The spectrum of benign dermal dendritic melanocytic proliferations.

Dermal melanocytoses (DMs) comprise a heterogeneous group of benign lesions, located on skin and mucous membranes, characterized by dendritic melanocytes in the dermis. Although they share common histopathological features, some variants may present only as bluish or grey patches, some only as papules/nodules/plaques and others may show combination of all of these lesions. Despite the fact that blue naevus (BN) is typically characterized with papulonodular lesions, its variants may show all of the aforementioned presentations. Mongolian spot, naevus of Ota and naevus of Ito are patchy DMs distinguished by their specific localizations. Apart from these classical forms, many atypical variants without unique clinicopathological characteristics have been described in the literature making the nomenclature of DMs more complicated. However, congenital dermal melanocytosis and acquired dermal melanocytosis seem to be crucial umbrella terms that encompass all patchy DMs in atypical locations. Papules or subcutaneous nodules on patchy lesions and association of epidermal pigmentation presenting as brownish patches may be encountered as rare features of DMs. On the other hand, delayed-onset subcutaneous nodules may be typical presentations of melanoma in patchy DMs; therefore, they deserve special attention. Large plaque-type BN with subcutaneous cellular nodules is a newly described entity, harbouring clinical features of various DMs together and has a high risk of melanoma. The whole spectrum of dermal dendritic melanocytic proliferations is discussed including novelties and controversial issues.

Cutaneous atypical papular CD8+ lymphoproliferative disorder at acral sites in a renal transplant patient.

A 20-year-old woman presented with a 2-month history of an acute symmetrical eruption, manifesting as asymptomatic ill-defined erythematous macules and hyperkeratotic papules on the palms. The patient was a renal transplant recipient, and the lesions had developed 2 months post-transplantation. Histologically, the eruption shared features of a reactive inflammatory condition called papular eruption of atypical CD8+ lymphocytes as well as primary cutaneous acral CD8+ T-cell lymphoma (a provisional indolent entity in the new World Health Organisation classification of lymphoid neoplasms, 2016). The latter disorder has been described to occur at acral sites in immunocompetent patients, whereas the former has previously been described only in patients infected with human immunodeficiency virus. The lesions in our patient healed after topical treatment with corticosteroids and alteration of immunosuppressive therapy, supporting the role of immunosuppression in this case. We classified our patient's condition as lying in the spectrum of the aforementioned two conditions, but the relationship between both diseases remains to be clarified. Awareness of these unusual conditions may prevent the use of unnecessary aggressive therapies in similar patients.

An update on cutaneous melanoma in Turkey: evaluation of 19-year data in a single tertiary centre and review of the literature.

BACKGROUND: Information on frequency of melanoma and its clinicopathological subtypes derived from dermatology clinics in Turkey is limited. OBJECTIVE: As data about melanoma show clear differences due to geographic and ethnic distribution, we scrutinized the rich data of our dermatology centre in Istanbul. METHODS: Consecutive patients diagnosed with melanoma in a tertiary dermatology clinic during the last 19 years were retrospectively investigated about the clinical presentation of the skin lesions during admission, frequency of subtypes and localization of the tumour. RESULTS: There were 227 patients with melanoma showing five different clinical presentations: 200 of them had totally 207 primary cutaneous melanoma (PCM) lesions, nine had PCM lesions associated with metastatic skin lesions, three presented with local recurrence, eight with only skin metastases and seven with regressed skin melanoma following systemic melanoma metastases. Histologically, 23.19% of the PCM lesions were intraepidermal (in situ) and Breslow thickness was less than 1 mm in 30.9% of the patients with invasive melanoma. The most common subtype was superficial spreading melanoma (SSM) (37.19%), followed by lentigo malignant melanoma (LMM) (31.4%), acral lentiginous melanoma (ALM) (19.32%) and nodular melanoma (NM) (6.76%). Head and neck region was the most common (34.78%) localization of PCM lesions. CONCLUSIONS: Different clinical presentations, including various types of cutaneous melanoma metastases, were seen. However, a great proportion of our patients were relatively early diagnosed, either having an in situ or an invasive PCM with a Breslow thickness ≤1 mm. Even though SSM was the most common subtype of PCM in our series, its rate was lower compared to many European countries. Furthermore, the rate of NM subtype was also low, while LMM and ALM rates were higher in comparison to studies originating from European countries. This striking discrepancy requires further studies to explain the probable causes.

Childhood mycosis fungoides: a report of 20 cases from Turkey.

