Onychomadesis is characterized by separation of the nail plate from the matrix with persistent attachment to the nail bed and often, but not always, eventual shedding. Onychomadesis has been associated with infection, autoimmune disease, critical illness and medications. To our knowledge a literature review of all associations with onychomadesis has not been completed previously. Most commonly, onychomadesis has been reported in association with pemphigus vulgaris and hand-foot-mouth disease, and following chemotherapy or antiepileptic medications. This article summarizes these key culprit associations, postulates the pathogenesis of nail matrix arrest and summarizes the clinical outcomes. We conducted a retrospective review of cases of onychomadesis reported from January 1960 to March 2013. Using the PubMed database, the literature was searched using the following terms: 'onychomadesis' and 'proximal nail shedding'. Also, an Ovid search was carried out using the same terms. In total 56 articles have been published, including our previously reported series of idiopathic onychomadesis. Articles pertaining only to Beau's lines and not true onychomadesis were excluded. Onychomadesis has been associated with autoimmune disease, other major medical illness, neonatal illness, medication and infection.

Onychomadesis: literature review.

Beau lines, onychomadesis, and retronychia are nail dystrophies with distinctive clinical findings. Trauma has been reported as the initiating factor in each of these entities. Infections, severe medical illnesses, major surgery/anesthesia, medication side effects, and autoimmune disease can produce Beau lines and onychomadesis. This article illustrates the common underlying pathophysiological mechanism that produces each of these nail dystrophies.

Beau lines, onychomadesis, and retronychia: A unifying hypothesis

To the Editor: Mycosis fungoides is a lymphoproliferative neoplasm, a T cell lymphoma. It is primarily a disease of adults but can start in the first decade of life as a hypopigmented patch. A paper on this disease, including different treatment modalities, was published in Pediatric Dermatology in 2000 (1). I would like to report a 9-year-old girl with hypopigmented mycosis fungoides successfully treated with UVA1-light (340–400 nm). We evaluated a 9-year-old, otherwise healthy white girl from Sweden with a solitary hypopigmented patch (5 × 9 cm) in her left groin (Fig. 1). The center of the patch was erythematous and scaly. It was first identified 4 years earlier and unsuccessfully treated with midpotency topical corticosteroid ointments. A biopsy specimen showed a bandlike infiltrate of atypical lymphocytes with cerebriform nuclei and extensive epidermotropism with the formation of Pautrier microabscesses. Immunohistochemical analysis revealed CD4+ and CD8+ atypical T lymphocytes. There was a monoclonal rearrangement of the T cell receptor gamma gene. Results of blood analyses were normal and no enlarged lymph glands were found. Results of chest radiographs and abdominal scan ruled out systemic involvement. The patient was treated with UVA1 irradiation. One month after the treatment was finished, the patch had decreased in size to a diameter of 7 cm. At this time, microscopy still showed a bandlike, lichenoid infiltration in the superficial part of the dermis. Atypical lymphocytes were also seen in the epidermis but no abscess formation. The T cell receptor gamma gene was now polyclonal. One year after treatment, the patient was in complete remission. Four years after therapy, the skin of the previously hypopigmented patch was normal. Psoralen + UVA-light (PUVA) is an effective treatment modality for mycosis fungoides in childhood (1). UVA1-light is a new treatment strategy for mycosis fungoides. This therapy resulted in complete remission including repigmentation for my patient. The girl is now free from disease 4 years after treatment. Recent in vitro studies have shown that UVA1-light induces immediate apoptotic cell death in human T lymphocytes (2). UVA1 generates reactive oxygen species (ROS), i.e., singlet oxygen or superoxide anions. ROS initiate lipid peroxidation of cytoplasmic membranes leading to structural and functional alterations of the cell, followed by apoptosis.

Hypopigmented mycosis fungoides in a child successfully treated with uva1‐light

Abstract Nail conditions are not only aesthetic concerns, and nail changes may be a clue to an underlying systemic diseases or infection. Without timely treatment, nail diseases can continue to worsen and significantly impair performance of daily activities and reduce quality of life. Examination of the nails is essential at every medical visit, and may uncover important findings. Brittle nail syndrome, onychomycosis, paronychia, nail psoriasis, longitudinal melanonychia, Beau’s lines, onychomadesis and retronychia are common nail disorders seen in clinical practice. These conditions stem from infectious, inflammatory, neoplastic and traumatic aetiologies. Though each nail condition presents with its own distinct characteristics, the clinical findings may overlap between different conditions, resulting in misdiagnosis and treatment delays. Patients can present with nail plate changes (e.g. hyperkeratosis, onycholysis, pitting), discolouration, pain and inflammation. The diagnostic work-up of nail disease should include a detailed history and clinical examination of all 20 nail units. Dermoscopy, diagnostic imaging and histopathologic and mycological analyses may be necessary for diagnosis. Nail findings concerning for malignancy should be promptly referred to a dermatologist for evaluation and biopsy. Nail disease management requires a targeted treatment approach. Treatments include topical and/or systemic medications, discontinuation of offending drugs or surgical intervention, depending on the condition. Patient education on proper nail care and techniques to minimize further damage to the affected nails is also important. This article serves to enhance familiarity of the most common nail disorders seen in clinical practice. It will highlight the key clinical manifestations, systematic approaches to diagnosis and treatment options for each nail condition to improve diagnosis and management of nail diseases, as well as patient outcomes. Key messages Nail disease is not only a cosmetic issue, as nail changes can indicate the presence of a serious underlying systemic disease, infection or malignancy. Nail pain and changes associated with NP are physically and emotionally distressing and may contribute to functional impairment and diminished quality of life. LM is a hallmark sign of subungual melanoma and this finding warrants further investigation to rule out malignancy.

Optimal diagnosis and management of common nail disorders

140 with intralesional MA. In the paromo-u group, 86% (120 of 140 lesions) achieved relative improvement according to the definition, and 14% (20 of 120 lesions) showed no response (P < 0.05). A reduction in size was more prominent than changes in erythema or induration (60% reduction in size, 36% reduction in erythema, and 24% reduction in induration). Minor side-effects observed included transient itching (40% of lesions), mild erythema (30%), and different degrees of local inflammation. After four injections of MA, all lesions showed a complete response based on our definition.

Infant alopecia universalis: role of topical PUVA (psoralen ultraviolet A) radiation.

Background
Alopecia areata (AA) is common during childhood and rarely reported in infants. The four reported cases of AA in infants all exhibited circumscribed patches of alopecia that appeared at birth or shortly thereafter.

Jo Anne LaRow

Papers

Alopecia areata universalis in an infant.