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A congenital melanocytic nevus is a benign melanocyte proliferation, that may be complicated by malignant transformation. We are reporting a three-year-old girl, who had a giant congenital melanocytic nevus on her back, that was treated by serial surgical excisions with tissue expander insertion. Histopathological examination confirmed the diagnosis of congenital melanocytic nevus with ganglioneuroma. Out of approximately 250 case reports on congenital melanocytic nevus, we identified only two reports of medium/large congenital melanocytic nevus with cutaneous ganglioneuroma. Due to the potential malignant transformation of congenital melanocytic nevus, reporting the features and characteristics of such rare findings may help in further understanding congenital melanocytic nevus, its associations, and prognosis.

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OBJECTIVES: To determine the distinctive clinical and pathological characteristics and outcomes of MF in a Saudi population. METHODS: We conducted a retrospective analysis of all MF cases diagnosed clinically with pathological confirmation at King Abdulaziz Medical City in Riyadh from January 2016 to July 2022. Variables include demographical, clinical, and pathological MF traits and disease outcomes. RESULTS: A sum of 73 patients were enrolled. The mean age was 44 years. The female-to-male ratio was 1.3:1.The mean duration between cutaneous manifestations and MF diagnosis was 33 months (2.7 years). Classic MF was the most common variant (60.3%), followed by hypopigmented MF (20.5%). Most patients (82.2%) had early-stage MF (IA, IB, and IIA). Patients who had CD4+/CD8+ with CD8 predominance had a favorable disease course (p=0.029). Topical corticosteroids were the most frequently prescribed treatment (79.5%). Three patients (4.1%) died from MF. The disease-specific survival rate for advanced-stage MF was 84.6%, which was significantly lower compared to early-stage MF (p=0.032). CONCLUSION: Among the Saudi population, MF has an earlier onset and slightly higher prevalence in females. Hypopigmented MF is more prevalent in this ethnic group. Immunohistochemical staining of CD4+/CD8+ with CD8 predominance may elucidate a favorable disease course.

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Malignant peritoneal mesothelioma (MPM) is a rare malignancy, presenting with non-specific and potentially-misleading manifestations. It represents a diagnostic pitfall as it mimics ovarian carcinoma. Maintaining a low diagnostic threshold, obtaining a detailed history, and utilizing immunohistochemical markers to diagnose MPM is crucial as early diagnosis and treatment might improve survival.

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Kaposi sarcoma is relatively common after solid organ transplantation, but very rare after hematopoietic stem cell transplant (HSCT). Here we are reporting a rare case of Kaposi sarcoma in a child after HSCT. An 11-year-old boy with Fanconi anemia was treated by haploidentical HSCT from his father. Three weeks after transplantation, the patient developed severe graft-versus-host disease (GVHD) which was treated by immunosuppressive therapy and extracorporeal photopheresis. Approximately 6.5 months after HSCT, the patient had asymptomatic nodular skin lesions over the scalp, chest, and face. Histopathological examination showed typical findings of Kaposi sarcoma. Later, additional lesions in the liver and oral cavity were confirmed. Liver biopsy was positive for HHV-8 antibodies. The patient was continued on Sirolimus which was already being used for the treatment of GVHD. Cutaneous lesions were also treated with topical timolol 0.5% ophthalmic solution. Within six months, cutaneous and mucous membrane lesions were completely resolved. Follow-up abdominal ultrasound and MRI showed the disappearance of the hepatic lesion.

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Monoclonal plasma cells form the solitary neoplasm known as solitary plasmacytoma. Isolated extramedullary plasmacytoma is less common than solitary bone plasmacytoma. An elderly male presented with coughing blood and was diagnosed with pharyngeal plasmacytoma with synchronous multiple myeloma. Herein, we present this challengingly rare case to increase awareness of this unusual entity.

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Interleukin-12 RB1 (IL12RB1) deficiency falls under the Mendelian susceptibility to mycobacterial disease. It is a rare genetic disease with autosomal recessive inheritance. It is characterized by recurrent infections with otherwise weak bacteria, such as mycobacteria and Salmonella. Often, when encountering a maculopapular eruption, a drug-related cause comes to mind. However, we report a case of IL12RB1 deficiency presenting with a maculopapular eruption, proven by a skin biopsy to be leukocytoclastic vasculitis. The patient was given antibiotics, which improved her skin lesions. Vasculitis should be considered in the differential diagnosis in patients with IL12RB1 deficiency presenting with a cutaneous eruption.

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Syringoma is a benign adnexal tumor of eccrine origin. Eruptive syringoma (ES) is a rare variant and can mimic other diseases especially lichen planus (LP). The dermoscopic observation of ES is not well described. We report here a case of ES, initially misdiagnosed as LP. The dermoscopic findings showed fine reticulate brown lines on a light brown background contrarily to the findings usually seen in LP. These findings prompt a skin biopsy which subsequently confirmed the diagnosis of ES. The use of dermoscopy in ES can be useful and may help differentiate it from other diseases.

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Sebaceous carcinoma is a rare malignant cutaneous neoplasm that is most commonly arises in the ocular region. Although it can occur in extraocular sites, sebaceous carcinoma is rarely encountered in the vulva. The use of immunohistochemical staining is very crucial to exclude other differential diagnoses including primary cutaneous and metastatic neoplasms. Unlike ocular sebaceous carcinoma, little is known about the clinical behavior and the prognostic factors in vulvar sebaceous carcinoma. Herein, we present a case of vulvar sebaceous carcinoma in a 27-year-old female, who presented with a labial tumor with lung metastases. To the best of our knowledge, only 11 similar cases were previously reported in the literature.

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Erythema elevated diutinum (EED) is a rare distinctive form of cutaneous leukocytoclastic vasculitis. EED typically presents with asymptomatic symmetrical erythematous-brown papules, nodules or plaques which favor the extensor aspect of extremities while distinctly sparing the palms. We report two cases of EED with a rare presentation limited to the palms.

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Rhabdomyosarcoma (RMS) is a soft tissue sarcoma that histologically resembles embryonic skeletal muscle. It is a rare malignancy in adults with a predilection in the head and neck, the genitourinary tract, and other extremities. However, RMS of the gastrointestinal tract is an even rarer condition that merits presentation and discussion. Here, we report on a case of primary duodenal embryonal RMS in an adult.

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BACKGROUND: Grover disease is an entity whose diagnosis is based on clinicopathologic correlation. Histopathologically, focal acantholysis is the most common finding. In some cases, there is prominent squamous atypia which can prove to be very challenging and the lesion may be confused with an epidermal neoplasm. OBJECTIVE: To report on atypical histopathological changes in Grover disease and to provide helpful clues to differentiate between the epidermal atypia seen in some cases of Grover disease and epithelial neoplasms. MATERIAL AND METHODS: We analyzed 33 cases of Grover disease histologically diagnosed at Wake Forest Baptist Medical Center, NC, between 2011 and 2017. Atypical changes in keratinocytes were defined as epithelial buds, nuclear pleomorphism, and dyskeratosis in all layers of epidermis or altered granular layer. RESULTS: Twenty cases (64%) showed foci with alteration of the normal keratinocytic maturation, whereas 18 cases demonstrated nuclear pleomorphism. Buds of epithelial cells emanating from the basal layer of the epidermis and granular cell alteration was present in 19 cases. CONCLUSIONS: The findings especially the presence of an altered granular layer may represent a diagnostic clue in cases of Grover disease with atypical changes.

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