Lipomas are common benign tumors of mature fat cells. Although most lipomas are asymptomatic, patients often seek their removal for cosmetic reasons. The current treatment modalities, including surgical excision and liposuction, are highly effective but have a high risk of scarring. When occurring in cosmetically sensitive areas such as the face, noninvasive treatment modalities should be considered to minimize the risk of scarring associated with traditional treatment options. Intralesional injections of deoxycholic acid are currently approved by the US Food and Drug Administration for the removal of excessive submental fat, and the use of this compound for the treatment of lipomas is mainly off-label, with only a handful of cases documenting such use having been reported in the literature.
In this study, we assessed the Kidney Donor Risk Index (KDRI) in Puerto Rican deceased kidney donors whose donations took place from 2009 to 2011 and evaluated short-term graft survival in the recipients of those kidneys. The results highlight differences between the distributions of KDRI values in the populations of the 48 contiguous states of the United States, Alaska, and Hawaii and that of Puerto Rico. Additionally, we evaluated the impacts of polyomavirus (BKV) infection and anti-donor HLA antibodies on the recipients.Of the 377 kidneys obtained from deceased donors by LifeLink of Puerto Rico from 2009 to 2011, 187 were transplanted in Puerto Rico. Data was collected from the deceased donors of these 187 kidneys for calculating KDRI, as well as from the transplant recipients. KDRI values of the donors were calculated using the same formula as previously reported for the United States; death-censored graft survival, incidence of antibody-mediated rejection, and prevalence of polyoma virus infection (BKV) were examined in the recipients.The mean KDRI value was 1.19. However, the distribution of KDRI values in the Puerto Rican population deviates substantially from that of the United States (not including Puerto Rico). A 1-peak distribution pattern describes Puerto Rican KDRI values. Graft survival for the study period was 89.6%. The prevalence of BKV was 16.9%. Of the patients studied, 6.25% developed overt nephropathy, 46.2% developed de novo post-transplant donor-specific alloantibodies, and 19.5% had pre-existing alloantibodies.Our study evidences the role of various characteristics in the distribution of KDRI values in the Puerto Rican population, suggesting that the identification of variables specific to a geographically distinct group may result in better donor categorization for predicting transplant outcomes. In addition, our graft-survival results, despite the elevated rates of BKV and anti-donor antibodies, highlight the increasing need to monitor the presence of antibodies in a prospective and an anticipatory manner to identify and manage patients at risk for antibodymediated rejection.
A 4-month-old boy presents to the dermatology clinic with a rash on his neck, axilla, and inguinal folds since 2 months of age (Fig 1). The patient was born at term, and birthweight was appropriate for gestational age. Birth and medical histories were normal, with no prenatal, perinatal, or postnatal complications. His developmental milestones and growth velocity are normal. Because the rash was presumed to be due to infection, he had been treated with mupirocin 2% ointment, clindamycin 1% topical solution, clotrimazole 2% cream, and, finally, a 10-day course of oral cephalexin. He had also been treated with topical corticosteroids of varying potencies for suspected contact dermatitis, but the lesions continued to worsen.His physical examination reveals macerated, erythematous, and scaly patches in the axillae, neck, and inguinal folds. The patient has no other systemic findings. Complete blood cell count and complete metabolic panel are normal. A skeletal survey is also normal. Given multiple treatment failures, a skin biopsy is performed on a yellow-brown papule on the leg, which confirms the diagnosis.The differential diagnosis includes irritant contact dermatitis, seborrheic dermatitis, psoriasis, atopic dermatitis, streptococcal perianal dermatitis, candidiasis, and acrodermatitis enteropathica. The biopsy revealed a dense lichenoid lymphohistiocytic infiltrate positive for CD45, CD68, CD1a, S-100, and langerin, consistent with Langerhans cell histiocytosis (LCH).LCH is an inflammatory neoplasm of myeloid-lineage progenitor cells. This condition was originally classified into 4 distinct entities, including Letterer-Siwe disease, Hashimoto-Pritzker disease, eosinophilic granuloma of