We present the case of a patient undergoing chemotherapy with 5-Fluorouracil (5-FU), irinotecan and cetuximab, who presented polymorphous skin lesions with oral and genital mucosal involvement. The differential diagnosis included paraneoplastic pemphigus, a severe autoimmune disease associated with neoplasms. The histology can be variable with acantholysis or lichenoid dermatitis. The presence ...

Darier's disease is a skin disorder characterized by multiple eruptions of hyperkeratosis or crusted papules at seborrheic areas with histologic acantholysis and dyskeratosis. It is caused by mutations in a single gene, being ATP2A2 and that is expressed in the skin and brain. The cooccurrence of various neurologic and psychiatric diseases with Darier's disease has been reported frequently in l...

Galli-Galli disease is a rare genodermatosis currently regarded as an acantholytic variant of Dowling-Degos disease. The 2 diseases have the same clinical features: reticular hyperpigmented macules in the great skin folds, erythematous scaly papules and plaques, comedo-like lesions, and pitted perioral scars, and the only differentiating characteristic is the histological finding of acantholysi...

BACKGROUND Acantholytic squamous cell carcinoma (ASCC) is an uncommon histopathologic variant of SCC, characterized by marked acantholysis, wherein the tumor cells demonstrate defective cohesion to one another in the cancer nest leading to a pseudoglandular or pseudovascular appearance. The most common site of ASCC is the sun-exposed areas of the skin. Sporadic cases of ASCC have also been repo...

resulting in dysphagia A 44-year-old man was admitted to our institution because of the appearance of painful cutaneous, oral, and genital vesicles. His previous medical history was unremarkable. He had had pharyngitis treated with amoxicillin 1 month before the hospitalization. On physical examination, symmetrically erythematous plaques, covered with vesicles, were seen. Because of subsequent ...

In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell-cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia. The pathology, at least partially, may depend on protease release from keratinocytes, but might also result from antibodies interfering with an adhesion function of Dsg3. However, a d...

The majority of pemphigus vulgaris (PV) patients suffer from a live-threatening loss of intercellular adhesion between keratinocytes (acantholysis). The disease is caused by auto-antibodies that bind to desmosomal cadherins desmoglein (Dsg) 3 or Dsg3 and Dsg1 in mucous membranes and skin. A currently unresolved controversy in PV is whether apoptosis is involved in the pathogenic process. The ob...

A 52-year-old man who had had WaldenstroÈm's disease for 5 years presented to our department with clinical manifestations of hyperviscosity syndrome including headaches and paresthesias of the extremities. All treatments for the haemopathy had been withdrawn 6 months earlier because of drug resistance. Besides splenomegaly, physical examination showed 2 types of cutaneous lesions: nonfollicular...

In humans, congenital and hereditary skin diseases associated with epidermal cell-cell separation (acantholysis) are very rare, and spontaneous animal models of these diseases are exceptional. Our objectives are to report a novel congenital acantholytic dermatosis that developed in Chesapeake Bay retriever dogs. Nine affected puppies in four different litters were born to eight closely related ...