Subungual Exostosis in a Young Soccer Player.

BACKGROUND: Subungual exostosis is a relatively uncommon, benign osteocartilaginous tumor of the distal phalanx of the toes or fingers in young adults, considered as a rare variant of osteochondroma. Differential diagnoses include subungual verruca (viral wart), pyogenic granuloma, osteochondroma, amelanotic subungual melanoma and glomus tumour. Misdiagnosis and total onychodystrophy frequently occur as a result of late treatment or inadequate treatment strategy. Dermoscopy could be a useful technique, involved in the diagnostic process, although X-ray examination and histopathology are mandatory for the diagnosis. CASE REPORT: We report a rare case of subungual exostosis of the great toe associated with repeated trauma of the nail bed. The lack of radiographic and histopathological examination could lead to misdiagnosis and inadequate treatment. Although completely benign, subungual exostosis should be considered in differential diagnosis of nail bed tumors in young adults, in order to avoid associated complications and unneeded aggressive surgical interventions. CONCLUSION: Complete excision of the lesion and delicate separation from the underlying nail bed structures results in total resolve of the problem, by providing the lowest risk of recurrences.

Advanced Pretibial Melanoma (APM): Clinicians Behaviour As Triggering Factor?

BACKGROUND: Pigmented lesions represent a broad spectrum of clinical conditions, both benign and malignant. The precise diagnosis is often a challenge, while the clinical diagnostic criteria could be misleading, as a result of the frequently atypical presentation of otherwise completely benign in nature lesions. The variety of therapeutic options for benign pigmented lesions including shave curettage, local laser destruction, electrocoagulation removal could sound enticingly both for the physician and patient, but they destroy the possibility for histological examination and provide a deceptively feeling of calm, that the problem is solved. If there is even a minimum chance for misdiagnosis, the risk could be a human life. Furthermore, a simple surgical excision could provide total resolution of the problem, with correct histological verification and further therapeutic measurements, if needed. CASE REPORT: We present a case of a patient, with advanced pretibial melanoma with multiple lung metastases, misdiagnosed as a seborrheic keratosis, treated with shave-curettage 6 months earlier, as we want to emphasize the importance of the correct therapeutic method in all cases with pigmented lesions with unknown origin, in order to minimize the risk of dramatic consequences of misdiagnosis of melanoma. So, we want to ask you- is this risk justified? CONCLUSION: So, we want to ask you - is this risk justified?

Neurofibromatosis Type 1 with Massive Ventricular Polyposis: First Report in the Medical Literature.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a multisystemic disorder with genetic background, characterised by specific cutaneous findings, skeletal dysplasias, and growth of both benign and malignant nervous system tumours. NF1 is caused by mutations in the NF1 gene, situated in chromosome 17q11.2, with an autosomal dominant pattern of inheritance and clinical manifestation of neurofibromas, malignant peripheral nerve sheath tumour, optic and non-optic nerve gliomas, congenital heart disease, cardiovascular and cerebrovascular disease and orthopaedic disorders. The incidence of gastrointestinal manifestations of NF1 is relatively low, compared to neurological disorders, presenting approximately in 5 to 25% of the patient, but later in life. CASE REPORT: We present a patient with NF1, ventricular polyposis and attentional disorders with cognitive phenotype, while both of her daughters also present with cutaneous manifestations of NF1. CONCLUSION: To the best of our knowledge, this is the first reported case of NF1 with ventricular polyposis as a gastrointestinal manifestation in the mother and NF1 with no signs of inner organ involvement in both of her daughters.

Para - And Intraurethral Penile Tumor - Like Condilomatosis.