bone, and Hand-Schuller-Christian disease. (1) Due to overlapping radiologic and histologic findings, Lichtenstein and Jeffe (1) grouped them under the term histiocytosis X in 1953. With the introduction of electron microscopy, Nezelof et al (2) identified Birbeck granules as specific components of normal epidermal Langerhans cells and of histiocytosis X lesions. Hence, the disease was renamed LCH. (3)LCH is most commonly due to the BRAF V600 mutation, which activates the MAPK-ERK pathway and causes rapid proliferation of cells. It affects 1 in 200,000 children each year, with a median age at diagnosis of 3.5 years, although it is also seen in the elderly. (4)(5)LCH can affect different organs and can be clinically classified as unisystem or multisystem, depending on the number of organs affected by the disease. It is also classified as unifocal or multifocal, depending on the number of lesions seen. Multisystem LCH is associated with the worst prognosis of any of these subtypes. (6)LCH most commonly affects the bones in up to 80% of patients, presenting as lytic lesions in the skull and in the vertebrae. (7) It is frequently associated with localized, tender, soft, and raised spots, although asymptomatic cases are also seen. (7)(8) Skin involvement is seen in approximately 33% of patients and is the second most common organ manifestation. (7) It presents as erythematous, scaly, or crusted papules and patches, mimicking a seborrheic dermatitislike or eczematouslike rash. (9) It may also present with erythematous or flesh-colored, ulcerated or umbilicated papules and plaques, as well as pustules, vesicles, and purpura. (9) Lesions are commonly located on the scalp, trunk, neck, inguinal folds, axillae, and perineum. Other organs less commonly affected include the pituitary gland, liver, spleen, lungs, lymph nodes, and central nervous system. (7) Therefore, evaluation should include a complete blood cell count, a complete metabolic panel, an erythrocyte sedimentation rate, coagulation studies, abdominal ultrasonography, chest radiography, and a skeletal radiographic survey to assess for systemic involvement. (7)Our patient developed skin lesions in the axillae, followed by the neck and inguinal folds. Although there are many cases that describe LCH involving the axillae, there is currently limited information regarding the number of cases in which this is the initial area involved. (9)It is important to identify and diagnose early skin manifestations because 87% to 93% of patients with cutaneous LCH also present with systemic involvement, which is associated with a mortality rate of approximately 20%. (6)(10) Rarely, patients with cutaneous LCH lack systemic involvement, as in our patient. This uncommon subtype occurs in approximately 2% of patients and is called isolated cutaneous LCH (IC-LCH). It mostly affects infants aged 0 to 2 years and generally has a good prognosis. (11) Evolution of lesions is variable. Lesions may remain stable in size, grow proportionately with the patient, or involute spontaneously, typically healing with scarring or pigmentation changes such as hyperpigmentation or hypopigmentation. (12)(13)(14)(15)Diagnosing this condition may be challenging because cutaneous manifestations can mimic some of the most common dermatoses observed in the pediatric population. The rates of misdiagnosis seen in LCH have been reported to be as high as 16%, resulting in a delay of care and contributing to morbidity and mortality. (9) For instance, scalp lesions may be misdiagnosed as seborrheic dermatitis. (13) Meanwhile, groin lesions may be confused with treatment-resistant diaper dermatitis, psoriasis, atopic dermatitis, intertrigo, streptococcal intertrigo, irritant contact dermatitis, and candidiasis, among others. (13) Therefore, it is not surprising that our patient received treatment for some of these conditions before visiting our clinic, all of which were futile. This highlights the importance of considering LCH in the differential diagnosis for intertriginous rashes seen in children. Prompt and appropriate treatment of their condition may be lifesaving and prevent long-term sequelae. (16)There is no standard treatment protocol for IC-LCH. (17) Thus, treatment varies from patient to patient. Current strategies are supported by case series rather than by prospective controlled trials. (17) For instance, the use of systemic therapy in IC-LCH is limited to patients with progressive disease. (18)(19) For unifocal IC-LCH, surgical resection of the lesion is both diagnostic and curative. For low-quantity multifocal IC-LCH, as seen in our patient, first-line