Pacinian corpuscle hyperplasia in a 6-year-old girl.

We present a rare case of Pacinian corpuscle hyperplasia (PCH) presenting with typical finger pain in a 6-year-old girl. As appendages in children are smaller than those in adults, diagnostic criteria are needed for pathological confirmation of PCH in pediatric patients.

Sensory innervation of the human palmar aponeurosis in healthy individuals and patients with palmar fibromatosis.

The human palmar aponeurosis is involved in hand proprioception, and it contains different sensory corpuscle morphotypes that serve this role. In palmar fibromatosis (classically referred to as Dupuytren's disease), the palmar aponeurosis undergoes fibrous structural changes that, presumably, also affect the nervous system, causing altered perception. We analysed the various sensory nerve formation morphotypes in the palmar aponeuroses of healthy subjects and patients with palmar fibromatosis. To do this, we used immunohistochemistry for corpuscular constituents and the putative mechanoproteins PIEZO2 and acid-sensing ion channel 2. Free nerve endings and Golgi-Mazzoni, Ruffini, paciniform and Pacinian corpuscles were identified in both the healthy and the pathological conditions. The densities of the free nerve endings and Golgi-Mazzoni corpuscles were slightly increased in the pathological tissues. Furthermore, the Pacinian corpuscles were enlarged and displayed an altered shape. Finally, there was also morphological and immunohistochemical evidence of occasional denervation of the Pacinian corpuscles, although no increase in their number was observed. Both PIEZO2 and acid-sensing ion channel 2 were absent from the altered corpuscles. These results indicate that the human palmar aponeurosis is richly innervated, and the free nerve endings and sensory corpuscles within the palmar aponeurosis undergo quantitative and qualitative changes in patients with palmar fibromatosis, which may explain the sensory alterations occasionally reported for this pathology.

Ageing of the somatosensory system at the periphery: age-related changes in cutaneous mechanoreceptors.

Decline of tactile sensation associated with ageing depends on modifications in skin and both central and peripheral nervous systems. At present, age-related changes in the periphery of the somatosensory system, particularly concerning the effects on mechanoreceptors, remain unknown. Here we used immunohistochemistry to analyse the age-dependent changes in Meissner's and Pacinian corpuscles as well as in Merkel cell-neurite complexes. Moreover, variations in the neurotrophic TrkB-BDNF system and the mechanoprotein Piezo2 (involved in maintenance of cutaneous mechanoreceptors and light touch, respectively) were evaluated. The number of Meissner's corpuscles and Merkel cells decreased progressively with ageing. Meissner's corpuscles were smaller, rounded in morphology and located deeper in the dermis, and signs of corpuscular denervation were found in the oldest subjects. Pacinian corpuscles generally showed no relevant age-related alterations. Reduced expression of Piezo2 in the axon of Meissner's corpuscles and in Merkel cells was observed in old subjects, as well was a decline in the BDNF-TrkB neurotrophic system. This study demonstrates that cutaneous Meissner's corpuscles and Merkel cell-neurite complexes (and less evidently Pacinian corpuscles) undergo morphological and size changes during the ageing process, as well as a reduction in terms of density. Furthermore, the mechanoprotein Piezo2 and the neurotrophic TrkB-BDNF system are reduced in aged corpuscles. Taken together, these alterations might explain part of the impairment of the somatosensory system associated with ageing.

Normal Pacinian corpuscles in the hand: radiology-pathology correlation in a cadaver study.

OBJECTIVE: To provide microdissection and histological confirmation of normal Pacinian corpuscles prospectively identified using MRI in a cadaver model. METHODS: 3-T MRI of a cadaveric hand specimen was performed with fiduciary markers on the skin. Based on previous descriptions, subcutaneous nodules representing presumed Pacinian corpuscles were localized with respect to the skin markers, and their sizes and depths were recorded. Focused ultrasound was performed to attempt to visualize the corpuscles. Subsequent microdissection was then performed and the presence and location of Pacinian corpuscles were recorded and compared with the findings on MRI. Histological evaluation for each identified corpuscle was performed. RESULTS: The MRI demonstrated 11 T2-hyperintense palmar subcutaneous nodules around the second through fifth metacarpophalangeal joints. None was visible sonographically. The first eight were dissected and proved to be normal Pacinian corpuscles histologically. In sites devoid of subcutaneous nodules on MRI, subsequent dissection failed to reveal any corpuscles. CONCLUSION: On MRI, normal Pacinian corpuscles appear as round or oval, T2-hyperintense subcutaneous nodules in the palms, clustered around the metacarpophalangeal joints, and should not be mistaken for pathological conditions.

