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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.

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.

We report two cases of lichen planus following COVID-19 vaccination in two middle-aged women, where the first patient presented with lichenplanopilaris (LPP) relapse and development of lichen planus 14 days after the second dose of AstraZeneca vaccine, and the other patient who had a previous scattered lesion of LP which extended and increased in severity after the first and second dose of Sinopharm. The suggested cause could be due to immune dysregulation and up regulation of T cell lymphocytes which was triggered after COVID-19 vaccination. What supports our hypothesis that LP had occurred due to COVID-19 vaccination, is that one of the patients responded successfully to Metronidazole. This means that the infection process after vaccination could be the cause in aggravating LP. To add, one of the suggested mechanisms for the appearance of LP or reactivation of a dormant LPP can be cytotoxic CD8 T-lymphocytes which increase the secretion of IFN-γ and IL-5 cytokines and may also result in basal keratinocytes' apoptosis leading to cutaneous manifestations. This was supported by the efficacy of Tofacitinib that was used in the other patient who presented with reactivation of LPP in addition to LP. Tofacitinib decrease the number of T cell infiltration and adjust IFN expression.

Histopathologic differentiation of lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) as two common causes of primary cicatricial alopecias remains challenging.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. HE investigation and Giemsa staining for the detection of mast cells was performed.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.The mast cell count detected by Giemsa staining could assist pathologists in distinguishing between LPP and DLE.

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.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.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.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%).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.

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: to assess the sensitivity and specificity of DIF on skin/mucosal smear for diagnose of pemphigus in the patients presenting with mucocutaneous erosive lesions. Methods: 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.

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.

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.

A 44-year-old female presented to the office with several months of hypopigmented patches on her face and extremities (Fig. 45.1). Wood’s lamp examination of the lesions revealed a chalky white enhancement. She had a history of hypothyroidism diagnosed 13 years ago which was under control with levothyroxine.

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 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.

Papulonecrotic tuberculid (PNT) is a relatively rare subtype of cutaneous tuberculosis believed to represent a hypersensitivity reaction to tuberculosis antigens. Yet, the simultaneous occurrence of PNT and Erythema Induratum (EI) is even more uncommon. Here, we present a case of concomitant PNT and EI in a 53-year-old woman without any previous history of Mycobacterium tuberculosis infection who was successfully treated with anti-tuberculosis agents without recurrence in a subsequent follow-up

Dear Editor-in-ChiefMalignant melanoma is a tumor arising from melanocyte; this tumor rarely occurs before puberty, with higher mortality rate in males and better survival rate in female patients affected by metastatic melanoma (1, 2). These facts propose that a relationship and association may exist between estrogens and melanoma. The effects of estrogens are mediated by estrogen receptor alpha and beta (3) that are members of the nuclear hormone receptor family. Estrogen receptors act by ligand-dependent binding to the estrogen-response element, leading to transcriptional regulation of target genes (4). Although these receptors have a high degree of homology in the DNA-binding domain, they are different in their N-terminal and ligand-binding domain (E-domain) (5). Moreover; the effects of these two receptors are also different, while estrogen receptor alpha is associated with stimulation of growth. Estrogen receptor beta (ERbeta) is associated with suppression of stimulation or inhibition of cells from multiplying (2). A number of reports show either a decreased expression of ERbeta messenger RNA and ERbeta protein or an increased estrogen receptor alpha/beta mRNA ratio in tumor versus normal tissue in several cancers such as breast, ovary, colon, and prostate (6, 7). As the expression of ERbeta in melanocytic lesions is controversial and finding new diagnostic methods to differentiate between benign and malignant melanocytic lesions is essential, the current study was conducted using immunohistochemical staining to characterize the expression of ERbeta in dysplastic nevi and melanoma. The expression of ERbeta was investigated in 10 patients with melanoma (five male and five female) and 10 patients with dysplastic nevi (seven male and three female) at the Department of Dermatology, Shahid Beheshti University of Medical Science, Tehran, Iran. All cases underwent immunohistochemical analysis according to the method described by de Giorgi et al. (2, 8). Only one of the patients with melanoma had ERbeta expression of grade III and the other nine patients had grade I, but all the dysplastic nevi had grade III staining. Comparison of melanocytes staining levels in the two mentioned groups with the Mann-Whitney U-test revealed a significant difference between estrogen receptor beta staining samples (P-value=0.0002). Results of the current study suggested a probable role for estrogen receptors in melanoma; in addition, it proposed ERbeta as a valuable diagnostic marker to differentiate between benign and malignant melanocytic lesions; however, according to the relatively small number of patients, further comprehensive studies should be conducted to confirm the current studyresults.

