases, the abdominal wall mass was a sporadic clinical finding in the framework of the total-body skin examination in patient with erysipelas. The lack of subjective symptoms, as well as the reported history for hysterectomy and previously abscessus were not enough indicative symptoms for the correct diagnosis. The diagnosis of non-complicated hernia was made via ultrasonography, while the clinical differentiation between hernia and other life-threatening conditions as aneurysms or tumor was not possible.

The “different face” of esophageal cancer: cutaneous manifestation of visceral malignancies

SummarySquamous cell carcinoma is the most common type of neoplasm of the esophagus with global incidence. Its early symptoms are often nonspecific as the disease could be detected only when metastases in various organs are already presented. Esophageal metastases present an extremely small part from all cutaneous metastases as the real incidence of cutaneous metastases due to cancer of the esophagus account for 0.5–9 % and only a small part of them are reported and rarely involve the facial region. Despite this, cutaneous metastases may be the first sign of malignancy of the esophagus, which immediately determined the worst prognosis and fatal outcome in these patients. Average survival prognosis at the time of diagnosis of esophageal carcinoma in stage IV is 4–6 months, while the survival-associated expectations in cases of associated skin lesions manifestation is 4 months. We present a rare case of esophagus carcinoma in advanced stage, presented with severe cutaneous metastasis in the face region, accompanied by heavy blood coughing and hematemesis, which led to fatal outcome in the reported patient. The incidence of cutaneous metastases due to this visceral malignancy is discussed, as we highlight the frequency of metastases as a first clinical sign in esophageal cancer. The mortality rate is high due to the advanced stage of progression of the disease or presented metastases spread at the time of diagnosis, while treatment-related mortality accounts 10.3 %.ZusammenfassungDas Plattenepithelkarzinom (SCC) ist der häufigste Typ maligner Ösophagustumoren mit weltweitem Vorkommen. Seine Frühsymptome sind häufig unspezifisch, und die Diagnose wird dann erst infolge Organmetastasierung gestellt. Kutane Metastasen des Ösophaguskarzinoms sind extrem selten und stellen nur 0,5–9 % aller Metastasen dieses Tumortyps dar, wobei das Gesicht sehr selten betroffen ist. Dennoch können Hautmetastasen das erste klinische Anzeichen einer Ösophagusneoplasie sein, was dann aber auf eine schlechte Prognose und einen fatalen Ausgang hindeutet. Im Stadium IV ist die Lebenserwartung auf 4–6 Monate beschränkt, ebenso wie bei Patienten mit einer Hautmetastasierung. Wir berichten über einen seltenen Fall eines fortgeschrittenen Ösophaguskarzinoms mit ausgedehnter fazialer Metastasierung, Hämatemesis und Hämoptysis, welches zum Tode führte. Die Inzidenz kutaner Metastasen bei viszeralen Neoplasien wird besprochen, und wir verweisen auf die Rolle kutaner Absiedlungen als Erstsymptom beim Ösophaguskarzinom hin. Die Mortalität ist hoch infolge der fortgeschrittenen Erkrankung, die therapiebedingte Mortalität beträgt 10,3 %.