Nasal oral fistula revealing Langerhans´ cell histiocytosis in adult patient: case report.

Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.

[Dermatofibroma with sebaceous induction]. / Histiocytofibrome avec induction sébacée.

BACKGROUND: Histiocytoma or dermatofibroma (DF) is a common benign skin tumour with several clinical and histopathological variants. Sebaceous induction overlying a dermatofibroma is rare and infrequently reported. Using a detailed clinical case, herein the semiological and dermoscopic features of this lesion are described and illustrated, and the differential diagnoses presented. PATIENTS AND METHODS: A 52-year-old man consulted for a physical examination, which revealed a firm papular lesion of the upper middle back. The upper part of the nodule was covered by a slightly hyperpigmented surface, with numerous small whitish lobules. Microscopic examination revealed a dermatofibroma with sebaceous induction. DISCUSSION: Sebaceous induction overlying a dermatofibroma is not frequent, and it occurs in most cases on or near the shoulder. The typical dermoscopic pattern involves many whitish globules or clumps grouped into clusters. The aetiology is unknown but could stem from a conducive microenvironment in shoulder skin, associated with growth factors secreted by the DF.

[A pink nodule on the face]. / Un nodule rose du visage.

We report the case of a 32-year old female patient, with no notable medical history, requiring dermatology consultation for evaluation of a nodule on her right cheek which had evolved over the past 10 years. Clinical examination showed a reddish dermal-based nodule with a smooth surface. The lesion measured 1cm in diameter and was located at the level of the right cheek (A). There was no adenopathy and the remainder of the clinical examination was normal. The patient underwent skin biopsy which showed tumor proliferation composed of fusiform cells with poorly limited eosinophilic cytoplasm and lightly atypical elongated nuclei without mitosis and with mononuclear inflammatory cell infiltrate at the level of the dermis. The epidermis was thinner. Anti-CD68 antibody was positive, while anti-CD34 antibody, PS100 and anti-AML were negative. The diagnosis of benign cellular histiocytofibroma was retained. The patient underwent total resection with a healthy resection margin of 5mm. The patient had a median 2-year follow-up with no recurrences identified. Benign histiocytofibroma mainly occurs in middle-aged women. It more often appears as an erythematous nodular, bluish, brownish or achromique dermal-based little painful but sometimes embarrassing lesion characterized by firm consistency and commonly located at the level of the lower limbs. Racial histiocytofibroma is rarely reported in the literature. Differential diagnosis includes Darier-Ferrand dermatofibrosarcoma, leiomyoma, Kaposi nodule and solitary fibrous tumor of the skin. Histologically, benign cellular histiocytofibroma is composed of pure intradermal disordered proliferation of fusiform cells arranged in bundles or in eddies and circumscribed by lymphocytic inflammatory reaction with presence of foamy histiocytes. The lesion is often highly vascularized with possibile hemorrhagic foci and especially, with angiogenesis images. In a minority of cases, especially in the case of huge histiocytofibromas, the epidermis is thinner and may even ulcerate. The immunohistochemistry shows the expression of CD68 and F XIIIa + positive cells while a lack of CD34, PS100 and Anti-AML expression. It is characterized by a chronic, benign evolution with possible spontaneous regression. The treatment is based on surgical resection.