Rumpel-Leede Phenomenon Following Radial Artery Catheterisation.

Rumpel-Leede phenomenon is a rarely diagnosed entity that can be seen in patients following the application of tourniquet-like forces to the extremities. This phenomenon describes petechiae and purpura secondary to venous compression and congestion, with its underlying aetiology involving the fragility of capillary vessels within the dermis. This condition is associated with chronic medical conditions such as diabetes mellitus, hypertension, dyslipidemia, peripheral vascular disease and systemic inflammatory diseases, including infections. In addition, patients with coagulopathy including thrombocytopenia or platelet dysfunction from antiplatelet use, or those with thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura, are predisposed to capillary haemorrhage and petechiae formation. In this report, we present a case of a patient who developed Rumpel-Leede phenomenon following catheterisation of the right radial artery with spontaneous resolution - where only five cases have been reported to date - with the aim to make clinicians aware of this condition and to avoid unnecessary interventions. LEARNING POINTS: Rumpel-Leede phenomenon is a benign condition that can be seen after tourniquet-like compression of a limb in those with capillary fragility.Dermatologists and other practitioners should remain aware of the phenomenon, helping to avoid unnecessary investigation.Rumpel-Leede phenomenon self-resolves, with only supportive treatment required with no reported lingering effects to date.

Generalized lichen nitidus in a 6-year-old girl with Down syndrome.

Lichen nitidus is a generally asymptomatic disease identified by shiny, flesh-colored papules that are often limited to the trunk, upper limbs, and genitalia. Here, we describe a generalized presentation of lichen nitidus in a 6-year-old girl with Down syndrome. Lichen nitidus shares a known association with Down syndrome, and several case studies document patients with Down syndrome developing generalized lichen nitidus. The extensive nature of our patient's lichen nitidus, as well as the uncommon distribution, particularly on the face, adds to the currently limited primary literature on this subject.

Seborrheic dermatitis with massive facial hyperkeratosis resembling acquired ichthyosis.

Seborrheic dermatitis is a common multifactorial skin disorder favoring the scalp, ears, face, and central chest. We present a case of an elderly woman with new-onset severe seborrheic dermatitis with massive facial hyperkeratosis mimicking ichthyosis. Clinicians should be aware of rare presentations of common conditions and should screen for associated medical comorbidities in new-onset severe skin conditions.

Development of Terry's nails after a gastrointestinal bleed.

Terry's nails are characterized by white opacification of the nails with effacement of the lunula and distal sparing. Described originally in 1954 by Dr. Richard Terry as a common fingernail abnormality in patients with hepatic cirrhosis, Terry's nails are now a known sequelae of other conditions such as congestive heart failure, chronic kidney disease, diabetes mellitus, and malnutrition. Often all nails of the hands are affected. Treatment of the underlying disease may result in resolution. We present a case of a 77-year-old man who developed Terry's nails following an acute gastrointestinal bleed and subsequent hemorrhagic shock. The development of Terry's nails after a gastrointestinal bleed has not been previously reported.

Incidental rhabdomyomatous mesenchymal hamartoma.

Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital tumor of the dermal and subcutaneous tissues, often presenting as a papule or a pedunculated or sessile mass at the midline on the head and neck. Although RMH is benign, some cases have reported associations with other congenital and syndromic anomalies. Thus, an accurate diagnosis is important, as an internal or systemic evaluation of the patient may be warranted. We report a case of a solitary RMH on the midline upper chest in a healthy 15-year-old girl.

Contrast-induced generalized bullous fixed drug eruption resembling Stevens-Johnson syndrome.

A 59-year-old woman with end-stage renal disease presented for suspected Stevens-Johnson syndrome that was ultimately diagnosed as generalized bullous fixed drug eruption (GBFDE) secondary to the administration of iodinated nonpolar radiocontrast. The patient had three previous episodes of a generalized bullous eruption after a thrombectomy, fistulogram, and an arteriovenous fistula revision, all requiring radiocontrast administration. Biopsies taken after previous eruptions demonstrated full-thickness epidermal necrosis, and she was diagnosed with Stevens-Johnson syndrome thought to be due to allopurinol. However, against medical advice she continued taking allopurinol and remained asymptomatic until the current presentation. Based on the clinical appearance and time frame of the eruptions, the patient was diagnosed with GBFDE due to radiocontrast. GBFDE, a rare variant of a fixed drug eruption, can be misdiagnosed as Stevens-Johnson syndrome due to their overlapping features of drug-induced whole-body blisters and variable degrees of epidermal necrosis.

Urticaria multiforme in a 2-year-old girl.

