Eruptive Basaliomas: "Why we have to Perform Surgery?" Or Said Otherwise: "Catch The Metatypical!"

BACKGROUND: Keratinocyte cancers are malignant diseases with a broad incidence of spread which tends to increase during the last couple of decades. The solar radiation plays a dominant role in the occurrence of BCC, but certain genetic phenotypes appear to be risky from an etiological point of view. Metatypical basal cell carcinoma (MTBCC) is a rare variant of BCC which combines the clinical and histological characteristics of BCC and SCC. Clinically they are indistinguishable from the conventional BCC, and only the histological examination can differentiate them. The MTBCC is a histological subtype which is considered more aggressive due to its ability to produce local recurrences or distant metastases. CASE REPORT: We present a 44-year old patient with multiple BCCs disseminated on the face and body. The biopsy established mixed type histology: three metatypical and four solid BCCs. The lesions were removed via elliptical excision with a field of operational security of 0.5 cm in all directions. CONCLUSIONS: The eruptive (multiple) BCCs are a challenge about the choice of a therapy option. This is because clinically completely identical tumours show different histopathological characteristics, namely those with a tendency to metastasise. Having in mind one of the hypotheses of metatypical BCC emergence - the improper or inadequate radiotherapy (as a choice of therapy) could trigger the transition of a conventional tumour to a metastasising one, the surgical treatment appears to be the most secure treatment method.

Primary Cutaneous CD30+/ALK- ALCL with Transition into sALCL: Favourable Response after Systemic Administration with Brentuximab Vedotin! Unique Presentation in a Bulgarian Patient!

BACKGROUND: Modern drugs could sometimes be a good solution even to problematic patients. The cutaneous and systemic forms of the CD30 positive anaplastic large T-cell lymphoma could often be described as a suitable target for therapy with Brentuximab vedotin. CASE REPORT: We present the first case of a Bulgarian patient with a histologically confirmed primary cutaneous T-cell CD30+/ALK- large anaplastic cell lymphoma-cALCL (therapeutically resistant to therapy with Methotrexate, radiation therapy and systemic corticosteroid therapy) who was successfully treated with Brentuximab vedotin. In several years, the patient has developed a comparatively fast skin progression as well as an initial systemic one which impacts inguinal and mediastinal nodes. After the implementation of 4 therapy cycles with Brentuximab vedotin, complete regression of the described by previous hospitalisations lymph nodes as well as 80% reduction of the cutaneous and subcutaneous located tumour formations were observed. CONCLUSION: The therapy of CD30+/ALK- anaplastic large T-cell lymphoma is a significant challenge for oncologists and dermatologists because it requires maximally efficient and minimally traumatic treatment in parallel. Therapy with Brentuximab is a new direction which shows extremely good clinical results and can be applied to the cutaneous as well as to the systemic form of anaplastic large-cell CD30 positive lymphoma. The key element by treatment with Brentuximab is suppression of the CD30- expression which, in turn, could be the cause of relapses. On that ground, patients with these lymphomas should be strictly monitored.

Comparative Analysis of the Applicability of Island Flap in Primary and Recurrent Basal Cell Carcinomas of Similar Localization.

BACKGROUND: Basal cell carcinoma belongs to non-melanoma skin cancers and is the most prevalent neoplasia that shows a tendency to increase over the last few decades. It occurs most often in skin areas exposed to sunlight. It is characterised by slow progression, low tendency to metastasising and good prognosis when the right choice of treatment has been made. The difficulty in the treatment of basal cell carcinomas is determined by their localisation and puts to the test the aesthetic potential of dermatosurgeons. Complete surgical excision is the standard approach in most uncomplicated cases. In relapsing basal cell carcinoma or carcinoma with aggressive or unfavourable histopathological characteristics, the clinician faces the dilemma of identifying the most appropriate method of treatment. To find the decision, help comes from the individualisation of each case and the related risk factors. CASE REPORT: Two cases of basal cell carcinoma of similar localisation are presented, where the carcinomas are removed using island flaps. In spite of the desire to observe the recommended field of surgical security (by the desire for the ultimate esthetic effect for the patient), one of the tumours was not completely removed, and as an alternative, reoperation was proposed using Mohs micrographic surgery (MMS). CONCLUSION: The choice of a surgical technique, which would guarantee a better outcome and could be applied depending on the individual risk factor in each patient, is discussed.

Nevus Blue as a Sporadic Finding in a Patient with a Blue Toe?

