Hailey-Hailey Disease With Coexistent Herpes Virus Infection: Insights Into the Diagnostic Conundrum of Herpetic/Pseudoherpetic Features in Cutaneous Acantholytic Disorders.

The specific histopathologic diagnosis of a primary acantholytic disorder takes into account the distribution and extent of acantholysis, presence or absence of dyskeratosis, nature of the dermal inflammatory cell infiltrate, and immunofluorescence findings. Herpes virus infection is a common cause of secondary acantholysis where distinctive viral cytopathic changes aid in making it a clear-cut diagnosis in majority of cases. We present a case of coexistence of Hailey-Hailey disease and herpes simplex virus infection to compare and contrast their histopathologic features. This is imperative because acantholytic cells from primary acantholytic disorders may occasionally show cytological features traditionally associated with herpes virus infection (pseudoherpetic changes). The objective of this article is to create a greater awareness of pseudoherpetic changes and also to explore the clinical significance of coexistence of a primary acantholytic disorder and herpes virus infection, as in this case.

Pseudomembranous changes in the dermis: A novel observation and potential clue for evolving lipodermatosclerosis?

Lipodermatosclerosis (LDS) is a well-recognized form of fibrosing panniculitis. Although chronic cases are readily diagnosed, early and evolving lesions may be clinically mistaken for cellulitis and other forms of panniculitis. Most pathologists are familiar with a pseudomembranous type of fat necrosis as a useful feature of chronic LDS. Although nonspecific, this distinctive pattern of fat necrosis helps in supporting a diagnosis of LDS in the appropriate clinical context. The histopathologic features of early and evolving LDS and those involving the dermis are less well documented. We report a case of early LDS showing extensive pseudomembranous changes in the dermis on a superficial skin biopsy where progression to a classic established lesion was documented clinically. We suspect that this previously unreported and unusual finding may be a histopathologic clue for evolving lesions of LDS.

Managing patients with hidradenitis suppurativa.

Hidradenitis suppurativa affects the apocrine-bearing areas of the skin. The onset is variable but usually occurs in the second and third decades of life, coinciding with development of the apocrine glands. The condition is characterised by painful, inflammatory papules and nodules which frequently progress to form abscesses, sinus tracts and hypertrophic scars. Bacteria are not felt to have a primary role in lesion formation, and abscesses are often sterile. The diagnosis of hidradenitis suppurativa is clinically based, without a specific diagnostic test. The most important non-genetic factors implicated in hidradenitis suppurativa are obesity and smoking. Locally recurring lesions can be treated surgically and more widespread disease may be better managed with a combination of medical treatment and surgery.

Managing actinic keratosis in primary care.

Actinic, or solar, keratosis is caused by chronic ultraviolet-induced damage to the epidermis. In the UK, 15-23% of individuals have actinic keratosis lesions. Risk factors include: advanced age; male gender; cumulative sun exposure or phototherapy; Fitzpatrick skin phototypes I-II; long-term immuno-suppression and genetic syndromes e.g. xeroderma pigmentosum and albinism. Actinic keratoses are regarded by some authorities as premalignant lesions that may transform into invasive squamous cell carcinoma (SCC) and by others as in situ SCC that may progress to an invasive stage. The risk of malignant change appears low; up to 0.5% per lesion per year. Up to 20-30% of lesions may spontaneously regress but in the absence of any reliable prognostic clinical indicators regarding malignant potential active treatment is considered appropriate. Actinic keratosis lesions may present as discrete hyperkeratotic papules, cutaneous horns, or more subtle flat lesions on sun-exposed areas of skin. The single most helpful diagnostic sign is an irregularly roughened surface texture: a sandpaper-like feel almost always indicates actinic damage. Dermatoscopy can be helpful in excluding signs of basal cell carcinoma when actinic keratosis is non-keratotic. It is always important to consider the possibility of SCC. The principal indication for referral to secondary care is the possibility of cutaneous malignancy. However, widespread and severe actinic damage in patients who are immunosuppressed is also a reason for referral.

Improving detection of non-melanoma skin cancer Non-melanoma skin cancer.

Basal cell carcinomas (BCCs) and squamous cell carcinomas (SCCs) are clinically and pathologically distinct and both are locally invasive. However, while BCCs rarely metastasise, SCCs have the potential to do so especially when they arise on the ears or lips. UV radiation is the most important risk factor for non-melanoma skin cancer (NMSC). The tumours most commonly arise in fair-skinned individuals on sun-damaged skin, especially the face. Incidence rises with age. Patients with one NMSC have a higher risk of developing another NMSC and of malignant melanoma. SCCs are frequently more difficult to diagnose than BCCs. Well differentiated lesions have a pronounced keratotic element. Poorly differentiated SCCs tend to be pink or red papules or nodules, lacking keratin, which may ulcerate. Around 5% of SCCs metastasise. High-risk SCCs include those: on the ear, lip, or sites unexposed to the sun and in chronic ulcers, scars or Bowen's disease. SCCs > 20 mm in diameter or > 4 mm in depth are high risk. Patients who are immunosuppressed, have poorly differentiated tumours or recurrent disease are also at increased risk. Patients with a slowly evolving or persistent skin lesion where cancer is a possibility should be referred to a dermatologist. Lesions suspected of being BCC should be referred routinely. Urgent referral should be reserved for cases where there is concern that a delay may have a significant impact because of the size or site of the lesion. Any non-healing lesions >1 cm with marked induration on palpation, showing significant expansion over eight weeks, should be referred urgently as they may be SCCs.

