Subcutaneous Plaques Research Articles

Subcutaneous Panniculitis-like T-cell Lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a primary, rare cutaneous T-cell lymphoma that resembles panniculitis and accounts for <1% of all peripheral T-cell lymphomas. It is characterized by multiple subcutaneous nodules and plaques, which usually involve the extremities. Histopathological findings include the presence of neoplastic T-cell surround and disrupt individual adipocyte membranes. Immunohistochemistry will confirm diagnosis by CD8+ and CD56− cells. Here, we report a case of a 29-year-old female who presented with cutaneous panniculitis-like lesions diagnosed with cutaneous T-cell lymphoma with the help of histopathology and immunohistochemistry.

OA14 Subcutaneous panniculitis-like T-cell lymphoma: a mimic of lupus erythematous panniculitis

Abstract Background/Aims Lupus erythematosus panniculitis (LEP) is a rare variant of cutaneous lupus erythematosus characterised by tender, erythematous subcutaneous nodules or plaques on the face, trunk and proximal extremities. Without treatment, profound lipoatrophy frequently occurs, which often has a devastating psychosocial impact. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cutaneous T-cell lymphoma, which may appear in a similar manner. SPTCL initially preferentially infiltrates the subcutaneous adipose tissue and symptoms vary widely depending on the stage of the disease. Early-stage disease presents as painless self-resolving subcutaneous nodules on limbs and trunk. Methods We present the case of a young woman who presented with progressively developing, widespread painful cutaneous lesions, pyrexia of unknown origin and general malaise for one month. She had a history of hurthle cell neoplasm and a strong family history of malignancy. Initial examination revealed 1-2 cm firm, skin-coloured subcutaneous nodules over her lower back, chest and thigh, with groin and axillary lymphadenopathy. Further lesions progressively developed over a three-month period, with lesions turning increasingly erythematous and with associated ulceration. Of note, a significant periorbital lesion appeared, associated with an ipsilateral facial palsy. Results Initial bloods revealed an elevated LDH with cholestatic liver function tests. PET-CT imaging showed an enlarged axillary node with extensive pathology throughout the subcutaneous fat of unknown aetiology. Due to the family history, underlying haematological malignancy and atypical Sweet’s were considered for this unusual presentation. Seropositivity for anti-Ro and ENA, alongside raised angiotensin converting enzyme, broadened the differentials to include LEP, mixed atypical connective tissue disease and cutaneous sarcoid. Notably, complement, myositis panel and creatine kinase were normal. Lupus vulgaris was considered due to high local prevalence and excluded with T-SPOT testing. Histopathological analysis of lesional skin from the upper arm found predominantly subcutaneous infiltrates of atypical lymphoid cells with irregular hyperchromatic nuclei and rimming of individual fat cells. T-cell clonality analysis detected clonal T-cell receptor beta (V-J and D-J) gene rearrangements consistent with SPTCL. Her orbital and cutaneous lesions responded well to six cycles of CHOEP (cyclophosphamide, hydroxydaunorubicin, oncovin, etoposide, prednisone) and the patient returned to functional baseline. Conclusion This case demonstrates the diagnostic difficulties when managing a patient with relapsing remitting panniculitis. Whilst the initial presentation of SPTCL here was classical, with widespread multifocal subcutaneous nodules, the appearance of facial symptoms in SPTCL is rare. Diagnostic uncertainty is frequent in both LEP and SPTCL due to the initial non-specific cutaneous presentation. This case emphasises the importance of clinicopathological correlation for accurate diagnosis. Interestingly, up to 20% of SPTCL patients have concomitant autoimmune diseases, and there is evidence suggesting a potential association between LEP and subcutaneous SPTCL beyond chance, leading some researchers to propose that these two entities exist along a spectrum. Disclosure D. Li: None. L. Spencer: None.