BACKGROUND: Blue nevus is an interesting finding, which aetiology and risk of locoregional and distant metastasis have not yet been fully clarified. It may be inherited or acquired, with sporadic cases usually presented as solitary lesions. It is often localised in the area of the head and less often on the arms, legs or trunk. Blue nevi are formations with relatively low but still possible potential for switching to melanoma. CASE REPORT: The patient we described was hospitalised for pronounced cyanosis of the small toe of the right foot, accompanied by painful symptoms at rest and pain symptoms for a few weeks. Using inpatient paraclinical and instrumental tests, the patient was diagnosed with cholesterol microembolism. During the dermatological examination, blue nevus on the contralaterally localised limb was also diagnosed as a sporadic finding. According to the patient's medical history, the finding had existed for many years, but in the last few months, the patient has observed growth and progression in the peripheral zone of the nevus without any additional clinical symptoms. CONCLUSION: Due to the risk of progression to melanoma, the lesion was removed by radical excision, and the defect was closed by tissue advancement flap.

Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient!

BACKGROUND: The Stewart-Treves syndrome with localisation in the region of the lower extremities is not something unusual as clinical pathology, but the clinical diagnostics is rather difficult, and it can be further complicated maximally because of: the similar locoregional findings in patients with other cutaneous malignancies. CASE REPORT: Presented is a rare form of an epithelioid variant of the Stewart Treves syndrome in a woman, aged 81, localised in the region of the lower leg and significantly advanced only for 2 months. The diagnosis was confirmed histologically and immunohistochemically. Amputation of the affected extremity was planned. Discussed are important etiopathogenetic aspects regarding the approach in patients with lymphedema and possibility for development of the Stewart Treves syndrome. CONCLUSION: Analyzing the evidence from the literature worldwide, we concluded that perhaps the only reliable (to some extent) therapeutic option in patients with Stewart Treves Syndrome is 1) the early diagnostics and 2) the following inevitable radical excision or amputation with the maximal field of surgical security in the proximal direction.

Interdigital melanoma simultaneously affecting two neighboring interdigital spaces : First description in the medical literature.

BACKGROUND: Interdigital melanoma, as a subtype of acral lentiginous melanoma, is relatively uncommon in the Caucasian population. It frequently goes unrecognized for a prolonged period of time prior to diagnosis, due to its asymptomatic nature and variable clinical appearance. METHODS: We report the case of a 67-year-old Caucasian woman who presented with interdigital malignant melanoma affecting two neighboring interdigital spaces. It had evolved over a period of more than 15 years and had been initially misdiagnosed as tinea pedis due to the macerated appearance of the lesion and a positive mycologic examination. RESULTS: We highlight the striking involvement of two adjacent interdigital spaces and the neighboring area of the sole of the foot by the tumor. The melanoma was staged as IIIC, with pathologic grading T4bN2bM CONCLUSIONS: The involvement of two adjacent interdigital spaces is unusual and, to our knowledge, has not been previously highlighted in the medical literature. It may be explained, in part, by the longstanding nature of the lesion in our patient.

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.

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.

Pigmented Paraaxillary Located "Complex" Basal Cell Carcinoma Imitating clinically irritated Melanocytic Lesion - Succesfull Surgical Approach in Bulgarian Patient.

BACKGROUND: Basal cell carcinoma (BCC) is the most frequently encountered neoplasm worldwide. While nodular BCC is the most frequent clinical subtype, other forms of BCC, such as superficial, cystic, morpheiform, infiltrative, and pigmented may also be encountered. CASE PRESENTATION: We present the case of a 67-year-old male with a relatively well-defined infiltrative, pigmented plaque with multiple colours and peripheral growth situated in the right axillary region. The histopathologic examination performed after complete surgical excision of the tumour revealed a complex pigmented BCC with macronodular, fibroepithelioma-like, cystic, focally infiltrative and basosquamous features. CONCLUSION: Uncommon locations of BCCs in sun-protected areas such as the axillary region require a higher degree of suspicion for diagnosis. The complex histology of the presented case, including subtypes with differing biologic attributes, emphasises the importance of histopathological examination in the diagnosis and therapeutic management of BCC.

Basal Cell Carcinoma Surgery: Simple Undermining Approach in Two Patients with Different Tumour Locations.

Basal cell carcinoma (BCC) is the most common human malignancy, accounting for the majority of all non-melanoma skin cancers (NMSC). In the past several decades the worldwide incidence of BCC has constantly been increasing. Even though it is a slow growing tumour that, left untreated, rarely metastasizes, it has a distinctive invasive growth pattern, posing a considerable risk for local invasion and destruction of underlying tissues, such as muscle, cartilage, bone or vital structures. Advanced BCCs include such locally invasive or metastatic tumours. Complete surgical excision is the standard therapy for most uncomplicated BCC cases with good prognosis and cure rates. Treatment of advanced forms of BCCs poses significant therapeutic challenges, most often requiring complicated surgery, radiotherapy, and/or targeted therapies directed towards the sonic hedgehog signalling pathway (SHH). We present two cases of large BCCs located on the scalp and posterior thorax, which underwent surgical excision with clear margins, followed by reconstruction of the defect after extensive undermining of the skin.

Interstitial Granulomatous Dermatitis (IGD).

