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 effect of chlorpromazine on the secretion of immunoreactive beta-MSH and prolactin in man.

The effect of chlorpromazine (50 mg. im) on the plasma concentration of immunoreactive beta-melanocyte-stimulating hormone (beta-MSH) and prolactin was studied in 8 hospitalized subjects with non-endocrine skin disorders. Plasma beta-MSH concentrations remained unchanged over a period of 7 h in 6 subjects. In the remaining 2 subjects there was a slight increase. Plasma prolactin concentrations were greatly increased in all subjects 1 1/2-3 h after the injection and had almost returned to pre-injection levels by 7 h. This suggests that the control of beta-MSH secretion in man, unlike that of prolactin in man and MSH peptides in other mammals, is not predominantly inhibitory. The reason for this discrepancy may be that beta-MSH is not a natural MSH in man and occurs as part of the lipotropic hormone (LPH) or as a breakdown product.

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.

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.

A sebotrophic stimulus in benign and malignant breast disease.

The sebum-excretion rate (S.E.R.) has been found to be increased in patients with benign as well as malignant breast disease. The S.E.R. was not increased in patients undergoing surgery for other diseases. It is considered that the sebotrophic hormone may also be mammotrophic. Drugs such as reserpine and the phenothiazines which increase sebum production may perhaps provoke benign as well as malignant breast disease.

IgA bullous pemphigoid: a distinct blistering disorder. Case report and review of the literature.

We report a patient with an eccrine carcinoma who developed localized blistering which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear IgA deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. A review of the literature reveals that in adults linear IgA deposition may occur in three separate situations: dermatitis herpetiformis, bullous pemphigoid and a third condition of which our case is an example which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma but the association lends further support to the concept that this eruption represents a variant of pemphigoid.

Thyroid disease and sebaceous function.

Sebum excretion rates (SER) were measured before and after treatment in patients with hypothyroidism and thyrotoxicosis. The mean SER in the former was significantly less than that in normal controls but there was no correlation between SER and the severity of the disease as indicated by serum thyroid-stimulating hormone levels. After treatment with L-thyroxine the SER increased but remained subnormal. By contrast the SER was not increased in patients with thyrotoxicosis and it was unaffected by treatment. The human sebaceous gland seems to respond to thyroid hormone mainly in the hypothyroid range.