Acantholytic oral squamous cell carcinoma with clear cell change - a rare amalgamated variant

ABSTRACT Background Acantholytic squamous cell carcinoma (ASCC) is an uncommon histological variation of oral squamous cell carcinoma (OSCC), accounting for fewer than 4% of all occurrences. The tumor shows a slight masculine predisposition, with the lower lip being the most commonly affected location. ASCC is reported to have a diverse biologic behavior, which explains its ability to metastasize to distant places and, thus, its poor prognosis. Similarly, clear cell change in OSCC is a rare occurrence with an unknown etiology that suggests its aggressive nature. Method and Results Histopathology reveals central acantholytic cells with numerous duct-like features. The presence of distinct cytological atypia contributes to the diagnosis of SCC. Special stains and IHC aid in distinguishing tumor from other histopathologically similar entities. Conclusion The case of a 29-year-old male presented here with an updated literature review highlights the need for histological study of the unique and seldom seen oral ASCC with clear cell change, which can be ignored because of similarities with other entities. Because recurrence rates are so high for ASCC, amalgamated clear cell change makes it critical for proper treatment initiation with a definite diagnosis. To the best of our knowledge, this is the first documented occurrence. Our experience with the present case suspected a more aggressive behavior due to a high Ki-67 index, anticipating a poorer prognosis in the oral cavity considering the patient's young age.

Case for diagnosis. Atypical Grover's disease

Abstract A 55-year-old male presented with an eight-month history of erythematous papules and plaques with demarcated areas of spared skin on his trunk, upper extremities, neck, and face. Grover's disease is a rare, acquired disorder of unknown origin, which is classically characterized by the appearance of erythematous papules on the upper trunk that are usually transient. As in the present case, there are reports of atypical disease, with facial involvement, pityriasis rubra pilaris-like lesions, and a more chronic course.

A Case of Persistent Acantholytic Dermatosis Presenting as a Chronic Single Patch / 대한피부과학회지

Transient acantholytic dermatosis, or Grover's disease, usually appears as pruritic erythematous papules and vesicles on the trunk in middle-aged men. The pathogenesis remains unclear, though sun exposure, heat, and sweating may be aggravating factors. A 58-year-old male visited our clinic for evaluation of an asymptomatic erythematous patch on the left temple that developed 40 years ago. Here, we report the rare case of Grover's disease with atypical features presenting as one large patch on the face.

Enfermedades acantolíticas: caracterización de pacientes con diagnóstico de enfermedad de Hailey-Hailey, enfermedad de Darier y enfermedad de Grover, entre los años 2007 y 2017 en el Hospital Clínico San Borja Arriaran y revisión de la literatura / Characterization of patients diagnosed with Hailey-Hailey disease: Darier's disease and Grover's disease, between 2007 and 2017 at the San Borja Arriaran Clinical Hospital and review of the literature

INTRODUCCIÓN: Las enfermedades acatólicas son un grupo heterogéneo de enfermedades que presentan como característica central histopatológica la acantosis. Generalmente presentan un curso de evolución crónica y recidivante, con variadas manifestaciones clínicas. OBJETIVO: caracterizar los pacientes con diagnóstico de enfermedad acantolítica, bajo 5 criterios clínicos y realizar una revisión de la literatura. MÉTODOS: Se realizó una revisión de la base de datos del Servicio Anatomía Patológica del Hospital San Borja Arriaran (HSBA) entre los años 2007 y 2017 y se complementaron con los antecedentes clínicos extraídos de las fichas clínicas.RESULTADOS: Se obtuvo un total de 13 casos. el 53,8% correspondieron a enfermedad de Darier, 20,6% a enfermedad de Hailey-Hailey y un 20,6% a enfermedad de Grover, obteniendo un promedio de edad al momento del diagnóstico de 22,5 años, 44,3 años y 47,6 años respectivamente. Los antecedentes familiares estuvieron presentes en el 53,8% del total de pacientes, ninguno de ellos presentaba estudio genético. El 61,5% de la muestra correspondió a pacientes de sexo femenino y el promedio de años de evolución previo al diagnóstico fue de 7,4 años para Darier, 8,6 para Hailey-Hailey y para Grover. El 100% de los pacientes con enfermedad de Darier y Grover estaban con terapia sistémica y el 66,6% de enfermedad de Hailey-Hailey con terapia tópica, todos con adecuada respuesta clínica. DISCUSIÓN y conclusiones: las enfermedades acantolíticas corresponden a genodermatosis poco frecuente cuyo diagnóstico y tratamiento constitu-yen un desafío para el dermatólogo