BI02 Disseminated toxoplasmosis presenting as panniculitis in an immunocompromised patient

Abstract Inflammatory dermatoses and inflammatory reaction patterns to underlying systemic disease are relatively uncommon in immunosuppressed organ transplant recipients (OTRs) and infection should always be suspected as a possible cause. We present an OTR with biopsy-proven panniculitis resulting from disseminated toxoplasmosis, only identified after extensive histological analysis. A 45-year-old Nigerian woman who was profoundly immunocompromised through a combination of a kidney transplant 12 months earlier, hyposplenism due to sickle cell anaemia and a CD4+ count 74 × 106 cells L–1 (> 455 × 106 L–1) presented with a 3-month history of > 20 painful, red–purple and hyperpigmented subcutaneous nodules and plaques up to 2 cm in diameter limited to her legs, associated with fatigue and weight loss. She only had childhood contact with cats. There was no lymphoedema or lymphadenopathy, and she was HIV negative. Urgent skin biopsy excluded Kaposi sarcoma and demonstrated a lobular, necrotizing panniculitis, a mixed inflammatory cell infiltrate and evidence of vasculitis. The cause was unclear; cultures for mycobacterial, fungal and bacterial infections were negative. However, on rereview of histology, within areas of inflammation and necrosis in the subcutaneous fat, there were a few scattered structures (10–30 μm in diameter) consisting of small basophilic periodic acid–Schiff-positive ‘dots’ within bright eosinophilic cytoplasm, raising the possibility of toxoplasmosis. Toxoplasma immuno­staining was positive. Further tertiary review of histology and immunostaining by the Hospital for Tropical Diseases agreed that there was no other causative organism and the panniculitis and vasculitis were consistent with active toxoplasmosis. The diagnosis of toxoplasma reactivation was supported by positive toxoplasma IgG on pretransplant bloods. Magnetic resonance imaging of the head was consistent with cerebral toxoplasmosis. She received treatment with clindamycin, pyrimethamine and folinic acid and, thereafter, long-term cotrimoxazole prophylaxis. Cutaneous toxoplasmosis is rare, with only three reported cases over the last 10 years. Historically, a wide spectrum of clinical morphologies has been described, including maculopapular rashes, nodules, ulcers, dermatomyositis and even mimicry of varicella and graft-versus-host disease. Most cases affect immunocompromised patients. In our patient, this was the result of reactivation of latent infection, which is more common in West Africa, coinciding with profound immunosuppression. In our case, a diagnosis was only made after extensive histological review. Given the varied morphologies of clinical presentations, it is possible that this condition may be easily missed, with potentially serious consequences. In summary, we highlight the importance of maintaining a high index of suspicion for infection and the need for careful clinicopathological correlation in OTRs presenting with apparently atypical inflammatory skin lesions.

Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. Clinically, Merkel cell carcinoma appears as a cutaneous or subcutaneous plaque or nodule, but this tumor diagnosis is rarely made clinically. Therefore, histopathology and immunohistochemistry are usually necessary. Primary tumors without evidence of metastases are treated with complete surgical excision and appropriate surgical margins. The presence of occult metastasis in a lymph node is frequent and a sentinel lymph node biopsy should be performed. Postoperative adjuvant radiotherapy increases local tumor control. Recently, agents that block the PD-1/PD-L1 pathway have shown objective and durable tumor regression in patients with advanced solid malignancies. The first anti-PD-L1 antibody used in patients with Merkel cell carcinoma was avelumab, but pembrolizumab and nivolumab have also shown efficacy. This article describes the current state of knowledge of the epidemiology, diagnosis, and staging of Merkel cell carcinoma, as well as new strategies for its systemic treatment.

Association of knee atypical lesions with foot ulcers and a peak of kappa IgM monoclonal immunoglobulin

Association of knee atypical lesions with foot ulcers and a peak of kappa IgM monoclonal immunoglobulin

Concurrent Occurrence of Nasopharyngeal and Disseminated Cutaneous Rhinosporidiosis: A Rare Presentation.

Concurrent Occurrence of Nasopharyngeal and Disseminated Cutaneous Rhinosporidiosis: A Rare Presentation.

