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.

Neglected Under Lip Cancer.

The lip represents an anatomical area of the interface between the skin and oral mucosa. It is, therefore, not entirely surprising that SCC of the lip exhibits mixed features between cutaneous and oral mucosal SCC, namely regarding risk factors and biological behaviour. The main risk factors for lip SCC include ultraviolet radiation exposure, low phototype, tobacco and alcohol use, and immunosuppression. Lip SCC usually presents clinically as a nodule or a tumour with a keratotic surface that commonly ulcerates. There is often a background of actinic cheilitis. The particularly exuberant presentation of our case can most probably be explained by the long evolution of a tumour before the patient reached medical attention. Patients with regional lymph node metastasis are usually offered regional lymph node dissection (usually of the neck). Radiotherapy and/or chemotherapy may be used in advanced cases, particularly in unresectable tumours, tumours with high-risk features and metastatic disease. The large size of a tumour in our case, most probably due to its long evolution, highlights the importance of timely diagnosis to avoid such extreme presentations and the consequent need for more aggressive treatment.

Sarcoidosis in A. C. Milan (1899)?

The pathogenesis, diagnosis and therapy of sarcoidosis as an autonomous disease are subjects of spirited discussions, which haven't found definitive conclusion yet. Distinguishing between sarcoidosis and sarcoid-like reactions (sarcoid - type granulomas) is not currently a medical "gold standard" and is not implemented in clinical practice. This leads to 1) misinterpretation of numerous available data; 2) difficulty in the interpretation of other unverified data, which is often followed by 3) inappropriate or inadequate therapeutic approach. Similarly to many other diseases, in sarcoidosis and sarcoid - types of reactions the concept of personalised approach and therapy should also be introduced. This methodology of clinical guidance is difficult, complex and not always achievable in the current medical status and relations (doctor-patient relationship; financial factor; time factor). It is appropriate to note that in some cases the guidelines or the so-called standards are neglected or not possible to put into practice with the aim of better therapeutic practices and strategies, as well as the achievement of optimal final clinical results (especially in patients with sarcoid granulomas). The sarcoid granuloma, even when it is sterile, should not be considered as the equivalent of sarcoidosis, i.e., sarcoidosis as an autonomous disease. Sure enough, exactly because of this fact, the personalised approach should not be an exception, but it has to gradually become a rule in medical practice. When clinical decisions are conformed to some of the latest modern concepts, officialised in the international databases, often the achieved results can be much better. We present a patient with a tattoo of AC Milan (1899) on his right arm, who subsequently developed localised sterile sarcoid granulomas in the area of the tattoo. Later the process became generalised on his whole body's skin, lungs and lymph nodes. It is unclear for the moment whether this condition should be interpreted as sarcoidosis as an autonomous disease or, instead, as a sarcoidal type of reaction with subsequent generalisation due to cross-reactivity against antigens present in other tissues with similarities to the exogenous pigments. Following the modern concepts regarding the pathogenesis of these two conditions, we introduced, in this case, an innovative, non-standard approach: 1) systemic and local immunosuppressive therapy, combined with 2) recommеndation for immediate surgical excision of the tattoo to remove the possible trigger of molecular and antigen mimicry.

Sweet's Syndrome (SS) in the Course of Acute Myeloid Leukaemia (AML).

Firstly described by Robert Douglas Sweet in 1964, febrile neutrophilic dermatosis is a disabling, not only cutaneous disorder, clinically characterised by fever and painful erythematous nodules, with a typical background of neutrophilia. Sweet's syndrome (SS) is a chronic inflammatory reactive disorder of unknown cause and incompletely established pathogenesis, although an interplay between genetic and environmental factors, including infections, is likely to occur. A significant part of cases has been demonstrated to be linked with malignancies, especially in the hematologic setting. Because of the underlying disease and related therapeutic measures, SS may present atypical clinical course, whereas the response to treatment is strictly dependent on the concurrent hematologic disease. Herein we describe a case of a lady who had a refractory form of SS, resulted in a paraneoplastic cutaneous disease, and AML. Surprisingly, clinical remission of SS followed cytotoxic chemotherapy while hematologic disorder obtained a further complete response.

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.

Locally Advanced Basal Cell Carcinoma with Intraocular Invasion.

