Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease with poor prognosis when associated with malignant neoplasm. We report the case of a patient with PNP associated with a CD20+ non-Hodgkin follicular lymphoma who was treated with Rituximab plus corticosteroids and short courses of cyclosporin. One and a half years after Rituximab therapy, oral ulcerations had cleared and oral methylprednisolone was slowly tapered down without further recurrences. In the course of the disease, the patient developed sepsis due to Listeria monocytogenes and viral infections by human herpes virus 1 and 3. At the end-stage of the disease she developed a cutaneous infection from Mycobacterium chelonae . The patient died 2 years and 7 months after the onset of PNP. Rituximab may be useful for PNP therapy, but further studies are necessary to confirm this hypothesis.

Acantholytic dyskeratosis is a histopathologic pattern defined by a hyperkeratotic and parakeratotic epidermis with intraepidermal clefts containing acantholytic and dyskeratotic keratinocytes. These typical features are distinctive but not entirely pathognomonic of Darier disease, since they may occur at cutaneous and mucocutaneous sites in other conditions such as Grover disease, acantholytic and dyskeratotic epidermal nevus (nevus of Starink), warty dyskeratoma, or acantholytic papular dermatosis localized to the vulvocrural area. We report a newborn girl who had congenital erosive papules and plaques located on the left thigh, left ankle, and right side of the neck. Histopathologic examination of a punch biopsy specimen disclosed findings typical of acantholytic dyskeratosis. In the absence of any family history or other manifestations of Darier disease, we propose the descriptive term "congenital acantholytic dyskeratotic dermatosis." This descriptor characterizes our patient's disease on the basis of the clinical and histopathologic findings and facilitates recognition of this condition until a putative genetic mutation can be demonstrated or ruled out.

Oral involvement in cutaneous CD30+ T-cell lymphoid proliferations is rare and has received little attention in the dermatologic literature. The authors report 2 patients with self-healing, recurrent papulonodular eruptions with the classic clinical, histopathological and immunophenotypic features of lymphomatoid papulosis, which developed two ulcerated papules and an ulcerative nodule on the dorsum of the tongue, respectively. The lesions appeared coincident with a new cutaneous relapse of the disease. Histopathological and immunophenotypic features were similar to those of the cutaneous lesions. All lesions regressed spontaneously after several weeks. Since then, and after follow-up periods of 3 and 7 years, respectively, no evidence of extracutaneous involvement has been detected. Oral involvement in lymphomatoid papulosis is an uncommon event, probably without prognostic significance. Previously reported cases are reviewed. The differential diagnosis of atypical T-cell lymphoid infiltrates observed in the oral mucosa is discussed.

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