Condyloma acuminata represents an epidermal manifestation, associated with the epidermotropic human papillomavirus (HPV). They have been reported as the most common sexually transmitted disease, with prevalence exceeding 50%, increased up to 4 times, within the last two decades, as the most common side of affection are the penis, vulva, vagina, cervix, perineum, and perianal area, with increased prevalence in young, sexually active individuals. Increased attention should be focused on lesions, caused by types, with moderate (33, 35, 39, 40, 43, 45, 51-56, 58) or high risk potential (types 16, 18) for malignant transformation, leading to further development of cancers of anus, vagina, vulva and penis, as well as cancers of the head and neck. A provident of coexistence of many of these types in the same patient could be seen in approximately 10-15% of patients, as the lack of adequate information on the oncogenic potential of many other types complicated the treatment and the further outcome. Although the variety of treatment options, genital condylomata acuminata still show high recurrent rate to destructive topical regiments, because of the activation of the viruses at some point, which emphasise the importance of virus- eradication, instead only of the topical destruction of the lesions. Despite decreasing the recurrent rate, the most important goal of immunisation is the reduction of the incidence of HPV-associated squamous cell carcinomas using either the quadrivalent (Silgard/Gardasil) or the bivalent (Cervarix) HPV (human papillomavirus) vaccine. We present a patient with periurethral condylomata acuminate, who refused performing of a biopsy for determining the virus type, as we want to emphasize the importance of the virus - treatment in all cases of genital warts, instead only of topical destruction of the lesions, not only because of the recurrence incidence rate, but also because of the well - known oncogenic potential of some HPV - types, as well as the unknown potential of various underestimated types, in contrast.

Another Case of Interdigital Located "Metastasing Hematoma"?

Interdigital spaces could be an area of affection of a various cutaneous conditions, most of them with benign origin. The spectrum of differential diagnosis of pigmented interdigital lesions with a recent occurrence is not so wide, in contrast. When considering pigmented lesions in the interdigital area, the most harmless differential diagnosis is a traumatic hematoma. But what would happen if we based our therapeutic behaviour or suspicious and unconfirmed harmless diagnosis, instead of considering the real life-threatening once with priority, if we kept in mind that acral lentiginous melanoma has rather an aggressive course and is the main cause of death in skin cancer patients? We present a case of misdiagnosed interdigital melanoma, treated as a hemangioma with curettage, with almost fatal consequences, in regard to uncontrolled tumor progression as a result of the wrong traumatic procedure in one hand, and the lack of adequate screening and follow up, leading to progress of the disease with lymph node metastasis and poor prognosis in general. We want to emphasise the importance of acral lentiginous melanoma with an unusual location in the differential diagnostic plan because, despite the early detection, early eradication with simple excision could save a life, or at least could provide a better prognosis.

Psoriasiform Dermatophytosis in a Bulgarian Child.

Although tinea capitis is the most common fungal infection in children, significant changes have been reported in its epidemiology worldwide, as a result from certain geographic, climatic and cultural differences in one hand, as well as the changes in its etiologic pattern. The clinical manifestation of the infection and the stage of inflammation vary from mild desquamation to severe suppurative indurated plaques in kerion - like the pattern, depending on the nature of the etiologic agent and the host-immune response. We report a case of tinea capitis profunda, caused by Trichophyton verrucosum in a 5 - year - old male patient, presented as a severe scalp and cutaneous desquamation, resembling histopathologically psoriasis, associated with severely indurated ringworm plaque in the temporal area. The performed histological examination revealed a psoriasiform pattern, without the typical Munro abscesses or Kogoj pustules. With the presented case, we want to emphasize the importance of the host's immune reaction to zoophilic dermatophytes, such as Trichophyton verrucosum, resulting in severe and often atypical clinical manifestation, as well as the possible "Id reaction", to avoid or minimise misdiagnosis and delayed therapy. The presented patient was treated with topical oleum acidy salicylic 10% and Terbinafine 125 mg daily with significant resolution of the complaints within the following two months.

A Patient with Multiple Keratinocytic Cancers (MKC): Uncommon Presentation in a Bulgarian Patient.

Keratinocyte skin cancers, including basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), are the most common cancer occurring in people with fair skin, worldwide. Despite all known triggers, several suggested contributors are still investigated. We will focus our attention on the personal history of previous cancers and radiation exposure as occupational risk factors, as in the presented case. We report a patient, with multiple BCCs, and subsequent occurrence of a SCC on photo-exposed area of the face, as we want to emphasize the importance of strict following up of these patients, regarding the risk for developing new tumors in short periods of time, no matter if the triggering exposure factor is known from the history, or not. Although keratinocytes tumours are associated with the low mortality rate, we focus the attention on the fact, that the history of non-melanoma skin cancer is associated with increased mortality.

Seborrheic Pemphigus, Antigen Mimicry and the Subsequent-Wrong Diagnostic and Therapeutic Approach?