Necrose gordurosa subcutânea do recém-nascido: relato de caso e revisão de literatura

INTRODUCTION: incomplete. RESULTS: Fatty necrosis of the subcutaneous tissue is an uncommon inflammation, with a still unknown pathogenesis, which appears in newborns in the first weeks of life. The lesions are characterized by areas of edema and erythema that progress to hardened and painless subcutaneous plaques or nodules. The risk factors for this panniculitis are hypoxia and perinatal stress, such as traumatic or prolonged delivery and, more recently, therapeutic hypothermia. The diagnosis is clinical, the evolution is self-limited and benign, but the level of serum calcium should be monitored, as hypercalcemia can occur, leading to severe symptoms. In our report, we describe a case of subcutaneous adipose necrosis associated with prolonged expulsive and therapeutic hypothermia. CONCLUSION: Although infrequent, early recognition of this condition, especially in newborns with risk factors, is important, and can guide clinical follow-up including monitoring calcium, pain control and not using antibiotics.

Medical mystery: Deposition of calcium oxalate and phosphate stones in soft tissues

Calcinosis cutis (CC) [1] is an unusual disorder characterized by calcium-phosphate deposition into cutaneous and subcutaneous tissues. There are five subtypes: dystrophic, metastatic, idiopathic, iatrogenic and calciphylaxis. Calciphylaxis or calcifying panniculitis is defined as small vessel calcification mainly affecting blood vessels of the dermis and subcutaneous fat. Despite the predominance of cases in patients with ESRD, calciphylaxis can also be found in patients with normal renal function and normal levels of calcium and phosphate. These cases are often referred to as nonuremic calciphylaxis (NUC), a heterogeneous category with several associations. Literature reveals an association with hyperparathyroidism (28%), malignancy (22%), alcoholic liver disease (17%) and connective tissue diseases (11%) while obesity, liver disease, high-serum calcium (Ca) × phosphorus (P) levels, combined therapies of calcium salts with vitamin D, warfarin and corticosteroids have been observed to increase the likelihood of this disease [2]. The lesions in both nonuremic and uremic calciphylaxis tend to be indistinguishable from each other, initially presenting as tender subcutaneous plaques that progress into nonhealing ulcers with overlying black eschar. Skin changes often begin with a livedo reticularis pattern that can progress to livedo racemes and ultimately retiform purpura. In our clinical case, we describe a patient with multiple risk factors for calciphylaxis, intense widespread calcification (vessels, tendons, joints) and cutaneous calcific stone of calcium and phosphate oxalate not elsewhere described before.

Subcutaneous fat necrosis of the newborn: a pictorial essay of an under-recognized entity.

Subcutaneous fat necrosis of the newborn is a self-limited disorder predominantly affecting full-term and post-term neonates during the first 6weeks after birth. Subcutaneous fat necrosis can be focal or multifocal and affect one or both sides with a predilection for areas of pressure in certain anatomical areas. Subcutaneous fat necrosis of the newborn is associated with perinatal asphyxia and other neonatal and maternal risk factors. Subcutaneous fat necrosis of the newborn presents as a self-limited area of dermal edema followed by indurated subcutaneous plaques, or nontender and mobile nodules, sometimes with skin discoloration [1-3]. The diagnosis is based on the child's history and physical examination, but when in doubt, imaging is helpful. US is the imaging modality of choice to confirm the diagnosis of subcutaneous fat necrosis of the newborn because it provides the best resolution of superficial lesions, requires no sedation and lacks ionizing radiation. US can also help evaluate and characterize other pathologies affecting the superficial subcutaneous soft tissues at this age. Familiarity with subcutaneous fat necrosis of the newborn is important to make a prompt and precise diagnosis and avoid unnecessary imaging tests or invasive procedures.

Subcutaneous Nodules in a 28-week-old Girl.

Subcutaneous Nodules in a 28-week-old Girl.

