Anne Howard

Follow up of 12 patients with trachyonychia

A follow‐up study was performed to determine the outcomes of all patients (five males and seven females, mean age 18.4 years) who were referred to a specialist nail clinic with trachyonychia. The average number of affected nails per patient was 8.8 fingernails and 6.2 toenails. None of our patients tested positive for onychomycosis. Nail biopsies were performed in two patients, revealing non‐specific changes. Eight patients did not present with or develop accompanying skin or mucosal disease. Two patients presented with alopecia areata and two others with psoriasis. Of our patients, 50% showed total resolution or marked improvement in their nail disease within the first 6 years regardless of treatment.

Twenty-nail dystrophy in a girl with incontinentia pigmenti

A 9‐year‐old girl with incontinentia pigmenti (IP) diagnosed soon after birth subsequently developed 20‐nail dystrophy at the age of 3 years. This persists unchanged 6 years later, and she has no other evidence of any other cutaneous disease, nor a family history of IP or nail dystrophy. Twenty‐nail dystrophy has been associated with a number of diseases including eczema, lichen planus, alopecia areata and psoriasis and a number of other congenital associations, but the association with IP appears to be unique.

Docetaxel-induced nail dystrophy.

A 73‐year‐old man with metastatic prostate cancer treated with weekly docetaxel chemotherapy for 5 months developed an acute nail dystrophy restricted to the fingernails. This was characterized by onycholysis, subungual haemorrhage and acute paronychia, progressing to a subungual abscess of the right index finger. Nail bed hyperaemia and haemosiderin‐like nail bed discoloration were present. Nail plate avulsion was performed to decompress the acutely painful subungual abscess. The right thumb, middle finger and left index finger demonstrated early, proximal white subungual collections of pus obscuring the lunula (onychophosis). Central nail plate fenestrations with a surgical drill led to exudation of purulent material. Cultures of the subungual abscess material yielded mixed organisms, possibly related to administration of flucloxacillin for 1 week prior to presentation. The patient completed a further two courses of docetaxel without sequelae, and the nail dystrophy appears to be resolving. Docetaxel‐induced nail changes are a common adverse effect, occurring in 30–40% of patients. Mild changes do not usually warrant the discontinuation of treatment.

Diseases of the generative nail apparatus. Part I: Nail matrix

Disorders of the generative structures of the nail produce a characteristic set of outcomes. The matrix is the seat of nail development and diseases or insults of this germinative epithelium will tell a story in the signs that unfold in the ensuing nail growth. This story will be influenced by the intensity, duration and extent of this pathology.

Efficacy, cutaneous tolerance and cosmetic acceptability of desonide 0.05% lotion (Desowen®) versus vehicle in the short-term treatment of facial atopic or seborrhoeic dermatitis

The differences between topical corticosteroids are based mainly on their potency, safety and patient acceptability. The aim of this study was to evaluate a mild‐ to mid‐potent topical corticosteroid, desonide 0.05%, on these three parameters in an Australian cohort of patients with facial seborrhoeic or atopic dermatitis. Eighty‐one adult patients were randomized to receive desonide 0.05% lotion or its vehicle, applied twice daily for 3 weeks under double‐blind conditions. In the active treatment group, 88% of patients had their skin condition cleared or almost cleared and only two patients experienced cutaneous adverse events (rash and pruritus). The acceptability of the lotion was high; 95% of patients stated they would use this topical corticosteroid again. These data support the short‐term use of desonide 0.05% lotion as a suitable agent for the short‐term treatment of facial dermatitis.

A case of multicentric reticulohistiocytosis responsive to azathioprine in a patient with no underlying malignancy

Multicentric reticulohistiocytosis (MRH), a rare histiocytic systemic condition characterized by mutilating arthritis and multiple cutaneous nodules, has been associated with malignancy including that of the breast, thyroid and colon. An unsubstantiated link with infectious agents such as mycobacterium tuberculosis has been described. Many treatments have been used with varying success. We describe the case of a 60‐year‐old man with MRH and no underlying malignancy who initially responded well to azathioprine and whose disease recurred upon cessation. A second course of azathioprine administered in conjunction with antituberculous treatment resulted in disease control. Broader associations and features of this rare disease are discussed.

Diseases of the generative nail apparatus. Part II: Nail bed

Nail bed insults may affect the resultant nail product in a number of ways. If focal in nature, the severity and duration of the insult will determine the appearance of the nail product. Widespread insults may alter the size, shape or colour of the nail plate. Nail bed insults tend to present with more immediacy than nail matrical insults, as there is no need to wait until the nail grows out until the sign is obvious. We are less interested in the history of the event, than we are with the clinical sign being presented to us. Should treatment be required for focal nail bed insults, treatment is likely to be local and curative. Widespread nail bed insults may be associated with systemic diseases and if a result of local problems these may be difficult to treat.

Dexamethasone iontophoresis for the treatment of nail psoriasis

Severe nail psoriasis can impact significantly on quality of life and is difficult to treat.

Multiple cutaneous leiomyomas leading to discovery of novel splice mutation in the fumarate hydratase gene associated with HLRCC

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare autosomal dominant condition, which manifests as cutaneous leiomyomas (CL), uterine fibroids and renal cell cancer (RCC). We describe the case of a 53‐year‐old woman who presented with multiple CL with a novel heterozygous canonical splice site mutation in intron 9 of the fumarate hydratase (FH) gene IVS 9–1 G>C (NM_000143.3:c 1391–1 G>C) that was not detected on initial screening of a mutation hotspot but was picked up on sequencing the remaining exons and splice site junctions. This report highlights the importance of clinical suspicion in the diagnosis of HLRCC in the absence of a family or personal history of cancer and despite initial genetic testing being negative.

Fingertip dermatitis refractory to topical corticosteroids associated with nail-patella syndrome

A 14‐year‐old girl and her 47‐year‐old father presented with fingernails that were hypoplastic, spoon‐shaped and ridged since birth. Fingertip dermatitis and paronychia were also observed in the daughter, which had been present since birth and had progressively worsened. The daughter denied trauma to her fingernails or chronic exposure to irritants and allergens. She had previously tried topical corticosteroids for 18 months without any benefit. We put forward the possibility of chronic paronychia and fingertip dermatitis, refractory to topical corticosteroids, as associations of digital nail–patella syndrome.