A destructive centrofacial granuloma: Case report.

INTRODUCTION: Centrofacial granulomas have several etiologies, which poses diagnostic difficulties and delays in management. Herein, we report a confusing case revealing granulomatosis with polyangiitis (GPA) in its localized form. CASE PRESENTATION: A 76-year-old man presented with a pruritic centrofacial placard that had been evolving for 3 years. On examination, there was a centrofacial infiltrated and erythematous papulonodular placard. Skin biopsies were not conclusive. The immunological assessment was negative. The evolution was marked by the extension of the placard, the destruction of the nasal pyramid and nasal mutilation. Only surgical biopsy revealed dermohypodermal, cartilaginous and endonasal non-necrotizing granulomatous tuberculoid inflammation with leukocytoclastic and necrotizing vascularitis. The diagnosis of GPA in its localized form was retained. Treatment with prednisone was initiated combined with monthly boluses of cyclophosphamide. The assessment for systemic involvement remained negative during the follow-up. CLINICAL DISCUSSION: Localized forms represent up to 29% of GPA cases. There are clinical, but also biological differences, since ANCA are found in more than 90% of diffuse forms and only in 50-78% of localized forms. Our case may represent a rare distinctive subset of GPA limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction. CONCLUSION: It is necessary to evoke GPA in its localized form and to perform multiple deep biopsies in front of any facial granulomatosis. Early diagnosis and appropriate treatment prevent mutilating and disfiguring sequelae.

Oral White Sponge Nevus: An Exceptional Differential Diagnosis in Childhood.

White sponge nevus is an autosomal dominant skin disorder characterized by white, irregular, diffuse plaques mainly affecting the oral mucosa. Histological findings of white sponge nevus are characteristic but not pathognomonic. We report a case of an oral white sponge nevus in a 6-year-old girl, which poses a problem in differential diagnosis with oral candidiasis. No treatment was performed because of the benign and asymptomatic nature of the lesions.

Hypotrichosis with juvenile macular dystrophy: Portuguese case.

Hypotrichosis with juvenile macular dystrophy is a rare congenital disease mainly found in the Druze population of Northern Israel. This disorder is caused by the CDH3 mutation encoding P-cadherin, which is expressed in retinal pigment epithelium and hair follicles. An 11-year-old girl who was born to related Portuguese parents, had hypotrichosis since birth and macular dystrophy diagnosed at age 5. Fundus examination and fluorescein angiography revealed located macular pigmentary abnormalities. No molecular analysis was done. A fundus examination should be considered mandatory in the assessment of congenital hypotrichosis.

Psoriasis and Staphylococcus aureus skin colonization in Moroccan patients.

UNLABELLED: Psoriatic lesions are rarely complicated by recurrent infections. The aim of our study is to determine skin colonisation and nasal carriage of Staphylococcus aureus in patients with psoriasis and in healthy persons. PATIENTS AND METHODS: a comparative study that include 33 patients with psoriasis and 33 healthy persons. Samples were taken from lesional and non lesional psoriatic skin and from healthy skin of control group. For S. aureus nasal carriage, we used sterile cotton tipped swabs. Out of 165 samples (66 skin samples and 33 nasal swabs), 26 S. Aureus strains were isolated in 26 persons, 57.69% in the control group and 42.3% in the psoriasis group. S. aureus skin colonization was found in one case (3%) in lesional psoriatic skin vs 9 cases (27.3%) in control skin OR=0.08 IC 95% (0.01-0.70) p=0.02 and in 12,1% in non lesional psoriatic skin vs 27, 3% in control skin (p =0,13). This colonization was less important in lesional psoriatic skin (3%) than in non lesional psoriatic skin (12.1%) p= 0.20. Nasal screening identified (7/33) 21, 21% S. aureus carriers in psoriasis group and in control group. Our results are in consensus with literature findings. They have confirmed the importance of antimicrobial peptides in Innate immunity of human skin. These peptides are normally produced by keratinocytes in response to inflammatory stimuli such as psoriasis. Their high expression in psoriasis skin reduces the risk of skin infection and skin colonization with S. Aureus.

Cutaneous sarcoidosis simulating porokeratosis of Mibelli.

We report a skin localization of systemic sarcoidosis, which presented with lesions that resemble porokeratosis of Mibelli. Skin biopsy showed non-caseating sarcoidal granuloma. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, our presentation is uncommon. Partial remission was obtained with hydroxychloroquine and prednisone.