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, β4 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.

Summary Laryngo-onycho-cutaneous (LOC) syndrome is a subtype of autosomal recessive junctional epidermolysis bullosa in which there is prominent skin and mucosal granulation tissue that can lead to delayed wound healing, laryngeal obstruction and blindness. Thus far, all cases are of Punjabi ancestry and have been shown to result from a founder mutation in the LAMA3 gene, notably involving a single nucleotide insertion mutation in exon 39, which is specific to the LAMA3A (designated exon 1 of LAMA3A) and not the LAMA3B1 or LAMA3B2 isoforms. Here, we describe a new pedigree with LOC syndrome. Affected individuals (from Iran) have the characteristic clinicopathological and molecular features of LOC syndrome: prominent granulation tissue (especially affecting the eyes), normal intensity laminin-332 immunostaining at the dermal–epidermal junction, and autosomal recessive mutations in the LAMA3A-specific exon. The pathogenic mutation is a homozygous nonsense mutation, designated p.Gln57X, which just affects the laminin-α3a transcript. These findings therefore expand the molecular basis of LOC syndrome.

Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. The patient had a favourable response following treatment with a low dose of systemic steroid, mycophenolate mofetil, and intravenous immunoglobulin (IVIG). He subsequently underwent surgery for resection of the tumor with nephrectomy and five courses of IVIG were administered after surgery due to a minor relapse. He was in remission in her last follow-up visit 16 months after surgery. The occurrence of PNP with IMT is notable; early detection and treatment are crucial for this tumor-associated autoimmune disease.

Granuloma faciale (GF) is a rare cutaneous disorder categorized as a localized form of small vessel vasculitis. Clinically, it manifests as single or multiple, well-demarcated, red-brown plaques, papules and nodules, nearly always confined to the face. Herein, we report a 39-year-old man with multiple red-brown, infiltrated plaques on his face and extrafacial lesions on the back, shoulders, and both arms. Skin biopsy revealed typical histopathological findings of GF. The patient failed to respond to pulsed dye laser, but intralesional triamcinolone combined with cryotherapy led to an acceptable response.

Epidermodysplasia verruciformis (EV) is a rare genodermatosis associated with a high risk of skin cancer. In this report, we present three Iranian brothers and their mother with extensive seborrheic keratosis-like (SK-like) viral warts. Initial facial lesions developed in the first decade and disseminated with time. The patients showed SK-like viral warts characterized by dark brown or black pigmented proliferative lesions with hyperkeratotic surfaces. The histopathological findings were consistent with the diagnosis of EV. There are few reports of familial epidermodysplasia verruciformis especially in a mother and her three sons.

A 70-year-old man presents with multiple asymptomatic, yellow to skin-colored firm papules scattered on the scalp; the lesions had been present for about 30 years. Histological findings are consistent with the diagnosis of steatocystoma. We review the English-language literature of the published cases of localized forms of steatocystoma multiplex.

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.

Cutaneous leiomyoma is a benign dermal tumor of smooth muscle. Leiomyoma can occur at any age but typically develops during adolescence or early adult life. Congenital leiomyoma is an extremely rare reported variant. A five-month-old boy presented to our clinic with a progressively enlarging mass on his occipital scalp. Microscopic examination of the solitary ulcerated nodule showed a non-encapsulated mass in the dermis composed of spindle-shaped cells. The diagnosis was confirmed by immunohistochemical studies, which were compatible with leiomyoma. Therefore, clinical suspicion of leiomyoma should be considered in the case of a solitary ulcerated nodule in an infant.