OBJECTIVES: The aim of this study is to determine the effect of cannabinoid receptor (CB) 2 inhibitor on desmoglein 3 (DSG3) expression in HaCaT cells co-cultured with pemphigus serum. METHODS: Immunohistochemical staining was used to compare CB expression in pemphigus patients and normal individuals. Enzyme-linked immunosorbent assay (ELISA) was employed to quantify the concentration of CB2 in the serum of pemphigus patients and normal individuals. A correlation analysis was performed to examine the relationship between the serum CB2 and DSG of pemphigus patients. The CCK-8 assay was used to evaluate the inhibitory effect of AM630 on HaCaT cells, and the half-maximal inhibitory concentration (IC₅₀) value was utilized to determine the experimental concentration. Serum from normal individuals (negative control group) and pemphigus patients (pemphigus group) was co-cultured with HaCaT cells at a 1∶1 ratio. HaCaT cells cultured in complete medium were used as the control group. HaCaT cells in the pemphigus group treated with AM630 were employed as the AM630 group. Real-time polymerase chain reaction (PCR) and Western blot were conducted to assess the expression levels of CB2, DSG3, and β-catenin. Cell dissociation experiments were conducted to evaluate the effect of AM630 on the adhesion of HaCaT cells. RESULTS: Immunohistochemistry revealed significant differences in CB2 expression between pemphigus and normal mucosa (P<0.000 1), but no difference was found in CB1 expression. ELISA analysis revealed a statistically significant difference in the expression levels of CB2 in the serum between normal individuals and pemphigus patients (P<0.001). The expression of CB2 in the serum of pemphigus patients exhibited a significant positive correlation with that of DSG3 (r=0.831, P=0.003). The CCK-8 assay indicated that the IC₅₀ of AM630 on HaCaT cells was 0.55 μmol/L. Real-time PCR and Western blot showed that the expression levels of CB2 and DSG3 increased in the pemphigus group, while the expression level of β-catenin decreased compared with that in the AM630 groups (P<0.05). CONCLUSIONS CB2 is highly expressed in oral mucosal pemphigus. AM630 inhibits overexpression of CB2 and DSG3 and underexpression of β-catenin levels, which can provide new therapeutic targets for pemphigus.
Humans ; Pemphigus/pathology* ; Receptor, Cannabinoid, CB2/metabolism* ; Desmoglein 3/metabolism* ; Acantholysis/metabolism* ; Mouth Mucosa/pathology* ; HaCaT Cells ; Coculture Techniques ; beta Catenin/metabolism*

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.
Acantholysis ; Hot Temperature ; Humans ; Male ; Middle Aged ; Skin Diseases ; Solar System ; Sweat ; Sweating

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.
Acantholysis ; Biopsy ; Carbon Dioxide* ; Carbon* ; Darier Disease ; Diagnosis, Differential ; Epidermis ; Humans ; Incidence ; Lasers, Gas* ; Middle Aged ; Pemphigus, Benign Familial ; Skin

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.
Acantholysis ; Biopsy ; Eosinophils ; Humans ; Leg ; Lymphocytes ; Middle Aged ; Pruritus ; Skin ; Thigh

Grover disease (GD), also referred to as transient or persistent acantholytic dermatosis, is an acquired pruritic papular or papulovesicular eruption characterized histopathologically by focal acantholysis and dyskeratosis. Because GD resembles several generalized papular diseases, the diagnosis of GD is a clinical challenge, particularly when dermatologists meet patients without papular eruptions. We experienced two elderly men showing eczematoid plaques with severe pruritus on their backs. Histopathological examinations revealed a focal acantholysis, spongiosis, and dyskeratosis, a pattern consistent with GD. We report two cases of GD with clinical features of nonspecific eczematous dermatosis.
Acantholysis ; Aged ; Diagnosis ; Humans ; Male ; Pruritus ; Skin Diseases*

Grover disease (GD), also referred to as transient or persistent acantholytic dermatosis, is an acquired pruritic papular or papulovesicular eruption characterized histopathologically by focal acantholysis and dyskeratosis. Because GD resembles several generalized papular diseases, the diagnosis of GD is a clinical challenge, particularly when dermatologists meet patients without papular eruptions. We experienced two elderly men showing eczematoid plaques with severe pruritus on their backs. Histopathological examinations revealed a focal acantholysis, spongiosis, and dyskeratosis, a pattern consistent with GD. We report two cases of GD with clinical features of nonspecific eczematous dermatosis.
Acantholysis ; Aged ; Diagnosis ; Humans ; Male ; Pruritus ; Skin Diseases*