It is well-established that drugs could be leading cause of occurrence of numerous diseases, including pemphigus, being either inducer or triggering factor of the autoimmunity. Despite medications, it should be kept in mind that chronic or acute infections are also capable of being a trigger in various types of cutaneous eruptions, including pemphigus. The rapidly obtained and uncompleted history for accompanied medication leads to general mistakes in the subsequent treatment approach, as the first step in such cases is discontinuation of the drug-inductor. The absence of this information guaranties the fail of the treatment. On the other hand, the lack of performed screening for chronic or acute hepatitis and tuberculosis is not the only mistake, regarding the high dosage of immunosuppressors that have been planned as regiment and the possible fatal effect on the infection's spread or exacerbation, but also because of the possible triggering ability of chronic or acute infection, which may play also a key role in the generation of antigen- or molecular- mimicry, as a potential source of antibodies reactive with various tissue antigens. It turns out that although the diagnosis of pemphigus in regular cases is usually not a challenge, the treatment occasionally could be, just because of a simple pitfall in anamnesis and screening, as in the presented case. Herein, we present a case of a patient with seborrheic pemphigus, which is strongly demonstrative for these statements, as we want to emphasise the importance of the first and the most powerful clinician's weapons - the patient's history and thorough examination.

Violet - Colored Inguinal Located Cutaneous Tumour?

Anaplastic large cell lymphoma (ALCL) represents an aggressive CD30 - positive T cell lymphoma, as it is the second most common T cell lymphoma and 2% to 5% of all non - Hodgkin lymphomas. The cutaneous involvement can be primary or secondary within systemic ALCL, resembling inflammatory and other neoplastic lesions both clinically and cytologically. Various pigmented cutaneous tumours with a different origin, cutaneous metastasis and B-cell lymphoma must be carefully considered in the differential diagnostic plan. While simple surgical excision is usually curative, with good prognosis, systemic involvement must also be excluded. We present a case of a patient, with clinically unspecific single violet nodular lesion, as the only clinical manifestation of ALCL. The diagnosis was confirmed histologically, as the surgical excision was enough therapeutic management, regarding the early disease stage. Further following up with the patient is mandatory, because of the high recurrence rate. We want to emphasise the diversity of clinical manifestation of ALCL, regarding the importance of its early diagnosis and treatment.

Disseminated Porokeratosis with Idiopathic Thrombocytopenia - Case Report and Literature Review of Porokeratosis and Related Disorders.

BACKGROUND: Porokeratosis is characterised by one or more atrophic patches surrounded by a distinctive peripheral keratotic ridge, typically found on sun-exposed areas, with several clinical variants and typical histological findings. Despite ultraviolet radiation, varies antibody - related autoimmune disease treated with systemic steroids and other immunosuppressive conditions such as chronic liver disease, HIV and organ transplantations have been implicated in its etiopathology. CASE REPORT: We present a case of porokeratosis, associated with idiopathic thrombocytopenia in 74 - year old, otherwise healthy male patient, as we discuss the previously reported associated disorders. CONCLUSION: Regarding all of the polymorphism of clinical presentation, associated disorders and treatment responses, we could conclude that disseminated porokeratosis is still an unknown well disorder, which will continue to surprise the physicians in future. The screening and follow up of the patients is mandatory in all cases because as we see, porokeratosis has multiple unexpected faces, which require circumstantial clinical and paraclinical behaviour.

Small Dysplastic Congenital Melanocytic Nevi in Childhood as Possible Melanoma Imitators.

Small pigmented lesions in children can represent a significant diagnostic challenge. If the diagnostic features and therapeutic approach are relatively well established in large and giant nevi, there is still much controversy regarding small and intermediate-sized congenital pigmented lesions that can lead to significant diagnostic challenges, both clinically and dermoscopically, and consequently to difficulty in defining the optimal approach in such cases. Although dermoscopy can be useful in the diagnosis of pigmented lesions, the diversity of clinical and dermoscopic features of pigmented nevi in children usually hinder the differentiation between them and melanoma. Histological findings after resection often show surprising results that do not correspond either to the clinical nor the dermoscopic features. With the present case, we want to emphasise the variable natural behaviour of melanocytic lesions in children, which sometimes leads to unnecessary surgical excisions, which should be avoided in pediatric patients.

Multiple Primary Acral Lentiginous Melanomas (MPALM).