Improving the management of rosacea in primary care.

Rosacea is more common in women than men and occurs more frequently in fair-skinned individuals, usually in the middle years of life. It tends to localise to the cheeks, forehead, chin and nose, sometimes showing marked asymmetry. Only very occasionally does it involve areas other than the face. Rosacea is usually characterised by erythematous papules, pustules, and occasionally plaques (papulopustular rosacea), which fluctuate in severity, typically on a background of erythema and telangiectasia. In some individuals, facial redness can be prominent and permanent (erythematotelangiectatic rosacea). Important distinguishing features from acne are a lack of comedones, absence of involvement of extra-facial areas, and the presence of flushing. Hypertrophy of facial sebaceous glands, sometimes with fibrotic changes, may result in unsightly thickening of the skin. Men, in particular, may develop marked enlargement and distortion of the nose. Occasionally, the predominant feature of rosacea is swelling of the eyelids and firm oedematous changes elsewhere on the face. Involvement of the eyes is an important, underdiagnosed complication that may result in significant ocular morbidity. Involvement of the external eye surfaces by rosacea usually necessitates ophthalmological advice. There is often no correlation between the degree of ocular and cutaneous rosacea, and ocular rosacea may occur alone. Rosacea is a disfiguring condition that can have a major psychosocial impact, and its detrimental effect on emotional health and quality of life is often overlooked.

Improving outcomes in patients with psoriasis.

Psoriasis is a heterogeneous inflammatory disorder that targets the skin and joints. It affects 1.3-2% of the population. The diagnosis of plaque psoriasis is usually straightforward, a helpful diagnostic clue is the tendency for silver scales to appear after gentle scratching of a lesion. Stress, streptococcal infection and drugs including beta-blockers, antimalarials and lithium may precipitate or exacerbate psoriasis. Psoriasis, especially when severe, predisposes to metabolic syndrome, and patients with psoriasis are at increased risk of ischaemic heart disease, hypertension, stroke, type 2 diabetes and hyperlipidaemia. Additionally, psoriasis sufferers appear at increased risk of uveitis, inflammatory boweldisease, lymphoma, non-melanoma skin cancer, COPD and venous thromboembolism. Psoriasis should be assessed on the basis of: severity, impact on physical, psychological and social wellbeing, symptoms of arthritis and the presence of comorbidities. Poor response to topical therapy may be as much to do with lack of compliance as with lack of efficacy. The number of treatments each day should be kept to a minimum, and patients should be reviewed after four weeks when initiating or changing topical therapy to improve adherence to treatment and assess response. The majority of patients with psoriasis can be managed in primary care, although specialist care may be necessary at some point in up to 60% of cases. Patients with erythrodermic or generalised pustular psoriasis should be referred for a same day dermatological opinion, and if psoriatic arthritis is suspected, early referral for a rheumatological opinion is recommended.

Be vigilant for scurvy in high-risk groups.

Scurvy is caused by prolonged dietary deficiency of vitamin C, the plasma concentration of which appears inversely related to mortality from all causes. Its clinical importance relates principally to its role as a cofactor in a number of enzyme reactions involved in collagen synthesis, dysfunction of which disrupts connective tissue integrity, resulting in impaired wound healing and capillary bleeding. In the UK, overt scurvy is diagnosed only rarely. However, subclinical vitamin C deficiency appears quite common, one study estimated that 25% of men and 16% of women in the low income/materially deprived population had vitamin C deficiency, with smoking a strong predictor. Because many of the early symptoms of vitamin C deficiency (fatigue, malaise, depression and irritability) are non-specific, the diagnostic possibility of scurvy is usually delayed until haemorrhagic manifestations occur. The classical cutaneous features consist of perifollicular purpura, contorted (corkscrew) hairs and follicular hyperkeratosis, particularly affecting the legs. Large areas of purpura or ecchymosis may occur. Swelling and bleeding of the gums is an early mucosal symptom, and progressively severe gum disease causes loss of teeth. Subperiosteal haemorrhage, particularly in the femur and tibia, can present as pain, pseudoparalysis, swelling and discoloration of the legs. Haemorrhage into joints and muscle is very uncomfortable. Diagnosis is generally made on the basis of clinical features, corroborated by a history of dietary inadequacy, and the subsequent rapid resolution of symptoms with the restoration of an adequate vitamin C intake.