Hyperplasia and Hypertrophy of Pacinian Corpuscles: A Case Report.

The proliferation of Pacinian corpuscles is a rare benign lesion that occurs frequently in the fingers of hands, with the only clinical sign of pain when a drastic temperature change happens. A case of an extremely rare lesion in which we observed hypertrophy and hyperplasia of Pacinian corpuscles has been reported.

An Extraordinary Association of Glomus Tumor and Pacinian Hyperplasia in the Hand of a Female Patient.

BACKGROUND: Glomus tumor is a benign neoplasm of the glomus body, a neuromyoarterial structure that regulates temperature and pressure in the cutaneous vasculature. Approximately 1%-4.5% of glomus tumors present in the hands of females; of these, 65% are seen in the subungual region of the index and long fingers. Pacinian hyperplasia is a benign lesion of the Pacinian corpuscle, a mechanoreceptor located in the subcutis of the hands and feet. METHODS: A 65-year-old woman with a history of hand trauma and a 1-year chief complaint of tingling, pain, and burning sensations in her proximal thumb underwent exploration of the digital nerve after an x-ray and 2 magnetic resonance imaging examinations failed to detect a mass. Two lesions immediately adjacent to each other were excised. RESULTS: Microscopic examination showed Pacinian hyperplasia, and a second proliferation of solid epithelioid cells related to benign blood vessels. Immunohistochemistry confirmed the epithelioid cells to be strongly positive for smooth muscle actin, CD34, and type 4 collagen, which is consistent with the phenotype of a glomus tumor. The cells were negative for S100 protein. CONCLUSIONS: The association of glomus tumor with Pacinian hyperplasia has rarely been reported in the literature. We present another rare case to bring awareness to this differential diagnostic consideration.

Digital Neuropathy by Hypertrophy and Hyperplasia of Pacinian Corpuscle - Usefulness of Microscopic Resection.

We present a patient with Pacinian corpuscle hypertrophy and hyperplasia in the hand and discuss the diagnosis and treatment of this rare condition. A 46-year-old woman presented with radiating pain of the left middle finger. A strong Tinel-like sign was elicited between the index and middle fingers. The patient frequently used mobile phone, with the corner of the phone consistently applying pressure on the palm. The surgery was carried out under the microscope and two enlarged cystic lesions under the epineurium were found in the proper digital nerve. Histologic examination revealed hypertrophied Pacinian corpuscle with normal structure. Postoperatively, her symptoms gradually improved. Preoperative diagnosis of this disease is very difficult. Hand surgeons should keep this disease in mind preoperatively. In our case, we would not have been able to identify multiple hypertrophic Pacinian corpuscles without the microscope. An operating microscope is recommended in a surgery of this nature. Level of Evidence: Level V (Therapeutic).

Tender papule rising on the digit: Pacinian neuroma should be considered in differential diagnosis.

Pacinian corpuscles are sensory nerve-end organs located in the deep dermis and subcutaneous tissue of the palms or soles. Pacinian neuroma is an extremely rare feature, defined as hyperplasia or hypertrophy of Pacinian corpuscles. About half of Pacinian neuromas present with point tenderness. There have been a limited number of cases reported around the world. We observed a 45-year-old woman with an 8-month history of a tender whitish papule on her left thumb tip. Histopathologically, an enlarged hypertrophic Pacinian corpuscle in subcutaneous tissue, surrounded by numerous nerve fibers, was found. Herein, we report a case of Pacinian neuroma presenting as a tender papule on a fingertip that was clearly related to repetitive trauma at that site. This case shows that a meticulous history and histological examination can lead to an exact diagnosis and proper treatment.

Cutaneous emboli of atrial myxoma: unusual case report and review of the literature.

Cardiac myxomas are difficult to diagnose, not only because of a lack of specific systemic symptoms but also because, even in those cases presenting with embolic disease, emboli are exceptional. The skin is one of the organs most frequently involved by myxomatous emboli. We report an extraordinary case in which emboli of a cardiac myxoma was present in the skin biopsy, but "camouflaged" among the normal Vater-Pacini corpuscles of the palm of the hand, We also review the existing literature on myxomatous emboli identified in skin biopsies.

First case report of Pacinian corpuscle hyperplasia following complex regional pain syndrome.

