Can Immunofluorescence on Skin/Mucosal Scraping Smear for Pemphigus Diagnosis Substitute Direct Immunofluorescence on Skin Biopsy?

Introduction: Few studies have been conducted on the use of Direct Immunofluorescence (DIF) on skin/mucosal scraping smear for diagnosis of pemphigus disease; however, the diagnostic value of DIF on the smear has not been fully evaluated. Objectives: The present study was carried out to assess the sensitivity and specificity of DIF on skin/mucosal smear for diagnose of pemphigus in the patients presenting with mucocutaneous erosive lesions. Methodology: A total of 89 patients including 40 males and 49 females aged between 23 and 80 years old with various bullous disorders were enrolled in the study. For definite diagnosis, all the patients were subjected to lesional biopsy for pathological studies and perilesional biopsy for DIF studies. In all the cases, skin/mucosal scraping smears were prepared from the perilesional healthy skin/mucosa and were stained with immunofluorescence conjugated anti-IgG. Results: Of 89 patients, 56 (63%) patients were diagnosed with pemphigus. Immunodeposits favoring the pemphigus were demonstrated in the 46 smears of 56 cases of pemphigus (sensitivity of 82%). No case with other types of bullous disease had positive DIF on the smear (specificity of 100%). Conclusion: The findings of the study showed that the sensitivity of DIF on the smear is not high enough to allow us replacing the conventional DIF with smear-DIF for diagnosis of pemphigus, while the specificity of 100% would allow the unequivocal identification of a subset of patients with pemphigus.

An extremely rare mucocutaneous adverse reaction following COVID-19 vaccination: Toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), is a type of delayed hypersensitivity reaction that requires urgent medical intervention. In the COVID-19 era, COVID-19 vaccines are currently being widely administered and mucocutaneous adverse reactions following vaccination have been reported; however, severe cutaneous adverse reactions associated with COVID-19 vaccines including SJS/TEN, are extremely rare. Herein, we describe a case of COVID-19 vaccination induced TEN which developed 1 day after receiving the first dose of Sinopharm COVID-19 vaccine with favorable clinical outcome.

The presence of mast cells in lichen planopilaris and discoid lupus erythematosus of the scalp: A quantitative study.

BACKGROUND: Histopathologic differentiation of lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) as two common causes of primary cicatricial alopecias remains challenging. METHOD: We performed a histopathologic study on a case series of LPP and DLE specimens to investigate the number, distribution, and morphology of mast cells as indices for differentiation of these two entities. H&E investigation and Giemsa staining for the detection of mast cells was performed. RESULT: A total of 74 cases comprising 50 cases of LPP and 24 cases of DLE were assessed. The mean mast cell count and percentage were significantly higher in LPP group (p < 0.001). Mean degranulated mast cell count and the mean intact mast cell count were also significantly higher in LPP patients (p < 0.001). Most of the specimens, 58 (78.4%), showed both perifollicular and perivascular distribution of mast cells without significant difference between two groups. The morphology of mast cells was predominantly round-oval in 85.5%, predominantly fusiform in 13.5% with more frequent fusiform morphology in DLE group. CONCLUSION: The mast cell count detected by Giemsa staining could assist pathologists in distinguishing between LPP and DLE.

Depigmentation of the vulvar area: Is it an alarming sign?

A 34-year-old female patient presented with recurrent bilateral hypopigmented macules on the labia majora. The lesions were treated with topical steroids, which led to mild improvement, but erosive plaques developed after discontinuing the treatment. Histopathological findings were compatible with extramammary Paget disease (EMPD), which was treated with radical vulvectomy with no recurrence in the next months of follow-up.

The diagnostic value of p63, p16, and p53 immunohistochemistry in distinguishing seborrheic keratosis, actinic keratosis, and Bowen's disease.

Seborrheic keratosis (SK), actinic keratosis (AK), and Bowen's disease (BD) are squamoproliferative disorders of the skin. Histologically, they may mimic each other and therefore, they might be misinterpreted, especially in small samples. The aim of this study is to clarify the expression of p63, p16, and p53 proteins in SK, AK, and BD and evaluate the efficacy of these markers in order to distinguish between the aforementioned lesions. A total of 46 cases were collected (15 SK, 16 AK, and 15 BD) and stained for p63, p16, and p53. The stain intensity and the cell distribution labeling were scored and then analyzed by SPSS software. All cases of BD which became positive for p53 revealed basal keratinocytes sparing. Instead, all or nearly all basal keratinocytes in AK cases were positive for this marker. These were also seen in p16 staining results and they were between AK and BD (P = .024). Our study demonstrates p16 and p53 are useful markers in separating AK and BD according to basal keratinocytes involvement and sparing, respectively.

Primary Kaposi sarcoma of the glans: A rare case in an HIV-negative patient.

First presentation of the Kaposi sarcoma (KS) on the penis is not prevalent, and it was reported in 2%-3% of the cases that mostly occurred in the HIV-positive patients. Here, we report a case of primary KS on the glans penis in an HIV-negative patient.

Development of keloidal morphea after treatment with cyclosporine in a case of recalcitrant generalized morphea.

