[Extra-nodal NK/T-cell lymphoma, nasal-type, revealed by cutaneous and ocular involvement]. / Atteintes cutanée et oculaire révélatrices d'un lymphome T/NK extra-nodal de type nasal.

BACKGROUND: Extra-nodal NK/T-cell lymphoma (ENKTL) is a form of highly malignant non-Hodgkin's lymphoma. There are two types: nasal forms primarily affecting the oropharyngeal sphere and so-called nasal-type extra-nasal forms in which primary skin involvement is the most common feature enabling diagnosis. Herein, we report a case of systemic nasal-type ENKTL (ENKTL-NT) that was diagnosed based on skin involvement associated with ocular involvement. PATIENTS AND METHODS: A 67-year-old female patient, without immunodepression, was admitted to the dermatology department for a worsening inflammatory scaly patch of skin on her right calf. Secondarily, further lesions appeared on her body as well as a generalized macropapular rash and sores. These were associated with fever spikes, as well as ophthalmoplegia and edema, preventing her from opening her right eyelid. Tests for infectious, autoimmune and inflammatory disorders were negative. A cerebro-orbital scan revealed infiltration and contrast enhancement of the right periocular fat without any mass effect or cerebral extension. A positron emission tomography (PET) scan revealed multiple hypermetabolic skin lesions. Histological analyses indicated dermal-hypodermal lymphomatous tumor proliferation, and immunohistochemical analyses revealed lymphocytes expressing NK-cell markers (strong CD56+ expression), cytotoxic markers (granzyme B and TIA-1), and the presence of Epstein Barr virus (EBV) in the tumor cells. The patient was diagnosed with systemic ENKTL-NT. Her condition deteriorated rapidly, with the onset of refractory macrophage activation syndrome leading to death due to multiple organ failure. DISCUSSION: Skin involvement in ENKTL is non-specific and uncommon, which can delay diagnosis. Treatment is based on polychemotherapy comprising L-asparaginase and possibly consolidation therapy with autologous or allogeneic hematopoietic stem cell transplantation. The prognosis of ENKTL-NT is poor due the more aggressive nature of the disease compared with the nasal forms, with frequent visceral involvement and macrophage activation syndrome. Skin involvement seems to be a poor prognostic factor. Although ocular involvement is documented, its association with skin involvement is rare and mainly secondary to nasal forms of ENKTL. This case of an extra-nasal form of ENKTL-NT with systemic involvement illustrates the difficulty of diagnosis and the poor prognosis of this type of lymphoma.

[Self-inflicted lesions in the context of hidradenitis suppurativa: Pathomimicry]. / Lésions auto-provoquées dans un contexte d'hidradénite suppurée : un cas de pathomimicrie.

BACKGROUND: Factitious disorders constitute a complex pathology for the dermatologist. Although a diagnosis is often indicated, it is difficult to confirm and treatment is complicated. Dermatitis artefacta is the somatic expression of an often serious psychiatric disorder consciously created by patients on their own cutaneous-mucosal surfaces but the motivation is unconscious and no secondary benefits are sought (in contrast to simulation). Pathomimicry represent a specific entity: the provocation of outbreaks of a known disease, triggered by voluntary exposure to a causative agent. Herein we report on a case of pathomimicry in a context of hidradenitis suppurativa. PATIENTS AND METHODS: A teenage girl whose main previous medical history consisted of grade-2 obesity and an episode of pubic abscess was seen at our clinic for axillary lesions. She presented in a state of negligence, was suspicious and aggressive, and refused to undress. After gaining her trust, clinical examination revealed prominent ulcerations (each with a granulated base) at a distance from the folds in the axillary areas, as well as typical hidradenitis lesions of Hurley Grade 2 with purulent openings and rope-like scars from the inguinal folds. Hospitalization was recommended and a positive outcome was achieved under antibiotic therapy with doxycycline, topical alginate and hydrocellular dressings. A psychiatric evaluation concluded that the patient was presenting dysmorphophobic narcissistic weakness, probably in reaction to recurrent harassment at school since childhood. Once she developed trust with us, which was difficult to establish, the patient admitted to having caused the lesions herself. Given the history and clinical data, as well as the negative laboratory tests, a diagnosis of pathomimicry was made. DISCUSSION: Several cases of dermatological pathomimicry (sustainment by the patient of an ulcer with a known cause, contact with an allergen found in eczema, or renewed use of a medication implicated in toxiderma) or systemic disease (insulin injection in a diabetic patient) have been reported. To the best of our knowledge, this is the first description of pathomimicry associated with hidradenitis suppurativa. Regarding therapy, aftercare should be multidisciplinary. Confessions should not be forced and confrontations, which risk serious psychiatric collapse, should be avoided. A reassuring attitude enables psychiatry to be applied once trust has been sustainably established, hence the crucial role of the dermatologist.