therapy is medium- to high-potency topical corticosteroids. (17) However, lesions may not respond to topical corticosteroids and tend to recur on discontinuation. (20)(21) Other agents used as second-line therapy in children include topical calcineurin inhibitors, nitrogen mustard, phototherapy, and imiquimod. (17)Recent studies have shown that topical imiquimod therapy can be effective for skin lesions seen in LCH in both children and adults. (21)(22) This immunomodulator acts by augmenting the type 1 T helper cell–mediated immune response and inducing the release of interferon-α, tumor necrosis factor α, and interleukin-1, among others. (22) It also enhances antigen presentation by Langerhans cells. Topical imiquimod has proved to be effective in lesions unresponsive to topical corticosteroids, all the while sparing patients from corticosteroid-induced adverse effects seen with long-term treatment, especially in flexural areas. It has been mainly used in adult patients with LCH for up to 6 months. (21) A single case report describes the use of topical imiquimod in the pediatric population, with resolution of lesions 5 months after initiating therapy and no recurrence 2 years after discontinuing imiquimod. (22) Minimal adverse effects were noted in both groups in the form of local skin irritation. (21)(22)Once the diagnosis was confirmed, the potency of topical corticosteroids was increased. However, triamcinolone acetonide 0.1% ointment was not effective. Tacrolimus 0.03% ointment was begun but was discontinued due to skin irritation. The patient was then started on imiquimod 5% cream daily, with resolution of lesions in 2 months (Fig 2). This therapy was tolerated well, with only mild skin irritation. She was referred to oncology for further management. Chemotherapy was not recommended because the skin lesions improved with topical therapy and the patient had no systemic findings. The patient was treated with imiquimod 5% cream 3 times daily once lesions resolved for 6 months, and treatment was then discontinued, with no evidence of recurrence of lesions after 9 months. Results of routine laboratory tests and imaging have been negative for systemic disease for the past year, including complete blood cell count, complete metabolic panel, abdominal ultrasonography, and skeletal radiographic survey. Patients with LCH should be followed for at least 5 years after the end of therapy; 5 years after the last disease reactivation in those who did not receive systemic therapy, as in our patient; and through the end of puberty. (7)
Psoriasis is a chronic, multisystemic, inflammatory disease that exhibits a wide array of clinical presentations and is associated with high disease burden. Advancements in our understanding of the pathophysiology of psoriasis have led to the development of novel therapeutic modalities known as biologics that inhibit the action of pro-inflammatory cytokines, offering a more targeted therapeutic approach and improved safety profile compared to traditional systemic agents. Psoriasis disease severity is usually the main driver when it comes to selecting the appropriate biologic for any given patient; however, with an increasing number of biologics from which to choose, one pressing question that is increasingly more common in clinical practice is, “How do we select the ‘right’ treatment for each patient?” We performed a review of the literature on biologic use in patients with psoriasis with a focus on specific systemic comorbidities. Here, we summarize currently available recommendations based on available studies.
Juvenile xanthogranuloma (JXG) is the most common histiocytic disorder of childhood, classically presenting as an asymptomatic, solitary, and yellowish papulonodule on the head or neck that spontaneously regresses after 3 to 6 years.1 The literature shows that some JXG patients develop multiple lesions; in addition, several clinical variants have been described, including micronodular, macronodular, and oral JXG.1 The most common extracutaneous JXG is the ocular form, which may be complicated by hyphema, glaucoma, and/or blindness.
Amivantamab is a monoclonal antibody targeted against the epidermal growth factor receptor (EGFR) and mesenchymal–epithelial transition factor (MET) receptor.1 Approved recently, on May 21, 2021, for non–small cell lung cancer.2 Cutaneous side effects are similar to those commonly observed with EGFR inhibitors, including papulopustular lesions, paronychia, and pruritus.3 The EGFR protein is essential for skin growth and skin barrier integrity, which reflects the cutaneous adverse effects observed with EGFR inhibitors.