Nasolabial Ulcerated Placard Revealing a Primary Cutaneous Aggressive Epidermotropic CD8+ T-cell Lymphoma.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare entity representing less than 1% of cutaneous lymphomas. It has an aggressive clinical manifestation with a poor prognosis. It is characterized by cytotoxic and epidermotropic CD8+ proliferation. It also expresses the TIA-1 marker. We report a new case for its display and aggressive character, diagnostic difficulty, and good therapeutic response to chemotherapy. This is a 62-year-old female patient admitted to the hospital for a nasolabial ulcerated placard evolving for two years. Clinical examination revealed submandibular lymph nodes. The specimen analysis associated with anatomoclinical manifestation was concluded for a primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. Tumor extent assessment did not show any secondary localization. The blood tests and serology were unremarkable. The patient had benefited from a CHOEP-type multidrug therapy protocol with complete healing of the lesion after three courses of chemotherapy.

Giant perianal condyloma acuminatum in an infant without sexual abuse.

Giant condyloma acuminatum is rare in infants. We report a case of giant perianal condyloma in a 10-month-old infant without sexual abuse. The patient was treated by surgical excision associated with electrocoagulation with no recurrence after 2 years. While both sexual and nonsexual transmissions are possible in patients with condyloma acuminatum, sexual abuse must always be considered in children.

[Small cell neuroendocrine carcinoma of the bladder: Report of five cases and a review of the literature]. / Carcinome neuroendocrine à petites cellules de la vessie : à propos de cinq cas et revue de la littérature.

Neuroendocrine small cell carcinoma of the bladder is a rare and aggressive tumour, accounting for less than 1% of all bladder tumours. Given its rarity and the absence of randomized trials, the therapeutic management of these tumours remains difficult. By analogy with small cell lung cancer, multimodal treatment is often proposed. Radical cystectomy plus chemotherapy and chemoradiation therapy are associated with better survival compared to monotherapy. We report our experience in the management of these tumours with literature review.

Immunohistochemical and genetic exploration of incompatible A blood group antigen expression in invasive micropapillary breast carcinoma: A case report.

INTRODUCTION: Invasive Micropapillary Carcinoma (IMPC) of the breast is a relatively rare subtype of invasive ductal carcinoma and represents the most inherently aggressive form. Expression of incompatible blood group A antigen in cancer of type O patients has been reported in several types of cancer, however, the biosynthetic mechanism and the genetic basis remain unclear until today. The aim of the present case report study was to evaluate the expression of incompatible blood group A antigen and to identify the genetic basis of this expression in IMPC of the breast. MATERIAL AND METHODS: One patient blood group O with Invasive Micropapillary Carcinoma was screened at Pathology Department of University Hospital CHU Ibn Rochd, Casablanca. ABH antigens expression was assessed by immunohistochemistry. ABO genotyping was performed by allele specific primers PCR-ASP and Exon 6 of ABO gene was sequenced with Sanger method. RESULTS: H antigen was expressed in endothelial and epithelial cells of normal tissue. However, H antigen expression was lost in both invasive micropapillary carcinomas. A antigen was expressed in IMPC with approximately 80% of positive cells. Tumor DNA was genotyped as heterozygous A/O. In normal DNA, we identified a single frameshift deletion c.320delA p.(Glu107Glyfs*12), which is removed from tumor DNA. CONCLUSION: Our findings suggest that incompatible A antigen expression in IMPC is due to glycosyltransferase A encoded by an A allele which is derived from O allele with a deletion at the position 320.

[Hidradenocarcinoma of the heel associated with inguinal metastases]. / Hidradénocarcinome du talon associé à des métastases inguinales.

