1/123. Mammary Paget's disease with acantholytic features and without any detectable breast tumor. Paget's disease is usually associated with an underlying adenocarcinoma of the breast. The initial manifestation is an eczematous or psoriasiform lesion of the nipple, soon extending to the mammary areola and then to the surrounding skin. The histology of the lesions is characterized by the presence, within the epidermal layer, of the so-called Paget's cells, i.e. large cells with vesicular nucleus and clear cytoplasm. The authors report a recent case of Paget's disease of the breast in a 75-year-old woman, unusual both for clinical course and observed histology. As for the clinico-evolutive aspects, although in the case observed the initial skin lesions appeared 20 years before, the different diagnostic procedures repeatedly performed showed no evidence in this patient of an underlying adenocarcinoma. The histologic aspect, on the other hand, was peculiar since the typical characteristics of an acantholytic disease were evident. The presence of intraepidermal cleavages with lost, at times, of the normal contacts among the cells of the Malpighian layer has been described only once in Paget's disease. This fact caused some diagnostic difficulties: however, the typical finding of the Paget cells, their positivity to histochemical methods such as cytokeratin and acid phosphatase allowed the diagnosis. The authors, at last, evaluate the need of a surgical therapy in Paget's disease of the breast without an underlying adenocarcinoma. ( info) |
2/123. Acantholytic variant of squamous cell carcinoma of the breast: a case report. Primary squamous cell carcinoma of the breast is a rare clinical entity. Two large review series found only five cases out of a total of 8351 breast malignancies. This case report presents a patient with metaplastic, pseudoangiosarcomatous carcinoma or acantholytic variant of a squamous cell carcinoma of the breast. This diagnosis was based on the histological finding of highly atypical, acantholytic squamous cells. Because the tumor stained positive for keratin and negative for factor viii, the diagnosis of angiosarcoma was ruled out. Although only scattered case reports have been published on this histological variant, these tumors tend to follow an aggressive course. ( info) |
3/123. Gingival lesions diagnosed as pemphigus vulgaris in an adolescent. Case report. Desquamative gingivitis (DG) is a fairly common disorder in which the gingivae show chronic desquamation. Originally considered to be related to hormonal changes at menopause, since many of the patients are middle-aged women, DG is now recognized to be mainly a manifestation of a number of disorders ranging from vesiculobullous diseases to adverse reactions to a variety of chemicals or allergens. Desquamative gingivitis can be an important early clinical manifestation of serious systemic diseases such as pemphigus vulgaris. The authors present a case that illustrates the importance of a specific diagnosis in patients with desquamative gingival lesions previously treated for 6 months as classical gingivitis. Gingival biopsy showed histologic patterns typical of pemphigus vulgaris. The patient was treated with systemic and topical corticosteroids in association with miconazole. The patient is now under control with low-dose systemic corticosteroids. Proper recognition of lesions in the oral mucosa leads, in several situations, to an early diagnosis of a systemic disease. ( info) |
| enalapril is a widely used antihypertensive drug with a very powerful in vitro acantholytic effect. It has been known to potentially induce pemphigus in genetically predisposed subjects. The action mechanism is complex and still only partially understood. We describe the case of a 66-year-old man, affected with intermediary basal cell carcinoma, in whom the histological examination showed suprabasal acantholytic clefts in the perilesional epidermis. Surprisingly a second biopsy taken from the apparently healthy skin of his back confirmed the presence of acantholytic changes. Clinical signs of pemphigus were absent. The patient's history did not reveal any relevant data but a mild arterial hypertension that had been treated for 1 year with 10 mg enalapril. Taking into account the patient's history (enalapril long-term administration), the absence of any bullous or erosive lesions and the histological findings, a diagnosis was made of in vivo enalapril-induced acantholysis. ( info) |
