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Pilomatricoma is a benign proliferative lesion of skin appendages that often affects the head, upper limbs, and lower limbs. The clinical appearance of the lesions is that of asymptomatic nodules measuring less than 3 cm. pathologically, these skin lesions show the presence of basaloid cell islands, eosinophilic cytoplasmic cells without nuclei, as well as hemorrhage and calcification. In this study, we present the case of an 8-year-old girl with a 5 × 5 cm skin lesion on the forearm, which lacked the typical firmness associated with pilomatricoma lesions during examination. After biopsy, the lesion was confirmed to be pilomatricoma. Furthermore, we have reviewed studies documenting pilomatricoma lesions with atypical clinical features. Based on reports of different clinical manifestations of pilomatricoma in these studies, we suggest that the clinical diagnosis of pilomatricoma should not be limited to the typical presentation of these lesions. In cases where the lesions exceed 3 cm in size, display cystic characteristics, are painful, or resemble keloids, consideration should also be given to the possibility of pilomatricoma.

Pilomatrixoma (PMX), also known as calcifying epithelioma of Malherbe, is a rare benign neoplasm that arises from the hair matrix cells, commonly in the head, neck, and upper trunk regions, infrequently affecting upper and lower extremities. It has to two peaks of presentation: under 20 years of age or between 50 and 65 years of age, slightly more common in females. The neoplasm exhibits diverse clinical manifestations and is frequently subject to misdiagnosis with alternative dermatological diseases. We present an atypical case of PMX affecting the upper extremity of a 62-year-old female patient. Surgical removal of the affected tissue under local anesthesia was performed, and subsequent histopathological analysis confirmed the presence of PMX. Based on the literature search we performed, we found out that this pathology is underreported in Jordan, with only one study published describing this tumor in the maxillofacial region. Physicians should be aware of this condition and its different presentations to include it in the differential diagnosis of suspected cases to provide the appropriate management and follow-up.

Pilomatrix carcinoma is a rare tumor arising from the hair follicle matrix cells most commonly seen in the head and neck region. Also known as \"calcified epithelial carcinoma of Melherbe,\" it was first reported in 1980 by Lopansri and Mihm. Since then till date to the best of our knowledge only around 125 cases were reported in literature, of which only 11 cases were reported to arise from histologically proven areas of previous pilomatrixoma which is the benign variant. One such case is being reported here along with the review of literature. A 50-year-old man presented with a swelling in the nape of his neck since 6 months, which was gradually increasing in size. He had a history of similar swelling at the same site 18 months back for which he underwent a surgery at a center outside. Final histopathology report was suggestive of pilomatrixoma with negative margin. Wide local excision of the tumor with 3 cm margin, placement of surgical clips followed by a primary closure was done. The final histopathology report is suggestive of pilomatrix carcinoma. The patient has no recurrence in 6 months follow-up. The differential diagnosis of pilomatrix carcinoma should be considered in cases of recurrent skin tumors. Wide local excision is the preferred treatment. Re-excision should be done in margin positive cases and cases where simple excision was done due to improper preoperative diagnosis. Due to the rarity of the disease, adjuvant treatment is not properly defined.

Pilomatrixoma is a rare benign skin adnexal tumor arising from the hair follicles. Its occurrence in the male breast is extremely uncommon with very few cases reported worldwide. Pilomatrixoma may masquerade as a malignant tumor due to the presence of foci of calcification. We present a case of a 51-year-old man with a six-month history of a firm-to-hard mass in his right breast. The mammographic and sonographic features were suggestive of skin-related lesions. The lesion was excised. Histopathology confirmed the diagnosis of pilomatrixoma.

Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed. We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD.

A conventional pilomatricoma presents as an asymptomatic, firm, subcutaneous nodule, commonly in the head, neck, and extremities of the paediatric population. However, its variants show diverse clinical features, which often pose a diagnostic challenge to clinicians. We recently treated an unusual case of pilomatricoma with cystic features in the upper chest of a 9-year-old girl, which manifested as a single solid tumour floating in a serous-filled sac formed by thick fibrous tissue. Herein, we present novel clinicopathological features of an early-stage pilomatricoma that should be clinically differentiated from a cutaneous abscess.

UNASSIGNED: Melanocytic matricoma with atypical features is a rare, biphasic adnexal neoplasm displaying hair matrix differentiation, with only 3 reported cases worldwide. Generally, the lesion comprised a solid matrical and supramatrical cell proliferation, admixed with intermediate cell aggregates with sparse anucleated \"shadow cells\" and a prominent pigmented melanocytic hyperplasia. We report the case of a 78-year-old man with a slow-growing crusted lesion on the frontal left scalp, which in a matter of 1-2 months became a 0.6 cm well-defined, black purplish exophytic nodule. Histopathologically, the lesion presented a well-circumscribed border with a nodular dermal growth pattern, presenting different architectural features varying from benign pilomatricoma-like changes to atypical features such as moderate-to-high nuclear pleomorphism in both basaloid (matrical/supramatrical) and epidermal (keratinous) components. Strong nuclear and cytoplasmic positivity for β-catenin was observed in matrical cells, whereas prominent cytoplasmic membrane positivity for Melan-A in dendritic melanocytes. Owing to the evidence of atypical cytological features, we propose the \"atypical/borderline\" category of melanocytic matricoma as part of a possible spectrum among matrical neoplasms. Pathologists should be aware of any atypical histopathological features while reporting cases due to their potential malignant transformation.

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Pilomatricoma is a rare benign skin adnexal tumor arising from hair matrix cells. It usually manifests as a firm-to-hard, well-circumscribed, nontender nodule often mistaken as a dermoid cyst. However, its clinical presentation has been reported to mimic keloid. In addition, although it occurs most frequently in the head and neck area, pilomatricoma is rarely reported on the auricle. Herein, we reported a case of pilomatricoma occurring on the posterior part of a 9-year-old girl\'s auricle. Unlike classic pilomatricoma, the tumor closely resembled a keloid. Nevertheless, histological examination following excision of the tumor revealed a dermal tumor arranged in solid nests consisting of basaloid and ghost cells along with foreign body reaction, calcification, and ossification, confirming the diagnosis of pilomatricoma. The rarity, atypical clinical presentation, and unique location of this tumor contributed to the misdiagnosis of this case. Therefore, we reviewed previous cases of pilomatricoma of the auricle reported in the literature to provide a comprehensive understanding of this rare entity.