Functional symptoms in dermatology: Part 2.

Functional disorders within dermatology present as various constellations of skin symptoms, but without evidence of organic pathology. Examples can include mucocutaneous pain syndromes, functional pruritus, somatoform pain disorder and rarer entities, such as undifferentiated somatoform idiopathic anaphylaxis and multiple chemical sensitivity syndrome. These conditions can have a significant impact on a patient's quality of life, and can present challenges in communication, investigation and management. The aetiology of functional disorders is not fully understood, but with an effective collaborative approach, a psychological explanation for these symptoms is often found. A structured approach to assessment can lead to a confident diagnosis, and understanding a patient's belief system and the impact of symptoms on their functioning can give better grounding for successful management. Treatment is dependent on the level of the patient's engagement with healthcare professionals, and often takes a measured and rehabilitative approach. Psychological therapies have been shown to be effective, often alongside both psychopharmacological and topical medications.

Functional symptoms in dermatology: Part 1.

Functional - or somatoform - symptoms are those that arise with no proven organic pathology. Also known as 'medically unexplained' symptoms, they can present in any medical speciality, including dermatology. Mucocutaneous pain syndromes and functional pruritus are two examples of functional disorders encountered by dermatologists. Patients presenting with somatoform symptoms have paradoxically complex and often subjectively severe symptomatology, yet minimal abnormalities on clinical examination or investigation. Such disparity can be frustrating and distressing for patients and clinicians alike, and there are many pitfalls regarding overinvestigation and misleading communication. However, with an honest and open approach - sometimes requiring collaboration with psychological services - management of functional symptoms can be effective, and patients can be successfully rehabilitated.

A bruise-like patch in a 4-year-old girl.

Dermatofibrosarcoma protuberans (DFSP), a rare medium grade sarcoma, occasionally occurs in childhood and is even more rarely present at birth. In children, the clinical appearance may be mistaken for a vascular malformation and so delayed diagnosis is not uncommon. Dermatofibrosarcoma protuberans is locally invasive and notorious for its high recurrence rate even after attempted wide local excision owing to extensive subclinical and asymmetrical extensions. In adult DFSP, Mohs Micrographic Surgery (MMS) is the treatment of choice because it offers a higher clearance rate compared to wide local excision. However, MMS may result in extended operating times owing to tissue processing and multiple stages. In children, this means a prolonged period under general anesthetic, which may be undesirable. We describe an interesting case of a 4- year-old girl diagnosed with DFSP. She underwent a modified MMS procedure in which she had two short general anesthetics. The advantage of MMS technique in which the full peripheral and deep margin of the specimen was examined.

Dermatoscopic features of extrafacial lentigo maligna.

The dermatoscopic features of classic lentigo maligna (LM) are well described; however, there is little literature available on extrafacial LM, which is a less frequently reported condition. The skin architecture is especially rich in adnexae on sun-exposed areas such as the face, relative to other parts of the body, thus it is possible that the dermatoscopic findings of extrafacial LM will differ from the usual findings of LM. We carried out a dermatoscopic study on three patients with extrafacial LM. The dermatoscopic patterns reflected the underlying histological features of the disease, with increased melanocytes seen along the rete ridges and around follicular ostia, which result in a unique pigment network architecture.

Eccrine porocarcinoma.

We present an unusual case of eccrine porocarcinoma in that diagnosis was delayed for many years after the initial lesion appeared, resulting in relatively large tumor.

Antibiotic prophylaxis in patients with valvular heart defects undergoing dermatological surgery remains a confusing issue despite apparently clear guidelines.

The guidelines pertaining to prescription of prophylactic antibiotics to prevent endocarditis during dermatological surgery appear clear and well-documented. The British Society for Dermatological Surgery, in agreement with the British Society for Antimicrobial Chemotherapy, state that antibiotic prophylaxis for endocarditis is not required for routine dermatological surgery procedures even in the presence of a pre-existing heart lesion. Pre-existing cardiac lesions include prosthetic valves, history of bacterial endocarditis, congenital cardiac malformation, rheumatic or other acquired valvular dysfunction, hypertrophic cardiomyopathy or mitral valve prolapse with regurgitation. It is important to distinguish between antibiotic prophylaxis for wound infection and that for bacterial endocarditis. Routine procedures, such as punches, shaves, curettage and simple excisions, performed on clean intact skin have an extremely low risk of wound infection (1-4%). The risk of wound infection increases to 5-15% with clean-contaminated skin surgery that includes procedures involving eroded or ulcerated skin, respiratory or buccal mucosa, flexural areas and protracted procedures such as Mohs' micrographic surgery. In such cases, antibiotic prophylaxis may be considered in patients with a cardiac lesion because a wound infection may result in bacteraemia and subsequent endocarditis. This should therefore not be considered 'routine' dermatological surgery. In contaminated, dirty and/or infected classes of wounds the risk of wound infection is higher (> 25%). Elective skin surgery should be postponed if possible until the wound infection is treated with therapeutic antibiotics.

Two squamous cell carcinomas arising in a linear epidermal naevus in a 28-year-old female.

The development of a squamous cell carcinoma (SCC) in a linear epidermal naevus is a rare event previously reported in long-standing and often extensive lesions of middle-aged to elderly patients. To the best of our knowledge, this case appears to be only the second report of an SCC arising in a linear epidermal naevus in a young patient and the first report of two SCCs developing in a small epidermal naevus, over a short time, in a young patient with no other risk factors.

Adult-onset Still's disease with atypical cutaneous features.

INTRODUCTION: The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of AOSD have been reported but these are not so well known. OBSERVATIONS: We report a patient with urticaria and fixed plaques and review the other 'atypical' cutaneous findings associated with AOSD. CONCLUSIONS: The diagnosis of AOSD can be made in the absence of the typical Still's rash but in the presence of other atypical cutaneous features.