Pacinian disorders are exceedingly rare, and the exact pathogenesis is still unknown. The most common symptoms are pain, sensory changes, and a visible or palpable mass, and diagnosis is usually made by pathological examination after the excision of the painful nodule. In this case report, we present the case of a 49-year-old male with Pacinian corpuscle hyperplasia located on the metacarpophalangeal joint, emerging at the same hand of the patient two years after the treatment due to complex regional pain syndrome (CRPS). To the best of our knowledge, this is the first case report revealing the association of CRPS with hyperplasia of Pacinian corpuscles.

Neural system antigens are recognized by autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in Colombia.

BACKGROUND: Endemic pemphigus foliaceus (EPF), is also known as "fogo selvagem" or "wild fire," reflecting the intense burning sensation of the skin reported by patients with this disease. Based on this finding, we tested for neural autoreactivity in patients affected by a new variant of EPF (El Bagre-EPF). METHODS: We tested 20 El Bagre-EPF patients, 20 normal controls from the endemic area, and 20 age- and sex-matched normal controls from outside the endemic area. We tested for autoreactivity to several immunoglobulins and complement. Both human skin and bovine tail were used as antigens. RESULTS: We detected autoreactivity to neural structures, mechanoreceptors, nerves, perineural cell layers of the arachnoid envelope around the optic nerve, brain structures, and to neuromuscular spindles; these structures colocalized with several neural markers. The patient antibodies also colocalized with desmoplakins 1 and 2, with the armadillo repeat protein deleted in velo-cardio-facial syndrome and with p0071 antibodies. Autoreactivity was also found associated with neurovascular bundles innervating the skin, and immunoelectron microscopy using protein A gold against patient antibodies was positive against the nerve axons. Paucicellularity of the intraepidermal nerve endings and defragmentation of the neural plexus were seen in 70% of the cases and not in the controls from the endemic area (p<0.005). Neuropsychological and/or behavioral symptoms were detected in individuals from the endemic area, including sensorimotor axonal neuropathy. CONCLUSIONS: Our findings may explain for the first time the "pose of pemphigus," representing a dorsiflexural posture seen in EPF patients vis-a-vis the weakness of the extensor nerves, and furthermore, the autoreactivity to nerves in EPF could explain the "burning sensation" encountered in EPF disease.

Bilateral symptomatic Pacinian corpuscle hyperplasia of the adult foot.

Pacinian hyperplasia in the human body has been rarely described in the literature with most cases reported involving the hand (Fassola et al., 2019). This hyperplasia is considered a reactive lesion as opposed to a neoplasm (Satge et al., 2001), with the most common presenting complaint being pain and swelling (Fassola et al., 2019). In this study, a case of bilateral Pacinian corpuscle hyperplasia in the adult foot not previously described in the literature is presented.

Ectopic lamellar Pacinian corpuscle within the thymus. Atypical or abnormal location?

Lamellar (Pacinian) corpuscle is a cutaneous encapsulated sensory corpuscle, mainly functioning as a rapidly adapting low-threshold mechanoreceptor with characteristic "onion skin"-like appearance. Even though the Pacinian corpuscle is typically located in the skin, histomorphologically and confirmed by using immunohistochemical methods, we have identified it within the interstitium of the thymus of a newborn with congenital heart defect. To the best of our knowledge, this is the first such account ever to be published. The structure of the thymic Pacinian corpuscle was the same as the Pacinian corpuscle in the skin. The ectopic thymic Pacinian corpuscle can be hypothetically explained as the consequence of impaired migration of neural crest cells, since neural crest-derived cells play an important role in the development of the Pacinian corpuscle, as well as the thymus and heart. In general, the occurrence of ectopic Pacinian corpuscle in different organs is rare. In the scientific literature, there are reports of its sporadic presence in the pancreas, in the lymph nodes, inside the prostate and the urinary bladder wall. Our report presents the first described case of the Pacinian corpuscle in a heterotopic location in the thymus. Similar to other incidental findings of this anatomic structure, explanation of its ectopic development, as well as its local function remains only speculative.

Penile pacinian neurofibroma.

Pacinian neurofibroma (PNF) is a lobulated benign neural tumor with prominent structures resembling pacinian bodies. These tumors most commonly occur in areas where normal pacinian bodies are found, such as the hands and feet. Although pacinian bodies are common in the penis, no cases of penile PNF have been reported to date. We present a case of PNF on the dorsal glans penis of a 47-year-old man. The lesion presented as a single flesh-colored papule and the biopsy showed a dermal neurofibroma consisting of bland spindle cells with wavy nuclei, without mitoses or atypia, and some nodular structures with a concentric arrangement and a pacinian appearance. Immunohistochemistry demonstrated positivity for CD34 and Vimetin and negativity for Epithelial Membrane Antigen (EMA). S100 was highly positive in the most central areas of the pacinian-like nodules, while the periphery and non-nodular parts of the neurofibroma were less intensively expressed.