BACKGROUND: Hidradenocarcinoma is a rare malignant tumour involving the sweat glands. It classically arises de novo, only rarely resulting from pre-existing hidradenoma. The literature contains few reports of lymph node metastasis in this tumour. We report a case of a patient with hidradenocarcinoma of the heel associated with inguinal node metastases. PATIENTS AND METHODS: We report the case of a 64-year-old patient with a history of chronic smoking, who in the last two years developed a painless nodule in his right heel, with no prior injury, and which gradually increased in size to become an ulcerated tumour. Physical examination revealed a rounded tumour mass, ulcerated in the centre, and associated with multiple inguinal adenopathies. Histological and immunohistochemical examination was suggestive of hidradenocarcinoma. The patient had undergone extensive local excision with inguinal lymphadenectomy. Histological examination showed infiltration of lymph nodes by the tumour with capsular rupture. Radiotherapy was subsequently given. The outcome was good without recurrence after 34 months of follow-up. DISCUSSION: Hidradenocarcinoma is a rare malignant tumour. Diagnosis is based on histological and immunohistochemical examination. However, hidradenocarcinoma may on occasion be difficult to differentiate from hidradenoma, a benign tumour, hence the interest of complete surgical resection with safety margins even in the absence of cytological malignancy. Local recurrences are common. The occurrence of lymph node metastasis during hidradenocarcinoma has been described only rarely in the literature. Such metastases usually occur after tumour resection. The specific features of our case are the rarity of lymph node metastases in hidradenocarcinoma coupled with the fact that these metastases were discovered upon diagnosis of the primary tumour.

ABO blood groups in relation to breast carcinoma incidence and associated prognostic factors in Moroccan women.

The association between blood groups ABO and different types of diseases was established in several previous studies. Our aim was to seek the possible association between the ABO blood group and breast cancer-associated prognostic factors. The Chi-squared analytic test was used to compare phenotypic ABO distribution among Moroccan blood donors and 442 cases of women suffering from breast carcinoma with archived files in Maternity Ward of University Hospital C.H.U Ibn Rochd between 2008 and 2011. High incidence of breast carcinoma was observed in blood type B patients (p < 0.05). Blood type B was associated with breast carcinomas overexpressing human epidermal growth factor receptor HER2 (p < 0.05) and high risk of cancer at age over 70 years (p < 0.001). Blood type A was associated with high risk of cancer among women younger than 35 years old. Blood type A and AB were associated with high incidence of lymph node metastasis (p < 0.05). Multivariate analysis has shown correlation between O blood type and estrogen receptor-positive tumor. Patients with blood group A, B, and AB were more likely to develop aggressive breast carcinoma. Further follow-up studies are necessary to clarify the role of ABH antigens in the progression of breast carcinoma.

Paronychia-like cutaneous leishmaniasis.

Chronic paronychia is an inflammatory recalcitrant disorder affecting the nail folds. We report one patient with paronychia revealing ungueal leishmaniasis. A 34-year-old man, resident in the north of Morocco, presented with a 6-month history of an inflamed proximal nail fold of the left thumb, resistant to antibiotics and anti-fungal treatments. En bloc excision of the proximal nail fold was done. The histopathological exam showed epithelioid granulomas with giant cells and the presence of leshmania amastigotes, leading to the diagnosis of ungueal leishmaniasis. Clinical aspects of cutaneous leishmaniasis can be very misleading. The paronychial form is rarely described. In endemic areas it is necessary for the physician to be aware of atypical skin presentations of leishmaniasis.

[Actinomycosic mycetoma of the foot in Morocco due to Actinomycetes viscosus]. / Mycétome actinomycosique du pied à Actinomycetes viscosus au Maroc.

We present the case of an actinomycotic mycetoma of the foot due to Actinomycetes viscosus. It evolved for nine years on the foot of a 26-year-old patient from a rural environment: Douar Inezgane (city in southern Morocco). Bacteriological study of the skin and grains confirmed the diagnosis. It showed positive bacilli on direct examination and on Gram staining and in positive culture. Histological study showed a polymorphous granulomatous inflammation without signs of malignancy with actinomycotic grains. Then we retained the diagnosis of primary cutaneous actinomycosis without visceral locations. The treatment was based on antibiotics: penicillin G by intravenous infusion for five weeks, relayed orally by amoxicillin associated with trimethoprim-sulfamethoxazole for long periods. After six months of treatment, we observed a favorable outcome with reduction of the swelling, nodules, lymphadenopathy, fistula's number and extension of time of issue of grains. The current follow up is 15 months. The primary cutaneous actinomycosis is still relevant in Morocco.