INTRODUCTION: Atytolic diseases are a heterogeneous group of diseases that present acanthosis as a histopathological central characteristic. They usually present a course of chronic and recurrent evolution, with varied clinical manifestations. OBJECTIVE: to characterize the patients diagnosed with acantholytic disease, with 5 clinical criteria and to carry out a review of the literature. METHODS: A review of the database of the Pathological Anatomy Service of the San Bor-ja Arriaran Hospital (HSBA) between 2007 and 2017 was carried out and complemented with the clinical records extracted from the clinical files. RESULTS: A total of 13 cases were obtained. 53.8% corresponded to Darier's disease, 20.6% to Hailey-Hailey's disease and 20.6% to Grover's disease, obtaining an average age at diagnosis of 22.5 years, 44.3 years and 47.6 years respectively. Family history was present in 53.8% of the to-tal patients, none of them had a genetic study. 61.5% of the sample corresponded to female patients and the average of years of evolution prior to diagnosis was 7.4 years for Darier, 8.6 for Hailey-Hailey and for Grover. 100% of the pa-tients with Darier and Grover's disease were on systemic therapy and 66.6% of Hailey-Hailey's disease with topical therapy, all with adequate clinical response. DISCUSSION AND CONCLUSIONS: acantholytic diseases correspond to rare genodermatosis whose diagnosis and treatment constitute a challenge for the dermatologist.

A Case of Papular Acantholytic Dyskeratosis Treated with Carbon Dioxide Laser / 대한피부과학회지

Papular acantholytic dyskeratosis is a collection of papular skin lesions that occur in the intertriginous and genital area. They show a characteristic histology of focal suprabasal acantholysis that distinguishes it from Hailey-Hailey disease or Darier disease. We describe a 50-year-old man with an asymptomatic papular eruption on the perianal area for several years. Histologically, a biopsy specimen showed diffuse hyperkeratosis and irregular acantholysis throughout the epidermis. We used carbon dioxide laser therapy as a therapeutic option. Despite causing a long and painful healing process, a considerable reduction of the symptoms was achieved. Although we do not know the precise nature or the incidence of this disease, papular acantholytic dyskeratosis should be included in the differential diagnosis of verrucous papules in perineal or perianal areas and carbon dioxide laser may represent a good therapeutic option.

A Case of Linear Grover Disease Distributed along the Blaschko Line / 대한피부과학회지

Grover disease (also known as transient or persistent acantholytic dermatosis) is a pruritic polymorphic papulovesicular eruption that is histologically characterized by the presence of epidermal acantholysis. It primarily occurs in middle-aged individuals and manifests as scattered erythematous or brown papules as well as papulovesicles on the sun-exposed skin of the trunk. A 52-year-old man had erythematous papules and patches linearly arranged on the left thigh and leg with mild pruritus. The skin lesions were successfully treated with a topical corticosteroid. However, 2 months later, the lesions recurred. The histological examination of a punch biopsy revealed focal acantholytic clefts with dyskeratotic cells, hyperkeratosis, and the infiltration of perivascular lymphocytes and eosinophils. Taken together with the late onset and lack of family history, we diagnosed this condition as Grover disease distributed along the Blaschko line, a condition presented here for the first time.

Enfermedad de Hailey- Hailey: reporte de casos entre los años 2001 y 2016 en un hospital universitario y revisión de la literatura / Hailey-Hailey disease: case report among the years 2001 and 2016 in a university hospital and review of the literature

Introducción: La enfermedad de Hailey-Hailey (pénfigo crónico familiar benigno) es una dermatosis ampollar, de evolución crónica, recidivante, autosómica dominante, de penetrancia y expresividad variables, localizada predominantemente en los pliegues. Métodos: Se realizó una revisión de la base de datos del Servicio Anatomía Patológica del Hospital Clínico Universidad de Chile (HCUCH) entre los años 2001 y 2016 y se complementaron con los antecedentes clínicos extraídos de las fichas clínicas. Resultados: Se obtuvo un total de 6 casos ilustrativos de la patología en estudio. Discusión y conclusiones: La enfermedad de Hailey-Hailey es una genodermatosis poco frecuente cuyo diagnóstico y tratamiento constituyen un desafío para el dermatólogo.

Background: The Hailey-Hailey disease (familial benign chronic pemphigus) is a bullous dermatosis, with chronic and recurrent evolution, autosomal dominant with variable penetrance and expressivity, localized predominantly in the folds. Methods: A review of the database of the Anatomic Pathology Service of the Hospital Clínico Universidad de Chile (HCUCH) between 2001 and 2016 was carried out and supplemented with the clinical history extracted from the clinical records. Results: A total of 6 illustrative cases of the pathology under study were obtained. Discussion and conclusions: Hailey-Hailey's disease is a rare genodermatosis whose diagnosis and treatment is a challenge for the dermatologist.