Calcinosis cutis in the auricle: a case report

Calcinosis cutis is a rare condition defined as the pathological accumulation of amorphous insoluble calcium salts in cutaneous and subcutaneous tissue. There are four subtypes - dystrophic, idiopathic, metastatic, and iatrogenic calcification. The dystrophic form is the most common type of calcinosis cutis, the major agent being trauma. Serum calcium and phosphorus values are within normal limits in these patients. The clinical manifestation generally involves small white papules, and subcutaneous nodules or plaques. The first choice in the treatment of calcinosis cutis is surgery. Surgical procedures have been recommended as the first and most effective option in almost all cases in the literature. We report a case of a patient presenting with growing, painless swelling following trauma to the auricle of the ear identified as dystrophic calcinosis cutis following surgical excision.

Calcinosis cutis in the auricle: a case report

Calcinosis cutis is a rare condition defined as the pathological accumulation of amorphous insoluble calcium salts in cutaneous and subcutaneous tissue. There are four subtypes - dystrophic, idiopathic, metastatic, and iatrogenic calcification. The dystrophic form is the most common type of calcinosis cutis, the major agent being trauma. Serum calcium and phosphorus values are within normal limits in these patients. The clinical manifestation generally involves small white papules, and subcutaneous nodules or plaques. The first choice in the treatment of calcinosis cutis is surgery. Surgical procedures have been recommended as the first and most effective option in almost all cases in the literature. We report a case of a patient presenting with growing, painless swelling following trauma to the auricle of the ear identified as dystrophic calcinosis cutis following surgical excision.

Subcutaneous panniculitis-like T cell lymphoma: a rare entity and its cryptic journey through pregnancy

<p class="abstract">Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of skin lymphoma that primarily is localized to the subcutaneous adipose tissue and accounts for less than 1% of all peripheral T-cell lymphomas. It presents with multiple subcutaneous nodules or plaques on extremities and has poor prognosis if accompanied by hemophagocytic syndrome. Differential diagnosis is panniculitis, lupus panniculitis and leprosy. We report such a rare case of a female with lupus erythematosus (LE) panniculitis like presentation with favourable outcome to oral steroids in pregnancy.</p>

Subcutaneous fat necrosis of newborn - A rare case report of lobular panniculitis in a neonate

Subcutaneous fat necrosis of newborn is a rare cutaneous disorder affecting neonates. It usually presents as subcutaneous nodules or plaques, within the first few weeks of life, following an eventful delivery. It is characterized by hypercalcemia, which may present with lethargy, irritability, hypotonia and dehydration, mimicking sepsis. Histopathology is proven to be the gold standard in diagnosis with characteristic lobular panniculitis, mixed inflammatory cell infiltrate and radially arranged crystals. This needs to be differentiated from other causes of lobular panniculitis, as early diagnosis and treatment to prevent long‑term complications are advocated. Education of parents regarding the disease and danger signs of hypercalcemia and weekly monitoring of serum calcium is recommended. Treatment based on rehydration, dietary vitamin D and calcium restriction, Furosemide and prednisolone are considered. We have discussed a case of subcutaneous fat necrosis, in an 8-week-old male baby.Key Messages: Subcutaneous fat necrosis is an important differential in neonates presenting with palpable subcutaneous nodules, along with sclerema neonatorum. Severe complications like hypercalcemia should be detected early and managed aggressively to prevent morbidities and mortalities associated with it. Symptomatic management, use of calcium lowering drugs and regular monitoring of calcium levels are recommended.

Recurrent Fevers and Painful Subcutaneous Nodules: A Case of Subcutaneous Panniculits-like T-Cell Lymphoma

Background: Subcutaneous Panniculitis-Like T-Cell Lymphoma is a cytotoxic T-cell lymphoma that infiltrates subcutaneous adipose tissue and rarely involves the lymph nodes. SPTCL accounts for less than 1% of non-Hodgkin’s lymphomas. Lesions are commonly localized to the trunk and lower extremities and may be relapsing and remitting in nature. Systemic symptoms such as fever and night sweats are common. SPTCL may closely mimic cellulitis, and other causes of skin and soft tissue infections. Definitive diagnosis is made with tissue biopsy showing atypical lymphocytes rimming adipocytes. There is no current standard of treatment. Prognosis is favorable with a 5-year survival rate as high as 91%. Case Report: A 40-year-old Chinese female presented with a two-week history of recurrent fevers and painful subcutaneous nodules and plaques, initially thought to be infectious in nature. Tissue sampling demonstrated CD4+ and CD8+ T-cells rimming adipocytes with a high Ki-67 proliferation index and an alpha beta T-cell receptor subtype. A diagnosis of SPTCL was established and the patient was treated with multiagent chemotherapy regimen consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP therapy), which resulted in resolution of her constitutional symptoms and cutaneous. Conclusion: SPTCL is a challenging diagnosis due to the wide array of etiologies of panniculitides and its close clinical resemblance to soft tissue infection. Early tissue biopsy should be employed to expedite management.

