[Regression of primary melanoma with metastases associated with amyopathic dermatomyositis]. / Dermatomyosite amyopathique associée à un mélanome régressif métastasé.

BACKGROUND: Dermatomyositis is a rare and serious inflammatory connective tissue disease characterized by a typical cutaneous rash and myopathy. Amyopathic dermatomyositis is a particular form of dermatomyositis involving only cutaneous signs and without myopathy present for over 2 years. PATIENTS AND METHODS: A 48 year-old woman presented with a 3-year history of cutaneous rash without myopathy characteristic of amyopathic dermatomyositis. Clinical examination revealed extensive axillary adenopathy, histological examination of which suggested secondary melanoma. The patient reported a black nevus in the axillary area that had disappeared 1 year earlier. Curettage of the lymph node was negative and the patient was treated with interferon (3M 3 times a week). Regression of the cutaneous signs was noted. DISCUSSION: The data, there have been no other reports of paraneoplastic amyopathic dermatomyositis associated with regression of primary melanoma. The literature contains few reports of dermatomyositis associated with melanoma. Amyopathic dermatomyositis may be associated with malignancy.

[Mycosis fungoides presenting as annular erythema]. / Mycosis fongoïde à type d'érythème annulaire centrifuge.

INTRODUCTION: Mycosis fungoides is a lymphoma, the classical clinical form of which involves erythematosquamous lesions. However, it can present various atypical aspects: hyper pigmentation or hypo pigmentation, suggestive of pyoderma gangrenosum or ichtyosis. We report a case of mycosis fungoides, unusual in its presentation in the form of centrifugal annular erythema. OBSERVATION: A 78 year-old man had developed a parapsoriasis in plaques for more than 20 years. In May 2002 he consulted because of the recent infiltration of one of the plaques, without concomitant pruritus. The clinical examination revealed 3 lesions of the popliteal groove of the right groin and the left cheek suggestive of centrifugal annular erythema. Histology, revealing Pautrier microabscesses, was compatible with the diagnosis of mycosis fungoides. Evolution was marked by the spontaneous regression of the plaque on the face and remission of the other two plaques after local treatment with chloromethin and topical corticosteroids. Nevertheless, new plaques appeared despite continued treatment, combined with PUVA therapy sessions. DISCUSSION: When searching the literature, we only found one other case of mycosis fungoides, the clinical aspect of which was a centrifugal annular erythema, but in which the histological examination confirmed the diagnosis of mycosis fungoides. Our case report is also unusual in the clinical regression of the lesion on the face, without treatment; this has only been reported in two cases. Mycosis fungoides can appear in various clinical forms. The centrifugal annular erythema form is rare, but this diagnosis should be evoked.

[Limbal stem cell deficiency associated with KID syndrome, about a case]. / Insuffisance limbique bilatérale associée à un syndrome KID. A propos d'un cas.

We report the case of a 30-year-old woman with KID (keratitis ichthyosis deafness) syndrome consulting for massive corneal neovascularization. She had already undergone two unsuccessful penetrating keratoplasties in her left eye. Visual acuity was limited to no more than light perception because of the opacity and neovascularization of the graft. In the light of this case of limbal cell deficiency, we discuss the ophthalmologic manifestations of KID syndrome as well as its clinical characteristics, physiopathology, and mode of transmission.