Pacinian neuromas and neurofibromas of the hands and fingers: a systematic review.

Tumours involving Pacinian corpuscles are rare. The literature identifies two main pathological disorders: the Pacinian corpuscle neuroma or hyperplasia and the Pacinian corpuscle neurofibroma. Published data are confusing and at times conflicting. This systematic review summarizes the available data in order to support clinicians in the differential diagnosis with other tumours responsible for unclear symptoms in the hands and fingers. We identified 67 pertinent articles. Although some similarities have been described, the two tumours have relevant differences, specifically when comparing age of the patient, location, symptoms, characteristic of a mass, and aetiology. All these factors should be taken into account in order to improve diagnostic accuracy. Despite the low incidence of unsuccessful surgeries, extraordinary measures are occasionally necessary to achieve complete resolution of symptoms.

Differential hypertrophy and atrophy among all types of cutaneous innervation in the glabrous skin of the monkey hand during aging and naturally occurring type 2 diabetes.

Diabetic neuropathy (DN) is a common severe complication of type 2 diabetes. The symptoms of chronic pain, tingling, and numbness are generally attributed to small fiber dysfunction. However, little is known about the pathology among innervation to distal extremities, where symptoms start earliest and are most severe, and where the innervation density is the highest and includes a wide variety of large fiber sensory endings. Our study assessed the immunochemistry, morphology, and density of the nonvascular innervation in glabrous skin from the hands of aged nondiabetic rhesus monkeys and from age-matched monkeys that had different durations of spontaneously occurring type 2 diabetes. Age-related reductions occurred among all types of innervation, with epidermal C-fiber endings preferentially diminishing earlier than presumptive Adelta-fiber endings. In diabetic monkeys epidermal innervation density diminished faster, became more unevenly distributed, and lost immunodetectable expression of calcitonin gene-related peptide and capsaicin receptors, TrpV1. Pacinian corpuscles also deteriorated. However, during the first few years of hyperglycemia, a surprising hypertrophy occurred among terminal arbors of remaining epidermal endings. Hypertrophy also occurred among Meissner corpuscles and Merkel endings supplied by Abeta fibers. After longer-term hyperglycemia, Meissner corpuscle hypertrophy declined but the number of corpuscles remained higher than in age-matched nondiabetics. However, the diabetic Meissner corpuscles had an abnormal structure and immunochemistry. In contrast, the expanded Merkel innervation was reduced to age-matched nondiabetic levels. These results indicate that transient phases of substantial innervation remodeling occur during the progression of diabetes, with differential increases and decreases occurring among the varieties of innervation.

Pacinian Corpuscles in Human Lymph Nodes.

The occurrence of Pacinian corpuscles associated to lymph nodes is an anatomical rarity and very scarce information exists in this regard. Here we examined immunohistochemically four Pacinian corpuscles found in the close vicinity of the hiliar blood vessels of lymph nodes (2 cervical, 1 axillary, and 1 inguinal) during routine surgical pathology. Pacinian corpuscles were normally arranged and displayed a pattern of protein distribution as follows: the axon was positive for neurofilament proteins and neuron specific enolase, the inner core cells showed intense S100 protein and vimentin immunostaining while they were negative for glial fibrillary acidic protein, type IV collagen and glucose transporter 1; vimentin, type IV collagen, and glucose transporter 1 were also observed also in the outer-core and the capsule. These results are in agreement with those reported for cutaneous Pacinian corpuscles, demonstrating that the immunohistochemical profile of these corpuscles is independent of its anatomical localization. The possible functional significance of Pacinian corpuscles in lymph nodes is discussed. Anat Rec, 300:2233-2238, 2017. © 2017 Wiley Periodicals, Inc.

Pacinian neuroma in adipose herniation of the buccal mucosa.

An interesting case of a trauma-induced tender mass of the buccal mucosa in a 45-year-old man was presented. Following surgery, the patient was relieved from pain. Microscopically, the mature adipose tissue is unique in that it contained a single enlarged Pacinian corpuscle near the deep margin. This is the hitherto undescribed intraoral lesion of Pacinian neuroma in the herniated buccal fat pad.