We describe a 2-year-old girl who presented to the emergency department for a 2-day history of a rapidly spreading, pruritic, erythematous rash. There were large polycyclic annular erythematous wheals on the face, trunk, and extremities. Urticaria multiforme was diagnosed. Systemic antihistamine therapy greatly regressed the dermal lesions in 24 hours. Urticaria multiforme, a benign cutaneous hypersensitivity reaction, is commonly misdiagnosed as erythema multiforme or a serum sickness-like reaction. Our case highlights the importance of differentiating urticaria multiforme to prevent unnecessary tests and provide proper treatment.

Filiform Verrucous Sarcoidosis of the Face: A Warty Report.

Sarcoidosis is a multisystem inflammatory condition of unknown etiology. Variability in the cutaneous features of sarcoidosis is profound, and its protean manifestations affirm the condition's designation as one of dermatology's "great mimics." Cutaneous phenotypes of sarcoidosis include but are by no means limited to ichthyosiform, alopecic, erythrodermic, angiolupoid, and verrucous variants. Verrucous sarcoidosis is an exceedingly rare manifestation, and previous reports of this phenotype are limited to 15 cases. Most cases in the extant literature presented on the extremities, with clinical features mimicking that of a common wart, or as verrucous crateriform nodules, ulcers, or cutaneous horns. Only 4 previous reports of facial verrucous sarcoidosis exist in the literature, and to our knowledge, no prior cases have demonstrated filiform lesion morphology. Here we present a case of filiform verrucous sarcoidosis in an otherwise healthy, middle-aged African American man, devoid of internal organ involvement and limited to the face, histopathologically confirmed by the presence of characteristic granulomata devoid of lymphocytic infiltrates.

Extensive Cutaneous Botryomycosis With Subsequent Development of Nocardia-Positive Wound Cultures.

Botryomycosis is a rare, chronic granulomatous infection caused by a response to bacteria, most commonly Staphylococcus aureus. Cutaneous manifestations, such as subcutaneous nodules, nonhealing ulcers, or sinus tracks, typically occur following inoculation of bacteria after trauma. Drainage from the skin lesions may contain yellow grains resembling those seen in actinomycosis and nocardiosis. A 20-year-old Hispanic male presented over the course of several years with a chronic nonhealing left posterior scalp wound. A car hit the patient when he was 2 years old and injured the scalp in the location of the skin lesion. Multiple wound cultures grew methicillin-resistant Staphylococcus aureus (MRSA), and biopsies were consistent with botryomycosis. He was treated with multiple surgical debridements, skin grafts, and various courses of oral and intravenous antibiotics with slight improvement. One reason for poor response to therapy was noncompliance with long-term home antibiotics. The most recent tissue culture grew MRSA in addition to Nocardia mexicana, and he experienced improvement on linezolid and minocycline. Although it is important to exclude nocardiosis and actinomycosis when diagnosing botryomycosis, our patient was diagnosed with botryomycosis after multiple biopsies and positive MRSA cultures 2 years prior to 1 positive N mexicana culture. Our case is a unique presentation of botryomycosis in an individual who subsequently developed Nocardia-positive wound cultures.

Case Report of Onychomycosis and Tinea Corporis Due to Microsporum gypseum.

BACKGROUND: Microsporum gypseum is a geophilic dermatophyte that colonises keratinous substances in the soil. Fur-bearing animals carry this dermatophyte but are rarely infected. Human infection can be acquired from the soil, carrier or infected animals, and rarely other humans. M gypseum is an uncommon cause of cutaneous infection in humans and typically manifests as tinea corporis, tinea barbae, and tinea capitis. Onychomycosis is rarely caused by M gypseum. CASE SUMMARY: We present a case of a 32-year-old white man who presented with a red scaly rash and nail dystrophy after adopting a pet rat 10 years prior to presentation. A fungal culture of a nail clipping grew out M gypseum, and the patient was treated with terbinafine daily for 6 weeks for dystrophic onychomycosis and tinea corporis. After the 6 weeks of treatment, the erythema at the proximal nail fold and distal finger had improved but still persisted. An additional 6 weeks of terbinafine daily completely resolved the clinical manifestations of onychomycosis. CONCLUSION: The increase in incidence of M gypseum onychomycosis over the past 2 decades is thought to be due to phylogenetic evolution of the dermatophyte from soil saprophyte to a human parasite. Increasing domestication of mammals is also thought to contribute to increasing incidence. Treatment consists of an extended course of terbinafine or itraconazole.

Merkel cell carcinoma in a vein graft donor site.

The development of malignancies in graft donor sites is rare and may be caused by de novo malignancies as well as metastatic and iatrogenic spread. Malignancies in graft donor sites are distinguished from Marjolin ulcers by some investigators because they occur in healed surgical wounds rather than in chronic wounds or unstable scars and tend to occur sooner after injury. We present a unique case of Merkel cell carcinoma (MCC) developing in a vein graft donor site 18 years after vein harvesting.

Role of the steroidogenic acute regulatory protein in health and disease.