We present a rare case of a patient with multiple primary acral lentiginous melanomas of the foot. We would like to highlight the importance of whole-skin examination in all patients, even by the general practitioners, aiming the maximal early detection of acral lentiginous melanomas, considering their rapid progression, early metastatic spread and extremely poor prognosis. It can be extrapolated from current literature; however, that appropriate management of these patients, including staging work and surgical intervention, is to be determined by the individual characteristics of the melanoma and the patient's concomitant risk factors, if any.

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.

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.

Khan Krum - "The Fearsome": A Noble Bulgarian with Iris-Fibroma?

A 40-year-old Caucasian, the otherwise healthy male patient, presented with 3-years history of a prominent tumor-like formation, located on his back, without subjective complaints. Initially, the lesion was 1-2 cm in size but gradually increased to a visible-pronounced tumor-like formation. No other dermatologic diseases, neither other known abnormalities were reported for the medical and family history. Softly-elastic on palpation tumor-like formation was established in the left infrascapular area of the back, measuring 8/9 cm in diameter. The lesion was clinically suspected for lipoma or another soft-tissue tumour with benign nature, so the patient underwent planned surgical excision under local anaesthesia. A tumour was excited with deep elliptical excision and furthered for histological evaluation, while the wound edges were sutured with single cutaneous stitches. Additionally, a large black-colored tattoo of the Bulgarian historical noble Khan Krum was also observed within the clinical examination, covering almost the whole upper 1/3 part of the patient's back Within the careful clinical observation of the tattoo, we noticed a fibroma-like lesion, affecting the noble's right iris. Fibromas are common benign tumours in Caucasians, composed of fibrous or connective tissue. They can be seen in all organs from mesenchymal tissue, in varies size. Eyelids are often commonly affected in elderly. Although very commonly seen in every day dermatological practice, fibromas have been never reported, located in the iris. In the presented case, a small "iris-fibroma", hidden within a black-colored tattoo was diagnosed in a patient with lipoma, as a sporadic clinical finding.

Dermatologic Surgery and Dermatologic Oncology as an Essential Part of the Modern Dermatology in Bulgaria.

Dermatosurgery and dermatooncology are an integral part of dermatology as a speciality, and this postulate is strictly respected in a high percentage of European dermatological units. Due to the fact that a number of other specialties interweave with the subject of therapy - the surgical treatment of the patient with skin tumors, the positioning of dermatosurgery as part of dermatology is generally controversial (according to some), and at the same time is often the subject of a number of debates and conflicts. These include maxillofacial surgeons, plastic surgeons, regenerative and reconstructive surgeons, surgical and medical oncologist, etc. The advantages of these specialities are mainly based on good medical practice and good surgical techniques that are applied. In contrast, their disadvantages are based on the lack of good awareness of the initial surgical approach as well as the need for time-adjusted and accurately performed additional surgical interventions which should befurthermore careful scheduled with the relevant oncology units. Losing this thread, in practice, it turns out that we are losing the patients themselves or, looking laconically, we are working with reduced efficiency and effectiveness. Although for the last 15 years the positions of these sub-sectors in Bulgaria had been underdeveloped, a certain ascent has been observed nowadays or from a couple of years ago. This advance is undoubtedly due to the influence of the German Dermatological School, presented by Prof. Dr. Uwe Wollina, Head of Department of Dermatology, Venereology and Allergology in Dresden, Germany, as well as due to other respected representative of the Italian Dermatological School - in the face of Prof. Dr. Torello Lotti, Head of the Dermatology Unit at G Marconi University of Rome, Italy.

Genital Bowen's Disease in a Bulgarian Patient: Complete Remission after Surgical Approach.

A 60-year-old male patient presented with complaints of persistent red to a brown-colored plaque on his scrotum, with duration of approximately three years. The patient had been treated with oral and topical antifungals for inguinal tinea for several months and after that with topical corticosteroids for eczema for several more months. None of the regimens achieved any therapeutic effect. The histopathological evaluation revealed the presence of atypical keratinocytes in all layers of the epidermis with the altered epidermal pattern, spread parabasal mitotic activity, without secondary satellites, multiple dyskeratotic cells and multinucleated cells. The diagnosis of an intraepithelial non-invasive squamous cell carcinoma, associated with koilocytic dysplasia and hyperplasia was made, meeting the criteria for Bowen disease. An elliptic surgical excision of the lesion was made, while the defect was closed with single stitches, with excellent therapeutic and aesthetic result. First described by John T. Bowen in 1912, Bowen disease (BD) represents a squamous cell carcinoma (SCC) in situ with the potential for significant lateral spread. Treatment options include the application of topical 5-flurorouracil cream - useful in non-hairy areas, imiquimod cream or destructive methods such as radiation, curettage, cryotherapy, laser ablation and photodynamic therapy, especially useful in nail bed involvement. Despite the early lesions, surgical excision is the preferred treatment option, regarding the potential malignant transformation risk.