Nodular or keloidal morphea, also known as nodular scleroderma, is a rare form of localized sclerosis (SSc) or morphea. In this paper, we reported a case of this rare entity with a review of the literature.

Acquired lymphangioma circumscriptum secondary to tuberculosis: A rare case report.

Lymphangioma circumscriptum is a benign lymphatic malformation that usually presents at birth or early childhood. Acquired oral lymphangioma has been reported secondary to radiation therapy and denture-induced trauma due to damage to previously normal lymphatics. To the best of our knowledge, this is the first report of acquired oral lymphangioma due to childhood tuberculous adenitis.

Bart syndrome associated with skeletal deformities: An uncommon case report.

Bart syndrome is a rare genetic disorder characterized by aplasia cutis congenita, epidermolysis bullosa (EB), and nail abnormalities. We reported an unusual case of Bart syndrome associated with skeletal abnormalities and bilateral clubfoot.

Coexistence of mucous membrane pemphigoid, vitiligo, and hypothyroidism: A second report of a new multiple autoimmune syndrome.

Mucous membrane pemphigoid (MMP) is a rare immunobullous disease affecting the mucous membrane and skin. An association of MMP with other autoimmune disease is uncommon. Multiple autoimmune syndrome (MAS) is a subclass of autoimmune disorders with a coexistence of three or more autoimmune disorder in a single patient. The second report of the association of vitiligo, autoimmune thyroid disease, and MMP shows that this combination can be considered as a new type of MAS, although its mechanism and pathology are unknown.

Using immunofluorescence (antigen) mapping in the diagnosis and classification of epidermolysis bullosa: a first report from Iran.

BACKGROUND: Immunofluorescence antigen mapping (IFM), is a newly introduced technique for diagnosis and classification of epidermolysis bullosa (EB) disease. The precise level of skin cleavage can be determined using monoclonal antibodies to EB-specific basement membrane zone protein. OBJECTIVE: To apply IFM technique in diagnosis and classification of EB and to identify utility and limitation of this method in our clinical setting. METHODS: IFM was done according to a described protocol by Pohla-Gubo et al. Monoclonal antibodies used for antigen mapping were against cytokeratin 5, cytokeratin 14, α6 integrin, ß4 integrin, laminin 332, Collagen IV, and Collagen VII. RESULTS: IFM was done for 95 referred patients, compromising 49 females and 46 males, aged 5 days to 45 years (mean = 9.5 years). Ninety cases were diagnosed with EB and classified as follows: EB simplex: (n = 13), junctional EB (n = 14), dystrophic EB (n = 62), and Kindler syndrome (n = 1). Diagnosis was not made in five cases as their specimens contained no blister. Confirmatory genetic analysis was done for five junctional cases from two families with clinical features of laryngo-onycho-cutaneous syndrome. Genetic molecular studies showed nonsense mutations in the last codon of exon 39 of the laminin α3a (LAMA3) gene (p.Gln57X) and a donor splice site mutation in LAMA3 (IVS57+5G>A) in the first and second family, respectively. CONCLUSION: IFM technique is relatively simple to perform, and interpretation of the results is not sophisticated. The proportion of inconclusive results will be decreased if the specimens contain freshly induced blister.

Disseminated Kaposi's Sarcoma with the Involvement of Penis in the Setting of HIV Infection.

Kaposi's sarcoma (KS) is a malignant proliferation of the endothelial cells. It typically presents with several vascular nodules on the skin and other organs. The penile localization of KS, particularly on the shaft area, is exceptional. We report an HIV-positive 34-year-old man who had multiple purplish-black plaques on his extremities and several small violaceous macules on the glans and shaft of the penis. Kaposi's sarcoma was diagnosed by histopathology.

Evaluation of elastic fibers pattern with orcein staining in differential diagnosis of lichen planopilaris and discoid lupus erythematosus.

Differential diagnosis of lichen planopilaris and discoid lupus erythematosus especially in late stages is a problem for clinicians and pathologists. Our aim was to find discriminator histopathologic findings that help us to achieve definite diagnosis without using immunofluorescence study. The histopathologic findings in 77 cases of lichen planopilaris were compared with those of 26 cases of discoid lupus erythematosus with Hematoxylin & Eosin and especially staining (Alcian blue pH 2.5, Periodic Acid Shiff, Orcein). Final histopathologic diagnosis was based on histologic findings, clinicopathological correlation, past medical history and immunofluorescence studies if were applied before. Then elastic fibers pattern in dermis and follicular sheath with orcein staining were described without having information about final diagnosis. New and subtle presentations of histologic changes were assessed. We compared all histopathologic finding for each staining method. Some histologic changes such as hypergranulosis, epidermal atrophy, mucin deposition, diffuse scar and some other patterns were not specific for any diagnosis. A setting of histopathologic findings and clinicopathological correlation were needed for accurate diagnosis. We had only one specimen for the vertical section, and we had no horizontal sections. Description of elastic fibers pattern in orcein staining may be helpful in achieving a specific diagnosis, but this is not completely reliable, and we had overlap features. Finally, immunofluorescence study may be recommended for suspicious cases.