Patient characteristics in tardive COVID-19 pseudoperniosis: a case series of 16 patients.

BACKGROUND: Acute pseudoperniosis (PP) has a recognized association with COVID-19 and tends to occur without cold precipitation in young, healthy patients, often without a clear history of COVID-19. These lesions usually resolve within 2 weeks and without long-term sequelae. In the early months of 2021, patients with delayed and protracted PP began to emerge. We have called this presentation 'tardive COVID-19 PP (TCPP)'. AIM: To consolidate and expand knowledge on TCPP, we describe the clinical characteristics, treatments and outcomes of 16 patients with TCPP who were reviewed by our outpatient dermatology service. RESULTS: The initial clinical manifestations were erythema, swelling and PP of the fingers in 56.2%, and of the toes in 31.2%, desquamation in 56.2% and acrocyanosis in 12.5%. Ten patients had eventual involvement of all acral sites. The median duration of symptoms was 191 days. Six patients reported close contact with a confirmed or suspected case of COVID-19, but only two had positive COVID-19 tests. Four patients experienced complete or almost complete resolution of symptoms, while the rest remain under active treatment. CONCLUSION: Unlike acute PP, TCPP has a protracted and delayed presentation that is typically associated with profound acrocyanosis. Patients with TCPP represent a new phenomenon that is part of the post-COVID-19 syndrome, with risk factors and pathophysiology that are not yet fully understood. Our data indicate that likely predisposing factors for developing TCPP include young age, a preceding history of cold intolerance and an arachnodactyloid phenotype. Anorexia, connective tissue disorders or sickle cell trait may also predispose to TCPP. In addition, low titre antinuclear antibody positivity, the presence of cryoglobulins, or low complement levels may represent further risk factors. Finally, prolonged low temperatures are also likely to be contributing to the symptoms.

Subcutaneous calcification presenting in a patient with mixed connective tissue disease and cutaneous polyarteritis nodosa.

Subcutaneous calcification often occurs in connective tissue diseases, most commonly scleroderma and dermatomyositis, but is rarely found in mixed connective tissue disease (MCTD). Cutaneous polyarteritis nodosa (PAN) is usually a primary skin disorder and although associated with connective tissue disease, has not been reported previously in MCTD. Calcinosis in cutaneous PAN is not a recognized feature. We describe the case of a 37-year-old woman who presented with tender ulcerated subcutaneous nodules on the lower legs consistent with cutaneous PAN, and she also showed features of MCTD with extensive secondary subcutaneous calcification. The use of systemic immunosuppressive treatment has improved the clinical features of PAN and MCTD but treatment of the calcification has proved challenging. No single medical or surgical treatment has been shown to be consistently effective in subcutaneous calcification, but the introduction of diltiazem in our patient has resulted in some improvement.

Ecthyma gangrenosum: an important feature of pseudomonal sepsis in a previously well child.

Ecthyma gangrenosum is a rare, distinctive skin disorder associated with potentially fatal underlying pseudomonal sepsis. Although typically occurring in neutropenic or immunocompromised patients, it can occasionally affect healthy children. The appearances are characteristic with small indurated vesicular papules progressing rapidly to infarcted necrotic areas with surrounding erythema and a typical black eschar. In young children, these are often accompanied by fever and diarrhoea. The absence of suppuration and slough distinguishes it from the more recognized pyoderma gangrenosum. Lesions can occur at any site although are most commonly found over the buttocks, limbs, axillae and perineum. We describe the case of a 28-month-old, previously well child who presented with typical features of ecthyma gangrenosum secondary to Pseudomonas infection who responded to appropriate antibiotic treatment. Despite a thorough search, no underlying cause was found. Early recognition and prompt treatment with antipseudomonal antibiotics is vital to reduce morbidity and potential mortality.

Penile carcinoma arising in balanitis xerotica obliterans.

Squamous cell carcinoma of the penis is an uncommon cancer, though in one study it accounted for 90% of all penile cancers. Its association with balanitis xerotica obliterans (BXO) is a rare though recognized occurrence. We describe a case of a 46-year-old Caucasian male who first presented to our open-access clinic with a mild phimosis. An elective circumcision was performed and histological examination of the circumcision specimen showed BXO. He was lost to follow-up but re-presented three years later with a persistent tender penile ulcer which on biopsy showed no obvious sinister pathology. He returned a further two years later with a short history of bleeding from the ulcer, and another biopsy now confirmed penile squamous cell carcinoma. Our case emphasizes the importance of regular review of patients with BXO, in particular those with persistent symptoms.