[Deep-seated granulomatous pyoderma gangrenosum: A novel variant?]. / Pyoderma gangrenosum granulomateux profond : une forme originale de pyoderma gangrenosum ?

BACKGROUND: We report an extremely rare case of pyoderma gangrenosum with the clinical features of pyoderma gangrenosum but with unusual histopathological findings and deep massive granulomatous infiltration through the dermis. PATIENTS AND METHODS: A 15-year-old girl presented with a two-month history of deep ulcers on the lower legs and forehead. She also presented abdominal pain with diarrhea and vomiting that disappeared after symptomatic treatment. Histology showed extensive granulomatous infiltration in the dermis and hypodermis and a micro-abscess in the dermis. An abdominopelvic CT scan revealed peritoneal fluid and tissue formation in a lateral carotid cave (6.56 cm/4.56 cm). Colonoscopy was normal. The patient was treated with oral prednisone, resulting in resolution of the problems of cutaneous lesions, peritoneal fluid and tissue formation. DISCUSSION: Our case involves a rare presentation of pyoderma gangrenosum (PG) inconsistent with any of the four clinical variants of PG and characterized histologically by the presence of deep granulomas in the dermis and hypodermis. Despite the atypical clinical presentation and unusual histopathological findings, PG seemed the most likely diagnosis after infectious diseases had been ruled out; the good outcome achieved with oral corticosteroids supported our diagnosis.

[Xanthoma disseminatum with asymptomatic multisystem involvement]. / Xanthoma disseminatum avec atteinte viscérale multifocale asymptomatique.

BACKGROUND: Xanthogranulomas belong to non-Langerhans histiocytosis of the second group in the Histiocyte Society classification. They comprise a heterogeneous group of rare entities frequently involving cutaneous tropism. Xanthoma disseminatum belongs to this group of non-Langerhans histiocytosis. We report a case of xanthoma disseminatum (XD) in which localized skin and mucous impairment revealed multisystem involvement. PATIENTS AND METHODS: A 28-year-old man presented with a two-year history of progressive yellow-orange and infiltrated xanthomatous papulonodular lesions of the face. Lesions of the oral mucosa and genital region were seen, with no functional repercussions. No ophthalmic or other complications were found. Histopathology showed a dense histiocytic infiltrate within the dermis with Touton giant cells, foamy multinucleated giant cells and inflammatory cells, without necrobiosis. Histiocytes were positive for CD68 but negative for CD1a. Gastric and lung involvement was seen and was confirmed at histology. Bone scintigraphy showed suspicious left ulnar hyperfixation suggesting bone involvement. No monoclonal gammopathy or diabetes insipidus was seen. Our patient was treated with corticosteroids 1mg/kg/day and thalidomide 100 mg/day. The outcome was marked by regression and exfiltration of the cutaneous lesions from the second week of treatment, with subsidence continuing at 3 months. DISCUSSION: This case involves a very rare form of xanthoma disseminatum. The localized facial skin lesions revealed multifocal non-Langerhans histiocytosis that was in fact asymptomatic. The diagnosis of XD was based on clinical, histological and immunohistochemical criteria. Xanthoma disseminatum is a non-Langerhans histiocytic proliferation first described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis in which the facial involvement is common, together with diabetes insipidus and normal lipid metabolism. The prognosis is determined by the presence of mucosal xanthomas and visceral involvement. Thus, all xanthogranulomas involving multiple lesions warrant screening for visceral involvement. Diagnosis of this entity can be difficult and is usually based on clinical and histopathological findings. In addition, treatment is complex and non-consensual.