We present a 103 - year - old patient, with duration of complaints of about ten years. The initial complaint had been presented as a small nodule, located on the eyebrow, which subsequently ulcerated and encompassed larger regions of the upper and lower eyelids. For the past three years, the patient also had complaints of a worsening of his vision, without seeking for medical help. Within the dermatological examination, an intraocular and periocular localised tumour was established, characterised by a raised peripheral edge and central ulceration. More careful examination revealed that the bulb was fully consumed. The patient refused further diagnosis and treatment. Advanced basal cell carcinomas with intraocular invasion are rare in general. If the patient refuses surgery, radiotherapy and systemic therapy with modern medications such as Vismodegib or Sonidegib are available as treatment options.

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.

Peri - and Intraocular Mutilating Advanced Squamous Cell Carcinoma: "Monsters Inside Your Body"?

Periocular malignancies represent between 5% and 10% of all types of skin cancers. The incidence of eyelid (but also the periocular located) malignancies seems to differ in distribution across the continents. The incidence of eyelid tumours (but also the periocular located tumours) in a predominantly white population determined that BCC is the most common malignant periocular eyelid tumour in whites. This finding has been replicated consistently throughout the literature, with BCC representing 85-95% of all eyelid malignancies, SCC representing 3.4 - 12.6%, Seb Ca representing 0.6 - 10.2%, and both melanoma and Merkel cell carcinoma representing less than 1%. Most periocular skin cancers are associated with ultraviolet radiation (UVR) exposure. Ultraviolet radiation causes local immune suppression, which, coupled with DNA abnormalities in tumour suppressor genes and oncogenes, leads to the development of skin cancers. We are presenting a 62 - year - old patient with a small nodule about 2 cm away from the lower lid of his left eye. A tumour was surgically treated. Several years later there was a tumour relapse, treated with radiotherapy and subsequent chemotherapy with Endoxan and Cisplatin. After the second relapse, he was treated surgically in general anaesthesia by orbital exenteration, removal of the orbital floor and resection of zygomatic bone and the maxillary sinus. A couple of months later, he developed a tumour relapse in the scars and the area of a primary tumour with tumour progression. A possible therapy with Cetuximab or radiation therapy was discussed as a possible treatment option.

Mucoepidermoid Carcinoma (MEC) of Parotid Gland with Massive Cutaneous Involvement: Bilateral Pedicle Advancement Flap (U - Plasty) As Adequate Surgical Approach.

Neoplasms of the major and minor salivary glands are morphologically and a clinically diverse group of neoplasms which lead the clinician to diagnostic and management challenges. This article aims to report a case of mucoepidermoid carcinoma in 83 - year - old woman who presented in the dermatology clinic with a tumour mass in the left auricular area. The patient complained of pain and abnormal bleeding of the mass. The lesion was examined, and surgical treatment was performed. A tumour was extirpated, and partial resection of both the parotid gland and the sternocleidomastoid muscle was done. Lymphatic dissection was performed. Post recovery was uneventful with no functional defects and abnormalities. The pathohistological result confirmed the diagnosis of mucoepidermoid carcinoma of the parotid gland with massive infiltration of the skin and the subcutaneous tissue. Lymph nodules with total metastasis of mucoepidermoid carcinoma and capsular invasion were additionally presented. Postoperative radiation therapy was planned.

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.

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.

Dermatosurgery Rounds - The Island SKIN Infraorbital Flap.

The main objective in dermatologic surgery is complete excision of the tumour while achieving the best possible functional and cosmetic outcome. Also we must take into account age, sex, and tumour size and site. We should also consider the patient's expectations, the preservation of the different cosmetic units, and the final cosmetic outcome. Various reconstructive methods ranging from secondary healing to free flap applications are usedfor the reconstruction of perinasal or facial defects caused by trauma or tumour surgery. Herein, we describe the nasal infraorbital island skin flap for the reconstruction in a patient with basal cell carcinoma. No complications were observed in operation field. The infraorbital island skin flap which we describe for the perinasal area reconstruction is a safe, easily performed and versatile flap. The multidimensional use of this flap together with a relatively easy reconstruction plan and surgical procedure would be effective in flap choice.

The "different face" of esophageal cancer: cutaneous manifestation of visceral malignancies.

Squamous 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 %.