We report the case of a 42 years old male patient suffering from skin changes, which appeared in the last 7-8 years. Two biopsies were performed during the evolution of the lesion. Both showed similar findings that consisted in a busy dermis with interstitial, superficial and deep infiltrates of lymphocytes and histiocytes dispersed among collagen bundles, with variable numbers of neutrophils scattered throughout. Some histiocytes were clustered in poorly formed granuloma that included rare giant cells, with discrete Palisades and piecemeal collagen degeneration, but without mucin deposition or frank necrobiosis of collagen. The clinical and histologic findings were supportive for interstitial granulomatous dermatitis. Interstitial granulomatous dermatitis (IGD) is a poorly understood entity that was regarded by many as belonging to the same spectrum of disease or even synonym with palisaded and neutrophilic granulomatous dermatitis (PNGD). Although IGD and PNGD were usually related to connective tissue disease, mostly rheumatoid arthritis, some patients with typical histologic findings of IGD never develop autoimmune disorders, but they have different underlying conditions, such as metabolic diseases, lymphoproliferative disorders or other malignant tumours. These observations indicate that IGD and PNGD are different disorders with similar manifestations.

Verrucous-Keratotic Malignant Melanoma (VKMM).

We report a patient with a verrucous keratotic variant of melanoma visiting the policlinic of Medical Institute of Ministry of Interior (MVR-Sofia), Department of Dermatology and Dermatologic surgery, with a keratotic verrucous lesion, located on the right thigh, partially deeply pigmented at upper right quadrant. The lesion had appeared three years ago before her presentation in the policlinic, and it had gradually enlarged and become darker in the last twelve months. The surface of the lesion was covered with thick hyperkeratotic lobules. The histologic evaluation revealed verrucous melanoma with a tumour thickness of 3 mm and Clark Level IV and focal ulceration. The tumour was staged as stage IIB (T3bN0M0). Sentinel lymph node biopsy was planned. Verrucous-keratotic forms of malignant melanoma occur more commonly in women and favour the extremities, but may be found on any anatomic site. Seventy-one percent of this melanoma type are situated on the upper and lower extremities. Although two-thirds of these neoplasms can be can be histologically graded according to the classification of Clark, one-third of these melanomas with marked verrucous hyperplasia and hyperkeratosis of the epidermis do not fit into his classification. Histological classification of patients with a verrucous keratotic type of melanoma may sometimes be extremely difficult. The marked papilliferous architecture of these lesions made an assessment of Breslow depth difficult. The presented case highlights the clinical existence and features of such benign-looking melanomas. It is therefore important for surgical pathologists to recognise this unusual variant of malignant melanoma, as it may be confused both clinically and pathologically with benign lesions.

Lichen Simplex Chronicus as an Essential Part of the Dermatologic Masquerade.

A 48 years old female patient had been suffering from the lesions presented for four years. They have started as small, pruritic patches which had been mechanically irritated and grew up in time. The patient had no associated comorbidities or allergies, and she was not under any medication. On physical examination, she presented one erythematous, exudative plaque, with dimensions of 2.5/4 cm, located on the proximal phalanx and interphalangeal articulation of the left thumb. All histopathological features were consistent with the diagnosis of lichen simplex chronicus. Some lesions of lichen simplex chronicus exhibit signs of pseudocarcinomatous, infundibular and sometimes eccrine ductal proliferation of keratinocytes. Although the pseudoinfiltrative aspect of the epithelial proliferation and its pronounced degree might mimic a well-differentiated lesion of squamous cell carcinoma, a lack of cellular atypia and atypical mitotic figures are features that do not support this diagnosis. On the other hand, long lasting lesions of lichen simplex chronicus may lead to alterations in the processes of keratinocyte proliferation and differentiation and eventually give rise to malignant transformation. The best treatment management is a psychodermatological approach, a combination of skin care with psychotherapy, in order to prevent relapses.

Acquired Ulcero-Mutilating Bilateral Acro-Osteopathy (Bureau-Barrière Syndrome).

We present a 35-year-old male patient with Bureau-Barrière syndrome. Bureau-Barrière syndrome is an ulcero-mutilating acropathy almost invariably associated with excessive alcohol intake. It presents with a triad of trophic skin changes with recurrent ulcerations, bone lesions and nerve damage. The clinical presentation includes chronic painless plantar ulcerations with periulcerous hyperkeratosis, hyperhidrosis, livedoid skin colour, nail dystrophy, widening and infiltration of the toes and common interdigital mycoses. Other non-specific skin changes related to the alcohol consumption are commonly observed as well. The condition affects mainly middle-aged men suffering from alcoholism. Often a bilateral location at the lower limb of male alcoholics has been described, as in our patient. Successful treatment of the Bureau-Barrière syndrome requires an interdisciplinary approach. Cessation of alcohol intake and smoking is of paramount importance.

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

Porokeratosis 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.