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PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome is a rare autoinflammatory disorder often arising in pediatric patients. We present a case of an 18-year-old female with a past medical history of growth failure, immunoglobulin A nephropathy, and inflammatory arthritis who presented to a pediatric dermatology clinic with findings of acne, psoriasiform dermatitis, and hidradenitis suppurativa, whose clinical, genetic, and laboratory findings were most consistent with PAMI syndrome. We conducted a literature review to better characterize this rare condition in the context of dermatologic findings. Recognition of the distinctive skin findings seen in PAMI syndrome can help distinguish it from other inflammatory disorders, enabling expedited diagnosis and treatment.

We report an immunocompromised patient with a complex cutaneous leishmaniasis (CL) who suffered from singular bone involvement of the little left toe due to Leishmania (L.) infantum infection. The diagnosis was confirmed by positive polymerase chain reaction (PCR) testing in skin and bone tissue. The patient was successfully treated with miltefosine. In immunocompromised patients with CL, extracutaneous manifestations should always be ruled out. This is the first case report describing osseous involvement in CL due to Leishmania infantum.
UNASSIGNED: Wir berichten über einen immunsupprimierten Patienten mit einer komplexen kutanen Leishmaniose (Erreger: Leishmania [L.] infantum), der eine ossäre Beteiligung des Digitus V des linken Fußes aufwies und erfolgreich mit Miltefosin behandelt wurde. Die Erregerdiagnostik erfolgte mittels positiver Polymerasekettenreaktion (PCR) in Haut- und Knochengewebe. Bei Immunsupprimierten sollte immer nach extrakutanen Manifestationen der Leishmaniose gesucht werden. Eine ossäre Beteiligung bei kutaner Leishmaniose mit L. infantum ist bisher noch nicht beschrieben worden.

Lupus erythematosus (LE) is an autoimmune inflammatory disease with complex etiology. LE may present as a systemic disorder affecting multiple organs or be limited solely to the skin. Cutaneous LE (CLE) manifests with a wide range of skin lesions divided into acute, subacute and chronic subtypes. Despite classic forms of CLE, such as malar rash or discoid LE, little-known variants may occur, for instance hypertrophic LE, chilblain LE and lupus panniculitis. There are also numerous non-specific manifestations including vascular abnormalities, alopecia, pigmentation and nail abnormalities or rheumatoid nodules. Particular cutaneous manifestations correlate with disease activity and thus have great diagnostic value. However, diversity of the clinical picture and resemblance to certain entities delay making an accurate diagnosis The aim of this review is to discuss the variety of cutaneous manifestations and indicate the clinical features of particular CLE types which facilitate differential diagnosis with other dermatoses. Although in diagnostically difficult cases histopathological examination plays a key role in the differential diagnosis of LE, quick and accurate diagnosis ensures adequate therapy implementation and high quality of life for patients. Cooperation between physicians of various specialties is therefore crucial in the management of patients with uncommon and photosensitive skin lesions.

Cardiovascular diseases are the leading cause of mortality and morbidity globally. Clinicians must know cutaneous signs of cardiovascular disease, including petechiae, macules, purpura, lentigines, and rashes. Although cutaneous manifestations of diseases like infectious endocarditis and acute rheumatic fever are well established, there is an indispensable need to evaluate other important cardiovascular diseases accompanied by cutaneous signs. Moreover, discussing the latest management strategies in this regard is equally imperative. This review discusses distinctive skin findings that help narrow the diagnosis of cardiovascular diseases and recommendations on appropriate treatment.

BACKGROUND: Early recognition of perioperative anaphylaxis, a life-threatening, usually IgE-mediated, immediate hypersensitivity, is essential, but bedside diagnosis is not always straightforward because clinical presentation may vary.
OBJECTIVE: To describe early characteristics of perioperative immediate hypersensitivity, with special attention to cutaneous phenotypes, and identify risk factors for IgE-mediated allergy.
METHODS: We retrospectively analyzed data from adults with suspected perioperative immediate hypersensitivity who were investigated in two academic medical centers. Multivariable logistic regression was conducted to evaluate associations among patient, clinical, and paraclinical characteristics and IgE-mediated allergy.
RESULTS: Of 145 enrolled patients, 99 (68.3%) and 46 (31.7%) were respectively categorized in the IgE-mediated allergy and non-allergy groups. Cutaneous vasoconstriction phenotype (pallor, piloerection, thelerethism, and sweating with or without cyanosis) occurring within minutes (or even 1 minute) of drug exposure was strongly associated with IgE-mediated allergy (adjusted odds ratio [aOR] = 28.02; 95% CI, 4.41-305.18). IgE-mediated allergy was always life-threatening in this setting. Other early factors associated with allergy were low end-tidal carbon dioxide 25 mm Hg or less (aOR = 5.45; 95% CI, 2.39-26.45), low mean arterial pressure 60 mm Hg or less (aOR = 3.82; 95% CI, 1.28-17.31), and early cutaneous vasodilation (erythema, urticaria, and/or angioedema) (aOR = 2.78; 95% CI, 0.73-20.54). Late cutaneous vasodilation after restoration of hemodynamics corroborated the diagnosis of allergy (aOR = 23.67; 95% CI, 4.94-205.09). The best-fit model including three readily available variables (cutaneous phenotype involving the three modalities [reference lack of cutaneous signs], low mean arterial pressure, and low end-tidal carbon dioxide) had an area under the curve of 0.91.
CONCLUSIONS: Cutaneous vasoconstriction phenotype is associated with the strongest risk of life-threatening allergy and thus may be regarded as pathognomonic of perioperative IgE-mediated anaphylaxis.