| Acantholytic foci have been reported several times in pityriasis rubra pilaris (PRP). Lichenoid tissue reactions were also mentioned in the literature regarding PRP. We report a 58-year-old patient who, after having colon cancer, had PRP with biopsies showing acantholytic lesions and a heavy lichenoid lymphocytic infiltration. Investigation by serial sectioning of the acantholytic lesion suggested an involvement of the intraepidermal eccrine duct and further investigation with carcinoembryonic antigen (CEA) staining demonstrated a CEA-positive eccrine duct in the acantholytic foci. We suggest that acantholysis in PRP is induced by proteolytic enzymes, urea, and other substances in eccrine sweat in keratin-plugged acrosyringia. This patient had a combination of three relatively rare features of PRP-acantholysis, lichenoid reaction, and a cancer background. ( info) |
6/123. Generalized pruritic eruption with suprabasal acantholysis preceeding the development of bullous pemphigoid. We report a patient who presented with a papular pruritic eruption of a 3-month duration that histologically showed suprabasal acantholysis accompanied of an eosinophilic inflammatory infiltrate that was consistent with the diagnosis of Grover's disease. Later, erythematous plaques and vesicles appeared which showed a histopathological pattern of eosinophilic spongiosis. The direct immunofluorescence (DIF) study showed lineal IgG and C'3 at the dermal epidermal junction which was consistent with the diagnosis of bullous pemphigoid. No anti-intercellular deposits of immunoglobulin g (IgG) or C'3 were observed. We consider that suprabasal acantholysis may represent the early phase of bullous pemphigoid. ( info) |
| A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made. ( info) |
8/123. Pseudovascular squamous cell carcinoma of the skin. The presence of acantholysis in squamous cell carcinomas (SCC) may rarely be so extreme that, histologically, it mimics a vascular tumour. However, careful histological examination and immunohistochemical study usually lead to the correct diagnosis. We describe such a case to highlight the clinico-pathological features of this rare form of cutaneous malignancy and to emphasize the difficulties in establishing the correct diagnosis. We also review similar cases reported in the literature. Pseudovascular SCC shows a higher degree of recurrence and metastasis than other variants of SCC. Acantholytic foci in these tumours may demonstrate changes in keratinocyte differentiation markers, and this may explain the more aggresive biological behaviour in the pseudovascular variant of SCC. ( info) |
9/123. Type 2 segmental manifestation of Hailey-Hailey disease: poor therapeutic response to dermabrasion is due to severe involvement of adnexal structures. In autosomal dominant skin conditions, two different types of segmental manifestation can be distinguished. Type 1 represents heterozygosity for a postzygotic mutation, resulting in a degree of severity similar to that of the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and shows an excessively pronounced involvement superimposed on the ordinary nonsegmental phenotype. We describe the clinical, histopathological and therapeutic aspects of the first case of type 2 segmental manifestation of Hailey-Hailey disease (HHD). A 24-year-old woman with a family history of HHD comprising four generations, presented with lesions of erythema and blistering arranged in a unilateral pattern following the lines of Blaschko. The disorder was first noted at the age of 3 months. At the age of 24 years, additional scattered symmetrical lesions involving the axillary and inguinal folds were noted. Histopathological examination of the severely involved linear skin areas revealed pronounced acantholysis within the deep adnexal structures, whereas clinically unaffected skin showed the typical histopathological features of the heterozygous phenotype with suprabasal clefting and acantholysis sparing the adnexae. dermabrasion was performed in the areas of segmental involvement. During a follow-up period of one year, no recurrence was noted, but 18 months after dermabrasion a recurrence was present in the left submammary and left perianal regions. This therapeutic resistance to dermabrasion may be explained by the presence of acantholysis within the adnexal structures of the skin as found in type 2 segmental HHD. ( info) |
10/123. Benign persistent papular acantholytic and dyskeratotic eruption of the vulva: a case report. We report a case of 44-year-old woman with persistent pruritic papules on the left and right labium majus of the vulva. Histopathologic examination of the vulvar biopsy specimen revealed a suprabasal separation of the epidermis with acantholysis and dyskeratosis. ( info) |