A Challenging Cutaneous T-Cell Lymphoma.

Cutaneous peripheral T-cell lymphomas not otherwise specified (CPTL-NOS) are rare neoplasms accounting for just 2% of cutaneous peripheral T-cell lymphomas (CPTL). Only very few case series have been reported. They represent a phenotypically and prognostically heterogenous group of CPTL that do not fit into any of CPTL well-defined subtypes. The authors report a case of a 64-year-old man with simultaneous plaque-like lesions and disseminated nodules growing rapidly on the face, trunk, and extremities over a 6-month period. There was no a history of preceding patches, erythematous plaques, rash, or pruritic lesions. These lesions were extending over 80% of the skin surface. Histopathologic analysis revealed dense diffuse infiltrates composed of mostly medium-sized to large lymphoid cells throughout the entire dermis without epidermotropism. Neoplastic cells were atypical with markedly pleomorphic nuclei. Immunohistochemistry showed that the tumor cells were positive for CD3, CD4, and CD5 with a loss of CD7. They were negative for CD20, CD8, CD56, CXCL13, PD1, TIA-1, granzyme-B, perforin, CD25, and CD30. The proliferative fraction was low, with MIB-1 labeling less than 10% of cells. The authors diagnosed the patient with primary CPTL-NOS. Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTL especially because most of these lymphomas have an aggressive behavior and exhibit an unfavorable prognosis.

[Vasculitis in Sweet's Syndrome]. / Vascularite au cours du syndrome de Sweet.

BACKGROUND: Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is non-infective dermatoses that exhibit a predominantly neutrophilic inflammatory infiltrate. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. AIM: To describe their clinical, pathological and therapeutic characteristics. METHODS: We report a series of 47 cases of Sweet's syndrome, collected in our institution between 1997 and 2011. RESULTS: The patient population consisted of 11 males and 36 females. The mean age was 47 years (28-74). An associated disease process was seen in 10 patients: inflammatory disease (3 cases), inflammatory bowel disease (2 cases), tuberculosis (3 cases) and diabetes (3 cases). One case of pregnancy was observed. In the prodromal phase, functional symptoms were reported in 38 cases (80.8%). Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in 8 cases. CONCLUSION: The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's diseases which requires careful surveillance.

[Gastric MALT lymphoma: anatomo-clinical data and outcome of early stage H. pylori-associated gastric MALT-lymphoma resistant to treatment]. / Lymphome du MALT gastrique : Particularités anatomo-cliniques et évolutives des formes résistantes au traitement.

AIM: To describe the clinical, endoscopic and histological particularities of early stage HP associated gastric MALT lymphoma resistant to anti Hp treatment and identify predicting factors of resistance. METHODS: We retrospectively studied 12 patients with primary low grade gastric localized MALT lymphoma treated with anti HP treatment and diagnosed at La Rabta Hospital from 1999 to 2009. RESULTS: The ultrasonography was normal in 5 patients between the 6 responding patients. Perigastric lymph nodes were found in non responders (33.3%). Hp eradication was achieved in 66% of patients not responding while Hp was eradicated in 100% of responders. The two non-responding patients with failure of eradication of Hp had a strain resistant to Clarithromycin Hp. CONCLUSION: Predicting factors of failure of anti HP: HP resistance to antibiotics, the proximal head, and the presence of perigastric lymph nodes. Recently, chromosomal aberrations and immune-histochemical markers have been implicated as factors of non response to anti Hp.

Infantile desmoplastic fibroma of the maxilla.

BACKGROUND: Infantile desmoplastic fibromatoses are benign fibrous tissue tumours, non-metastasizing but locally aggressive and with high likelihood of recurrence. Many cases occur in young children between 18 months and 3 years and commonly present as painless mass of the submandibular region. The maxilla is rarely involved. AIM: Through this report and a review of literature, we are going to study clinical, pathological and prognostic characteristics of this affection. CASE REPORT: We report here a case of an aggressive desmoplastic fibroma arising in a 20-months-old infant originating in the left anterior maxillary wall. Clinical examination of the face revealed a non tender, firm, expansion of the left maxilla of 2.5 cm of diameter. The diagnosis of DF of the maxilla was made on the basis of histological features on biopsy specimen. No other location of the fibromatosis was found. The child had a favourable course. CONCLUSION: DF is an intraosseous, non metastasizing and locally aggressive fibrous tumour. Although its benignity, it could engage vital prognosis since it can extend into vital organs.