Giant Congenital Melanocytic Nevus (GCMN) - A New Hope for Targeted Therapy?

We present a 6-month-old male patient, who was consulted with dermatologist by his parents, because of a pigmented lesion, present since birth, covering almost the all skin of the back and buttocks. A sharply bordered, unequally coloured congenital pigmented nevus, measuring approximately 21 cm in diameter was observed in the whole body skin examination. The lesion was affecting the lower 2/3 of the skin of the back and the top half of the gluteus area, extending to the lateral part of the tors, forward the abdomen and the upper lateral part of the hips, composed by multiple darker-pigmented nests and several lighter areas, with single depigmented zones, hairy surface, irregularly infiltrated on palpation. Congenital melanocytic nevi are presented in approximately 1% of newborns, while giant congenital melanocytic nevi (GCMN) are the most uncommon subtype of them; with occurrence rate 1 in 50,000 births. They affect 2% of a total body surface or presenting in a diameter larger than 20 cm in older children. Although not common, the possible malignant transformation remains one of the most important considerations related to them, as the related lifetime risk of melanoma is 4% to 10%. Treatment recommendations include non-surgical methods as dermabrasion only within the first two weeks of life, for prevention the possible melanocytic deeper migration, while serial surgical excisions or tissue expanders could be useful treatment tool even in later stages. Nevertheless, cosmetic result is not always satisfactory, and the risk of malignant changes remains, in cases of previous melanocytic migration in deeper layer. Recent article suggests the potential role in the treatment of GCMN with NRAS inhibitor trametinib, approved for treatment of advanced melanoma, associated with underlying NRAS mutations. Although promising, the drug could be useful in paediatric patients, only with associated NRAS gene mutation. It is still unclear whether it could be helpful, independent of the NRAS status.

New Safety Margins for Melanoma Surgery: Nice Possibility for Drinking of "Just That Cup of Coffee"?

BACKGROUND: The American Joint Committee on Cancer (AJCC's) skin melanoma surgical treatment recommendations from 2011 are characterised by a prima facie "freedom of choice" regarding how extensive should be the excisions for melanomas with tumour thickness up to 2 mm and melanoma in situ. It is unclear why the recommended surgical security margins vary between 0.5 and 1 cm for melanoma in situ, whereas for melanomas with a tumour thickness of up to 1.99 mm, the range of variation is also between 1 and 2 cm, without specifying when the surgical field should be broader and, narrower, accordingly. This "uncertainty or lack of intent" of the guilders often leads to the same surgical approach to melanomas at different stages, or to a different approach in cases of melanomas at the same stage, in contrast. Therefore, this should be defined as wrong, logically. CASE PRESENTATION: We present 3 patients with cutaneous melanomas, treated with similar fields of surgical security. Current issues, generated within the framework of melanoma's surgery guided by the recommendations of the AJCC are also discussed. A new surgical approach in patients with melanoma is recommended, discussed for the first time in world literature. We hypothesize that the introduction of a certain recommendations for a 2 cm surgical field in all directions during the initial excision, combined with the parallel performance of a sentinel lymph node biopsy, will lead in fact to several important advantages: 1) avoiding of the secondary excision in at least 70% - 90% of the patients (depending on the tumor thickness), 2) minimizing the risk of lymphatic effusion change and misinterpretation of the sentinel lymph node biopsy's results in patients with secondary excision; 3) optimization of the surgical team's work; 4) minimizing the possibility of unprepared/uninformed personnel to take part in decisions for treating a specific disease such as skin melanoma, 4) facilitating the appropriate patients' group selection at the appropriate stage when involving them in different studies, leading to equal leveling of the initial positions. CONCLUSION: Whether the proposed approach will be subjected to a detailed discussion of AJCC's expert's remains currently unclear.