Traumatic Panniculitis-the Spectrum

Panniculitis, when induced by physical trauma or by chemical agents at injection sites, presents as indurated subcutaneous nodules or plaques. The clinical picture may vary, but the context makes usually the diagnosis easy. Three case histories from our department illustrate the spectrum of traumatic panniculitis: subcutaneous nodules at injection sites, a large subcutaneous mass disproportionate to mild trauma, and, at the other end of the spectrum, severe adipose tissue necrosis with liquefied fat discharging through surface wounds. Traumatic panniculitis is self-limiting and only requires symptomatic treatment.

Neonatale subcutane vetnecrose met opvolging van de hypercalciëmie

Subcutaneous fat necrosis of the newborn with follow-up of the hypercalcemia A thirteen-day-old boy is diagnosed with subcutaneous fat necrosis of the newborn after the appearance of erythematous, subcutaneous plaques on the right shoulder. This rare, usually self-limiting cutaneous disease is mainly diagnosed in term and postterm infants. The diagnosis is confirmed by a skin biopsy. Various etiological hypotheses are suggested, although the exact pathogenesis needs to be elucidated. The disease might be associated with several maternal and foetal predisposing factors, including therapeutic hypothermia at birth as part of hypoxic ischemic encephalopathy. It is a benign inflammation of the subcutaneous adipocytes that can be accompanied by life-threatening complications, such as hypercalcemia and thrombocytopenia. The latter requires rigorous follow-up and, in some cases, also treatment. The skin damage should only be treated symptomatically with analgesia.

Research Progression of Subcutaneous Panniculitis-like T-Cell Lymphoma--Review

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare cutaneous malignant lymphoma derived from cytotoxic T cells that mainly involves subcutaneous adipose tissue rather than epidermis and dermis. It usually occurs in young and middle-aged population, and the etiology is currently unclear. Clinically, SPTCL is characterized by subcutaneous plaques, nodules, and skin ulcers with swell and ache, mainly presenting in limbs and trunk. SPTCL has been restricted to cases that express α/β phenotype, whereas cases with γ/δ phenotype are categorized to cutaneous γ/δ+ T cell lymphoma by WHO-EORTC classification. The standard treatment for SPTCL is not definite with a relatively good prognosis. The recent research progression of SPTCL is summarized in this review.

A Large Subcutaneous Plaque on the Left Flank: Challenge.

A Large Subcutaneous Plaque on the Left Flank: Challenge.

Severe Thrombocytosis in a Newborn with Subcutaneous Fat Necrosis and Maternal Chorioamnionitis

Background Subcutaneous fat necrosis (SFN) is a form of transient panniculitis that presents commonly in infants with a history of perinatal insult, particularly hypothermia. It is characterized by subcutaneous nodules and plaques that appear over bony prominences on cheeks, shoulders, buttock, and thighs. SFN is usually associated with various complications including hypercalcemia, thrombocytopenia, hypertriglyceridemia, and hyperglycemia. Case Presentation. We present a unique case of a female infant with a history of maternal chorioamnionitis who presented with SFN at 11 days of life with thrombocytosis. The platelet count decreased during the hospital stay, and thrombocytosis resolved over the course of the next two weeks. She did not have any other hematological or metabolic abnormalities associated with SFN. Conclusion Infants with perinatal stress are at increased risk of developing SFN during the first month of life. Infants with a diagnosis of SFN should be monitored closely for various hematological and metabolic abnormalities that can have serious consequences.