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphomas in adults. In the recent past, several reports have focused on an increased prevalence of MF in children. OBJECTIVE: The present study was aimed to evaluate the clinical characteristics, treatment modalities and disease progression in childhood MF patients from Turkey. METHODS: In a retrospective analysis of 368 MF patients in a referral center at Istanbul, Turkey, 20 patients were diagnosed before the age of 18 years and were included in the study. RESULTS: Childhood cases constituted 5.4% of all MF patients. The age at the time of diagnosis ranged between 2-18 years with a mean of 9.20 ± 4.52 and a median value of 9.50 years. The clinical presentation of MF consisted of solely patches in 60%, followed by plaques or plaques and patches in 40% of the patients. Hypopigmented lesions were seen in 45% and purpuric lesions in 30% of the patients. Four patients (20%) were diagnosed to have unilesional MF. The large majority of the patients (95%) had T1 N0 M0 or T2 N0 M0 disease. Sixteen patients were followed between 1-13 years (median: 3.75 years). All the patients were treated with skin directed treatments including topical corticosteroids, topical bexarotene, topical carmustine, narrow band UVB and PUVA. None of the patients progressed to an advanced stage. After an initial clearance, 69.2% of the patients were observed to have recurrences. CONCLUSION: In our experience, patches were more prevalent among childhood MF cases. Overrepresentation of hypopigmented and purpuric lesions was remarkable. Progression to an advanced stage was not seen. However, recurrences after discontinuation of therapy were common.

Acquired Acrodermatitis Enteropathica Syndrome in a Kidney Transplant Receipt: A Case Report.

Acrodermatitis enteropathica syndrome (AE) is a clinical entity that results in severe zinc deficiency. It can be genetic or acquired. Acquired AE has been reported in patients with chronic liver disease, malabsorption syndrome, sickle cell anemia, and chronic renal failure. We present a kidney transplant recipient with skin rash and watery diarrhea. The patient had low serum zinc levels, which quickly resolved after zinc supplementation. Skin biopsy showed cytoplasmic pallor and vacuolization and ballooning degeneration of keratinocytes within the superficial epidermis, which may have led to confluent necrosis of keratinocytes. Large amounts of keratinosome-derived lamellae were found in the intercellular spaces in the keratinized area, probably related to disturbance of keratinosome metabolism due to zinc deficiency.

Merkel-cell carcinoma arising after liver transplantation: a case report.

Merkel-cell carcinoma is a rare and an aggressive neuroendocrine tumour of the skin that has been reported to be common in transplant recipients. Herein, a 25-year-old woman who developed Merkel-cell carcinoma after liver transplantation is reported.

An unusual localization of thymoma.

A 41-year-old man with myasthenia gravis was referred to our clinic to undergo a thymectomy by video-assisted thoracic surgery. After physical examination of the thorax and evaluation by computed tomography, a mass lesion was noticed in the neck and thymectomy was performed by partial sternotomy combined with a cervical incision. The cervical thymoma was discontinuous with the thymus. Thymoma in the neck has rarely been reported. In this case report we emphasize the possible presence of thymoma in the neck and the importance of neck evaluation in patients with myasthenia gravis.

Clinicopathologic evaluation of nodular cutaneous lesions of Behçet syndrome.

Among the cutaneous manifestations, nodular lesions are rather common in Behçet syndrome. The histologic nature of these lesions has been a matter of controversy. To establish their distinguishing features, biopsy specimens from nodular lesions of 24 patients with Behçet syndrome, 25 with nodular vasculitis (NV), and 20 with erythema nodosum (EN) were compared. Statistical analysis revealed insignificant differences between most of the histologic features of Behçed syndrome and NV. However, neutrophil-predominating infiltrate in the subcutis was more common in Behçet syndrome, while necrosis and granuloma formation were encountered more frequently in NV. The differences between Behçed syndrome and EN were more significant. Septal panniculitis, lymphocyte-predominating infiltrate, absence of many vascular changes as well as vasculitis, and necrosis were features in favor of EN. Nodular lesions of Behçet syndrome are mainly neutrophilic vascular reactions with histologic features similar to NV but significantly differing from EN associated with other systemic diseases.

Clinical outcomes of renal cholesterol crystal embolization.

Cholesterol crystal embolization is an increasingly recognized disease, presenting with a wide clinical spectrum, usually occurring in elderly men who undergo an angiographic procedure or vascular surgery. We report three patients who developed systemic cholesterol embolic disease and varying degrees of renal failure after angiographic interventions of the coronaries.

Dyskeratosis congenita associated with Hodgkin's disease.

Dyskeratosis congenita is a rare, hereditary, multisystem disorder characterized by mucocutaneous changes, pancytopenia and increased incidence of malignancy. Different types of neoplasia have been reported in association with dyskeratosis congenita. We present a second case associated with Hodgkin's disease. Delayed appearance of dermatological signs and association with chronic hepatitis B are other unusual features of this case.

Desmoplastic trichilemmoma: a rare tumor of the eyelid.

PURPOSE: To report an upper eyelid mass which proved to be a desmoplastic trichilemmoma. METHODS: A 60-year-old man had a slowly enlarging upper eyelid mass. The tumor was excised. The pathologic evaluation of the tumor was centered on the differential diagnosis. RESULTS: The clinical appearance of this lesion is nonspecific and can simulate a verruca, follicular keratosis, or basal cell carcinoma. Central desmoplasia, outer root sheath differentiation of the tumor cells, and CD34 positivity are the main characteristics that allow differentiation from basal cell carcinoma. CONCLUSIONS: Proper recognition of a benign neoplasm that may be misdiagnosed as basal cell cancer can prevent aggressive surgical treatment.