Steroid hormones are an important class of regulatory molecules that are synthesized in steroidogenic cells of the adrenal, ovary, testis, placenta, brain, and skin, and influence a spectrum of developmental and physiological processes. The steroidogenic acute regulatory protein (STAR) predominantly mediates the rate-limiting step in steroid biosynthesis, i.e., the transport of the substrate of all steroid hormones, cholesterol, from the outer to the inner mitochondrial membrane. At the inner membrane, cytochrome P450 cholesterol side chain cleavage enzyme cleaves the cholesterol side chain to form the first steroid, pregnenolone, which is converted by a series of enzymes to various steroid hormones in specific tissues. Both basic and clinical evidence have demonstrated the crucial involvement of the STAR protein in the regulation of steroid biosynthesis. Multiple levels of regulation impinge on STAR action. Recent findings demonstrate that hormone-sensitive lipase, through its action on the hydrolysis of cholesteryl esters, plays an important role in regulating STAR expression and steroidogenesis which involve the liver X receptor pathway. Activation of the latter influences macrophage cholesterol efflux that is a key process in the prevention of atherosclerotic cardiovascular disease. Appropriate regulation of steroid hormones is vital for proper functioning of many important biological activities, which are also paramount for geriatric populations to live longer and healthier. This review summarizes the current level of understanding on tissue-specific and hormone-induced regulation of STAR expression and steroidogenesis, and provides insights into a number of cholesterol and/or steroid coupled physiological and pathophysiological consequences.

Up-regulation of steroid biosynthesis by retinoid signaling: Implications for aging.

Retinoids (vitamin A and its derivatives) are critical for a spectrum of developmental and physiological processes, in which steroid hormones also play indispensable roles. The StAR protein predominantly regulates steroid biosynthesis in steroidogenic tissues. We have reported that regulation of retinoid, especially atRA and 9-cis RA, responsive StAR transcription is largely mediated by an LXR-RXR/RAR heterodimeric motif in the mouse StAR promoter. Herein we demonstrate that retinoids are capable of enhancing StAR protein, P-StAR, and steroid production in granulosa, adrenocortical, glial, and epidermal cells. Whereas transient expression of RARα and RXRα enhanced 9-cis RA induced StAR gene transcription, silencing of RXRα with siRNA, decreased StAR and steroid levels. An oligonucleotide probe encompassing an LXR-RXR/RAR motif bound to adrenocortical and epidermal keratinocyte nuclear proteins in EMSAs. ChIP studies revealed association of RARα and RXRα with the StAR proximal promoter. Further studies demonstrated that StAR mRNA levels decreased in diseased and elderly men and women skin tissues and that atRA could restore steroidogenesis in epidermal keratinocytes of aged individuals. These findings provide novel insights into the relevance of retinoid signaling in the up-regulation of steroid biosynthesis in various target tissues, and indicate that retinoid therapy may have important implications in age-related complications and diseases.

Synergistic activation of steroidogenic acute regulatory protein expression and steroid biosynthesis by retinoids: involvement of cAMP/PKA signaling.

Both retinoic acid receptors (RARs) and retinoid X receptors (RXRs) mediate the action of retinoids that play important roles in reproductive development and function, as well as steroidogenesis. Regulation of steroid biosynthesis is principally mediated by the steroidogenic acute regulatory protein (StAR); however, the modes of action of retinoids in the regulation of steroidogenesis remain obscure. In this study we demonstrate that all-trans retinoic acid (atRA) enhances StAR expression, but not its phosphorylation (P-StAR), and progesterone production in MA-10 mouse Leydig cells. Activation of the protein kinase A (PKA) cascade, by dibutyrl-cAMP or type I/II PKA analogs, markedly increased retinoid-responsive StAR, P-StAR, and steroid levels. Targeted silencing of endogenous RARα and RXRα, with small interfering RNAs, resulted in decreases in 9-cis RA-stimulated StAR and progesterone levels. Truncation of and mutational alterations in the 5'-flanking region of the StAR gene demonstrated the importance of the -254/-1-bp region in retinoid responsiveness. An oligonucleotide probe encompassing an RXR/liver X receptor recognition motif, located within the -254/-1-bp region, specifically bound MA-10 nuclear proteins and in vitro transcribed/translated RXRα and RARα in EMSAs. Transcription of the StAR gene in response to atRA and dibutyrl-cAMP was influenced by several factors, its up-regulation being dependent on phosphorylation of cAMP response-element binding protein (CREB). Chromatin immunoprecipitation studies revealed the association of phosphorylation of CREB, CREB binding protein, RXRα, and RARα to the StAR promoter. Further studies elucidated that hormone-sensitive lipase plays an important role in atRA-mediated regulation of the steroidogenic response that involves liver X receptor signaling. These findings delineate the molecular events by which retinoids influence cAMP/PKA signaling and provide additional and novel insight into the regulation of StAR expression and steroidogenesis in mouse Leydig cells.