Enfermedad de Hailey-Hailey: presentación de dos casos / Hailey-Hailey disease: report of two cases

La enfermedad de Hailey-Hailey es un trastorno autosómico dominante, caracterizado por acantólisis de los queratinocitos. Se produce por mutaciones en el gen ATP2C1, que codifica la bomba de Ca++/Mn++ del aparato de Golgi. Clínicamente, se presenta como placas eritematosas con erosiones y fisuras principalmente en pliegues. Es una enfermedad crónica que cursa en brotes. No existen tratamientos específicos ni totalmente efectivos. Se utilizan corticoides y antibióticos tópicos, terapias sistémicas, quirúrgicas y físicas. Se presenta el caso de una paciente con enfermedad auto-limitada y otra, con lesiones extensas con mala adherencia al tratamiento y poca respuesta al mismo.

Hailey-Hailey disease is an autosomal dominant disorder characterized by acantholysis. It is caused by mutations of ATP2C1 gene encoding the secretory pathway Ca++/Mn++ ATPase localized in Golgi apparatus. It usually presents as erythematous, erosive plaques with fissures at folds and sites of friction. The course is chronic, with relapses and remissions. Treatment options are neither specific nor totally effective for this disease. Topical corticosteroids in combination with antibiotics, systemic, surgical and physical therapy can be used. We report a female patient with self-limited disorder and another one with extensive disease, poor adherence and little response to treatment.

Comments to "Localized Darier's Disease Mimicking Lichen Simplex Chronicus on the Back" / 대한피부과학회지

No abstract available.

Acantholytic Anaplastic Extramammary Paget's Disease: A Case Report and Review of the Literature

Extramammary Paget's disease (EMPD) is an uncommon intraepithelial neoplasm that most commonly arises on the vulva and perianal region. Very few cases of EMPD revealing a histological Bowenoid appearance have been reported. This study describes scrotal EMPD presenting with histological features of Bowen's disease in a 79-year-old man. He presented with a 5-year history of a pruritic erythematous plaque and a verrucous papule on the scrotum. The verrucous papule histopathologically showed Bowenoid features, and the erythematous plaque demonstrated acantholytic EMPD. Immunohistochemical findings revealed strong expression for carcinoembryonic antigen, Cam 5.2, epithelial membrane antigen, cytokeratin (CK) 7, and pancytokeratin (AE1/AE3) in both areas, but negative CK20 staining, supporting the overall diagnosis of primary acantholytic anaplastic EMPD. This is the first reported case of acantholytic anaplastic EMPD in the Korean literature.

Acantholytic Acanthoma with an Atypical Presentation / 대한피부과학회지

Acantholytic acanthoma is a rare benign neoplasm of epidermal keratinocytes and this commonly displays as a solitary, asymptomatic keratotic papule on the trunk. The histologic features are hyperkeratosis, acanthosis, papillomatosis and prominent acantholysis. Dyskeratotic cells are occasionally present, but prominent dyskeratosis is not a characteristic feature of acantholytic acanthoma. Herein, we report on a case of acantholytic acanthoma with several dyskeratoses, and this occurred in a 54-year-old woman who presented with a keratotic plaque that repeatedly pealed on the umbilicus.

Acantholytic Squamous Cell Carcinoma on the Right Foot / 대한피부과학회지

Acantholytic squamous cell carcinoma is a well-defined, rare variant of squamous cell carcinoma in which significant portions of the neoplastic tissue show, on microscopy, a pseudoglandular or tubular pattern. It usually presents in aged patients as an erythematous nodule with scales, crust, and ulceration on sun-exposed areas. Herein we report a case of acantholytic squamous cell carcinoma that occurred on the Rt 4th toeweb of a 70-year-old man.

Pyogenic Granuloma Showing Incidental Focal Acantholytic Dyskeratosis / 대한피부과학회지

Incidental focal acantholytic dyskeratosis (IFAD) has been described for various cutaneous lesions. A 58-year-old man presented with a purple colored, 5 mm crusted papule on the back. An excisional biopsy was performed and histopathological examination showed angiomatous tissue occurring in a discrete mass. It was enclosed by a collarette of acanthotic epidermis. The angiomatous tissue comprised a variably dilated network of blood-filled capillaries, which was surrounded by myxoid stroma. Hyperkeratosis, acantholysis, dyskeratosis and intraepidermal clefts were also focally present. Correlation of the clinical features and the histopathology results established a diagnosis of pyogenic granuloma showing IFAD.