The head-up tilt test -- a cause of myocardial infarction.

INFARCTION: A 74-year-old man with no known ischaemic heart disease presented to the Cardiology Department with a history of multiple episodes of pre-syncope. During a head-up tilt test to investigate a neurocardiogenic cause, after glyceryl trinitrate provocation he became profoundly hypotensive and unwell. Subsequent ECGs and Troponin-T levels confirmed a Non ST-Elevation Myocardial Infarction. Angiography confirmed coronary artery disease. This case highlights a rare complication of tilt testing and emphasises that the test is not without risk.

Lichen sclerosus.

Lichen sclerosus is commonly seen in middle-aged and elderly women but it affects both sexes, can occur at any age and anywhere on the body and has been reported in most races. Its aetiology is uncertain but there is an increased incidence of auto-immune antibodies in lichen sclerosus and an association with auto-immune disease such as vitiligo, thyroid disease and alopecia areata. Lichen sclerosus has been observed in different generations of a family but no consistent genetic pattern has emerged. Lichen sclerosus typically affects the anogenital region and presents with an intractable 'burning' pruritus. Extragenital lesions do not itch. Oral lesions occur in lichen sclerosus and may resemble lichen planus. Lichen sclerosus exhibits the isomorphic or Koebner phenomenon. The literature supports the view that lichen sclerosus is not a precancerous lesion but there is a significant association with vulval squamous cell carcinoma. Patients with inactive, treated lichen sclerosus should be reviewed annually. The treatment of choice of uncomplicated genital lichen sclerosus is a topical steroid of a potent or super-potent category. In males, circumcision may be indicated if topical measures are unsuccessful. In women, surgery is considered predominantly for cancer confirmed by tissue examination.

Prenatal diagnosis and variable presentation of recessive X-linked ichthyosis.

Three infants with X-linked ichthyosis have been observed following pregnancies in which placental sulphatase deficiency (PSD) was suggested prenatally by low oestrogen excretion and an abnormal urinary steroid excretion pattern. This was confirmed in two cases by the absence of placental enzyme activity. In one case labour was spontaneous but all deliveries required Caesarean section. At 8 months the first infant showed an eczema in an atopic distribution but when seen at 5 years had typical X-linked ichthyosis. The skin of the second child peeled extensively at the age of 2 days but was not troublesome for 2 years, when ichthyosis vulgaris was diagnosed on clinical grounds. This pattern has persisted for 3 years. The third infant showed a mildly scaly skin in the neonatal period but at 3 months the features and distribution of X-linked ichthyosis were apparent. X-linked ichthyosis may have a variable presentation which is not always apparent at birth.

Intestinal permeability in patients with atopic eczema.

Intestinal permeability was investigated in adult patients with atopic eczema by in vivo and in vitro techniques. Patients with symptoms of 'immediate' food allergy were specifically excluded. A 51Cr-labelled ethylenediaminetetraacetate absorption test was carried out in eighteen patients. Their mean (+/- s.d.) 24-hour urine excretion following oral administration of the test substance (2.1 +/- 0.9%) did not differ significantly from that of thirty-four normal controls (1.9 +/- 0.5%). Small bowel permeability was estimated directly in jejunal mucosal samples in ten patients with three permeability probes of differing molecular weight. Mucosal permeability did not differ significantly from that of fifteen control patients for any of the test substances. Two patients had abnormal results by both tests and in one this was due to coeliac disease. These results suggest that altered intestinal permeability is not important in the pathogenesis of eczema. Patients demonstrating increased intestinal permeability should undergo jejunal biopsy to exclude significant small bowel disease.

Prognostic value of tumour thickness in cutaneous malignant melanoma.

The relation between survival and histological features in 91 patients with malignant melanoma was studied and the results were analysed by Clayton's method for interpretation of censored survival data. There was a significant correlation between tumour thickness and survival. The risk of dying from malignant melanoma after 10 years of follow up was less than 15% if the primary tumour was less than 1.5 mm thick but more than 80% if the lesion was thicker than 8 mm. The type of melanoma, level of invasion, mitotic rate, and presence of ulceration also correlated with survival, but these variables are related to tumour thickness.