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UNASSIGNED: During the neonatal life cycle, various dermatological conditions are common. In comparison to the skin of adults, neonates\' skins are more susceptible to infections in the first week of their lives. These infections often lead to many dermatological skin complications and create worrisome among parents. Hence, it is crucial to diagnose and manage such affected neonates at the earliest. This study aimed to highlight and estimate the occurrence of numerous dermatoses and assess various skin changes that occurred physiologically and pathologically in neonates.
UNASSIGNED: In this cross-sectional observational study, 474 neonates were enrolled in the neonatal intensive care unit. The whole newborn skin surface, comprising the palms and soles, scalp, mucous membranes, genitalia, hair, and nails, was scrutinized under adequate light, and all changes that occurred over the skin were observed and recorded. The sample size estimation was undertaken from the references that have the least prevalent cutaneous dermatosis: hypertrichosis desquamation and napkin dermatosis. A statistical analysis like the Chi-square test was performed to associate the type of dermatosis with the parameters of age, gender, delivery type, and birth weight.
UNASSIGNED: Of the total 474 cases, 90 (18.98%) had single dermatosis, while the remaining 384 (81.01%) had more than one form of dermatosis. Among the 384 cases, sebaceous gland hyperplasia (SGH) in 105 (22.15%), Epstein pearls in 50 (10.54%), erythema toxicum neonatorum (ETN) in 40 (8.43%), physiological desquamation in 25 (5.27%), lanugo hair in 20 (4.21%), miliaria in 22 (4.64%), salmon patch in 13 (2.74%), cradle cap/seborrheic dermatitis in 6 (1.26%), vernix caseosa in 12 (2.53%), transient neonatal pustular melanosis in 13 (2.74%), congenital melanocytic nevus in 20 (4.21%), hemangioma in 15 (3.16%), neonatal acne in 5 (1.05%), napkin dermatitis in 10 (2.10%), cutis marmorata in 6 (1.26%), milia in 2 (0.42%) intertrigo 3 (0.63%), collodion baby in 2 (0.42%), and neonatal occipital alopecia in 2 (0.42%) neonates each and others, respectively.
UNASSIGNED: The findings from the present study were representative of a specific racial/geographic distribution and will assist in adding or comparing the prevalence of neonatal dermatosis with other geographic regions as the array of dermatological characterizations in neonates varies as per time and place. This study aims to provide insight into the future implications in the neonatal dermatology domain and avoid further skin complications.

We present the case of a woman in her 70s who was diagnosed with rheumatoid vasculitis (RV) after initially presenting with systemic joint pain and erythema. RV, a rare complication of rheumatoid arthritis, involves inflammation of blood vessels, leading to various skin manifestations. The patient\'s complaints included fever, generalized joint pain, and skin manifestations that initially resembled erythema multiforme. However, a skin biopsy revealed vasculitis, which guided the RV diagnosis. Although rheumatoid arthritis primarily affects the joints, systemic implications such as RV can arise in rare cases. This case underscores the importance of a holistic and meticulous diagnostic approach, especially in older patients, as early detection and treatment are crucial for managing disease progression and associated complications. Collaborative care involving multidisciplinary teams is vital to achieving optimal outcomes in complex cases.

Metastatic Crohn\'s disease (MCD) is a rare cutaneous manifestation of Crohn\'s Disease (CD), defined as non-caseating, granulomatous skin lesions non-contiguous with the gastrointestinal (GI) tract. Most patients with MCD either have concomitant classic manifestations of CD or develop them within a few months to years. We report a case of MCD without known involvement of the GI tract, after more than three years from diagnosis. After failure or intolerance to several conventional treatments, including oral corticosteroids and azathioprine, adalimumab was initiated with a good response.  Diagnosis of cutaneous CD is made by a combination of clinical and histopathological findings. Therapeutic options include topical, intralesional, and systemic corticosteroids as well as topical and systemic immunosuppressants and immunomodulators. Surgical excision may be considered for refractory cases.