A Case of Acantholytic Squamous Cell Carcinoma

Acantholytic squamous cell carcinoma is a well-defined variant of squamous cell cancer in which significant portions of the neoplastic proliferation show a pseudoglandular or tubular microscopic pattern. It usually presents as a nodule with various colors, and it is accompanied by scaling, crusting, and ulceration on the sun-exposed areas of older aged individuals. Histologically, the tumor consists of a nodular, epidermal-derived proliferation that forms island-like structures. At least focally or sometimes extensively, the tumor cells shows a loss of cohesion within the central gland-like or tubular spaces. This tumor resembles the structure of eccrine neoplasms, but it is negative for dPAS, CEA and mucicarmine and it is only positive for EMA and cytokeratins. Herein we report a case of acantholytic squamous cell carcinoma that occurred on the face of an 82-year-old woman.

A Case of Unilateral Darier's Disease; Acantholytic Dyskeratotic Epidermal Nevus? / 대한피부과학회지

Darier's disease is an uncommon genodermatosis, characterized by the symmetric eruption of keratotic papules, focal loss of epithelial adhesion and abnormal keratinization. A unilateral, or localized variant has been identified as a mosaic form, involving linear or 'zosteriform' epidermal lesions with clinical and histological features of Darier's disease. Some suggest that unilateral lesions should be classified as acantholytic dyskeratotic epidermal nevi, rather than localized Darier disease. A 17-year-old male presented with unilateral zosteriform distribution of brownish hyperkeratotic papules with ipsilateral v-notching of the thumb nail. A biopsy specimen revealed acantholysis, suprabasal cleft and multiple dyskeratotic cells. We report a case of unilateral Darier's disease which further supports unilateral dermatologic manifestation as a variant of Darier's disease.

A Case of Acantholytic Acanthoma / 대한피부과학회지

Acantholytic acanthoma is a benign neoplasm of the epidermal keratinocytes. It presents as a solitary, asympomatic, keratotic papule or nodule. The histopathologic features are outstanding acantholysis throughout the malpighian layer, hyperkeratosis, papillomatosis, and acanthosis. It is usually seen in older men, most commonly on the trunk. It has a tendency to be diagnosed clinically as a keratosis. Only 4 cases of acantholytic acanthoma have been reported in Korea. We report a case of acantholytic acanthoma in a 70-year-old man who presented with keratotic papules on his back.

A Case of Isolated Acantholytic Acanthoma / 대한피부과학회지

A solitary acantholytic acanthoma is a benign tumor which presents as a solitary asymptomatic, keratotic papule or nodule. The acantholysis, implied in the term, is the histologic hallmark. It may mimic pemphigus vulgaris, pemphigus vegetans, or Hailey-Hailey disease, but displays no evidence of these disorders. We report a case of isolated acantholytic acanthoma in a 78-year-old man, where the histologic feature is focal acantholytic dyskeratosis.

A Case of Acantholytic Dyskeratotic Epidermal Nevus / 대한피부과학회지

Acantholytic dyskeratosis is a regular histologic feature in Darier's disease, transient acantholytic dermatosis and warty dyskeratoma. It is also occasionally observed as an incidental histological finding in linear epidermal nevus. We report a case of acantholytic dyskeratotic epidermal nevus in an 11-month-old boy. The patient showed unilateral, brown colored verrucous plaque on the right shin. Histological finding showed hyperkeratosis, irregular acanthosis & focal acantholytic dyskeratosis.

A Case of Focal Acantholytic Dyskeratosis Presenting as a Solitary Papule / 대한피부과학회지

Focal acantholytic dyskeratosis (FAD) was introduced by Ackerman to describe a group of clinically various dermatologic conditions having a similar histologic appearance. The characteristic histologic findings included (1) suprabasilar clefts around preserved papillae, (2) acantholytic and dyskeratotic cells at all levels of the epidermis, and (3) hyperkeratosis and parakeratosis. Almost solitary lesions of FAD are histologically incidental or clinically nodular(warty dyskeratoma). The papular form of solitary FAD is an extremely rare condition identified by the reviewing of specimens or reported as a solitary lesion developing in the mouth. We report a case of focal acantholytic dyskeratosis presenting as a solitary papule on the perianal area.

A Case of Focal Acantholytic Dyskeratosis Occurring on both the Lip and the Anal Canal

Focal acantholytic dyskeratosis has a distinctive histological pattern that is associated with various clinical expressions. It rarely occurs on the lip or the perianal area. We report a patient with focal acantholytic dyskeratosis occurring on both the upper lip and the anal canal. Histopathologically, the lesions showed hyperkeratosis, suprabasilar clefting, epidermal acantholysis and dyskeratosis. This case represents the first report of a focal acantholytic dyskeratosis occurring on both the lip and the anal canal.