Darier's disease is an autosomal dominant acantholytic skin disorder. The disease is characterized by symmetric distribution of hyperkeratotic papules in seborrheic regions of the skin and nail changes with potential for exacerbation by ultraviolet light, heat, occlusion, or stress. It presents as generalized (hypertrophic or vesiculobullous type) or localized (linear or zosteriform type) condition. A 53-year-old male patient presented with hyperkeratotic verrucous papules on the back that had an onset in childhood. Clinically, his condition resembled lichen simplex chronicus. The lesions were exacerbated by sun exposure or sweating. A 3-mm punch biopsy specimen revealed prominent acantholysis with hyperkeratosis and parakeratosis. Numerous corps grains and corps ronds were present near the granular layer. We report a case of localized Darier's disease mimicking lichen simplex chronicus on the back. The skin lesion improved following treatment with a topical corticosteroid. Localized Darier's disease should be considered as one of the differential diagnoses of lichen simplex chronicus on the back.
Acantholysis ; Biopsy ; Edible Grain ; Darier Disease* ; Diagnosis, Differential ; Hot Temperature ; Humans ; Male ; Middle Aged ; Neurodermatitis* ; Parakeratosis ; Skin ; Solar System ; Sweat ; Sweating ; Ultraviolet Rays

PNP is a rare autoimmune mucocutaneous blistering disease associated with neoplasms, most frequently of the lymphoproliferative type. As PNP is clinically characterized by polymorphous mucosal lesions and cutaneous eruptions, it is important to differentiate it from erythema multiforme, Stevens-Johnson syndrome, lichen planus, and other bullous diseases. A diagnosis of PNP can be confirmed by immunologic studies such as direct and indirect immunofluorescence, immunoblotting, immunoprecipitation. Rare PNP cases related to nonhematological solid tumors have been reported. A 54-year-old male visited us with generalized pruritic scaly lichenoid lesions on the whole body from 5 weeks prior to his first visit. He also presented with extensive painful ulcers and erosions on the oral mucosa and lips for 2 months. Histopathologic findings showed lichenoid infiltration with vacuolar interface change, lichenoid interface dermatitis, keratinocyte apoptosis, and suprabasal acantholysis with cleft. Indirect immunofluorescence using normal human skin showed IgG deposition at the intercellular space. Immunoblotting using normal epidermal extracts in the serum of patient detected antibody to the 190 kDa (envoplakin), 210 kDa (periplakin) molecules polypeptides. He also had a hepatocellular carcinoma and chemoradiotherapy done before. The diagnosis of paraneoplastic pemphigus (PNP) was made. To our knowledge, there are only two reports of PNP associated with hepatocellular carcinoma worldwide, yet no report in Korean literature. Herein, we report the first case of PNP associated with hepatocellular carcinoma in Korea.
Acantholysis ; Apoptosis ; Blister ; Carcinoma, Hepatocellular* ; Chemoradiotherapy ; Dermatitis ; Diagnosis ; Erythema Multiforme ; Extracellular Space ; Fluorescent Antibody Technique, Indirect ; Humans ; Immunoblotting ; Immunoglobulin G ; Immunoprecipitation ; Keratinocytes ; Korea ; Lichen Planus ; Lip ; Male ; Middle Aged ; Mouth Mucosa ; Pemphigus* ; Peptides ; Skin ; Stevens-Johnson Syndrome ; Ulcer

Herpes zoster duplex, particularly herpes zoster duplex symmetricus, is a very rare manifestation of herpes zoster that occurs in immunocompetent patients. A 23-year-old Korean man presented with grouped erythematous, ruptured, crusted vesicles on plaques and papules on his forehead and both temporal areas. He had unexplained pain around the cutaneous lesions for the past 2 weeks, and the vesicles developed 1 week ago. A Tzanck smear test of the skin lesion showed multinucleated giant cells. A punch biopsy taken from the vesicle on the forehead revealed an intraepidermal vesicle with acantholysis and balloon cells at the floor of the vesicle. He was diagnosed with herpes zoster involving bilateral dermatomes.
Acantholysis ; Biopsy ; Floors and Floorcoverings ; Forehead ; Giant Cells ; Herpes Zoster ; Humans ; Skin ; Young Adult

Pemphigus is a group of blistering autoimmune diseases that affect the skin and mucous membranes with autoantibodies against desmoglein. Among the pemphigus subtypes, pemphigus erythematosus is one of the rare lupus-like variant subtypes of pemphigus that shares common clinical and histopathological manifestations with systemic lupus erythematosus. The lesions appear clinically as erythematous erosive patches on the face in a butterfly distribution, and they manifest subcorneal acantholysis histopathologically. There is concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis. Two cases of pemphigus erythematosus have been reported domestically. Here, we report a case of pemphigus erythematosus in an 18-year old woman.
Acantholysis ; Autoantibodies ; Autoimmune Diseases ; Basement Membrane ; Blister ; Butterflies ; Complement System Proteins ; Desmogleins ; Epidermis ; Extracellular Space ; Female ; Humans ; Immunoglobulins ; Lupus Erythematosus, Systemic ; Mucous Membrane ; Pemphigus ; Skin