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Acrodermatitis continua of Hallopeau
Published in Robert Baran, Dimitris Rigopoulos, Chander Grover, Eckart Haneke, Nail Therapies, 2021
Second-line systemic therapy involves the use of cyclosporine and biologic agents. A recent review described that most of the biological therapies available for treating plaque psoriasis have shown response in the management of ACH as well, although higher doses and combination therapy are often required. The humanized anti-TNF-alpha monoclonal antibody infliximab, 3 mg/kg, was given intravenously. After 4 months, a substantial improvement of the lesions was observed. Etanercept 50 mg twice a week and methotrexate 10 mg/week seems to control the disease satisfactorily. Adalimumab, in combination with acitretin 50 mg/day, proved to be efficacious in dealing with a case of refractory disease. Adalimumab was also used as monotherapy in three patients with a dramatic and sustained response. The previous treatment of two of the three had failed with other biologic therapies. This showed that adalimumab can be effective in acrodermatitis continua, where other biological treatment has failed. Ustekinumab has been reported to be effective both as monotherapy and combination therapy with acitretin. There have also been published cases of ACH responding to the IL-17A inhibitors secukinumab and ixekizumab. There is one case report of treatment-resistant ACH reporting rapid improvement with the IL-1 inhibitor, anakinra. Brodalumab might be considered as a possible valid choice for the management of recalcitrant ACH, with two reported cases in the literature. Apremilast may be useful as the second-line treatment of ACH that is recalcitrant to conventional therapeutic modalities, as three case reports have demonstrated.
Ornithine transcarbamylase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
In chronic management of OTCD (Table 26.1), citrulline is substituted for arginine, as it is more palatable. Most patients are also treated with phenylbutyrate [65] as a source of phenylacetate. To many, phenylbutyrate is so unpalatable that a gastrostomy tube is required in order to avoid poor compliance, and even then, patients complain of a taste. For these reasons, sodium benzoate is preferred by some patients and some authorities. Most patients require restriction of the intake of protein, and most receive mixtures of essential amino acids to minimize the intake of nonessential nitrogen. Protein restriction and the use of benzoate/phenylbutyrate and deficiency of essential amino acids may lead to Kwashiorkor. Acrodermatitis-like skin lesions have been reported [66]. Complexities of management of OTCD were discussed in a patient diagnosed prenatally with a deletion who developed neurologic disease and anasarca, despite exemplary management and avoidance of hyperammonemia. Whole genome sequencing led to the diagnosis of a contiguous gene deletion involving chronic granulomatous disease, retinitis pigmentosa, and McLeod syndrome [10]. The authors stressed the need for detailed genetic analysis.
Psoriasis: Clinical Spectrum
Published in Siba P. Raychaudhuri, Smriti K. Raychaudhuri, Debasis Bagchi, Psoriasis and Psoriatic Arthritis, 2017
Chelsea Ma, Smriti K. Raychaudhuri, Emanual Maverakis, Siba P. Raychaudhuri
Palmoplantar pustulosis and acrodermatitis continua of Hallopeau are distinctive forms of localized pustular psoriasis. Palmoplantar pustular psoriasis is characterized by pustules on the palms and soles with an erythematous base and scale. It is often associated with psoriatic nail involvement. While plaque formation is not normally seen in palmoplantar pustulosis, this variant may be associated with plaque psoriasis. Acrodermatitis continua presents as a pustular eruption on the fingers and toes [41]. The pustules subsequently become confluent and can spread proximally to the dorsal hands and feet. Pustules may become generalized; if left untreated, the disease may lead to osteolysis of the distal phalanx.
Dermatology for the internist: optimal diagnosis and management of atopic dermatitis
Published in Annals of Medicine, 2021
Shanthi Narla, Jonathan I. Silverberg
Most importantly, all criteria highlight the importance of excluding other conditions such as scabies, seborrhoeic dermatitis, irritant or allergic contact dermatitis (ACD), ichthyosis, cutaneous T-cell lymphoma (CTCL), psoriasis, photosensitivity dermatoses, immunodeficiencies, and erythroderma of other causes [12,51]. Immunodeficiencies include Netherton syndrome, severe combined immunodeficiency, Wiskott-Aldrich syndrome, Autosomal dominant hyper-IgE syndrome (AD-HIES), Autosomal recessive HIES, Immune dysregulation polyendocrinopathy, enteropathy X-linked syndrome (IPEX), and acrodermatitis enteropathica [51]. Acrodermatitis enteropathica should be considered in infants with periorificial and acral dermatitis accompanied by alopecia, diarrahea, and failure-to-thrive. Workup for acrodermatitis enteropathica includes measuring plasma zinc concentration levels and screening for the SLC39A4 gene mutation [52–54].
Guselkumab for the treatment of palmoplantar pustulosis
Published in Expert Opinion on Biological Therapy, 2020
PPP has been discussed since the 1930 s. Barber et al. distinguished PPP from ‘Acrodermatitis continua Hallopeau’[6] (ACH), a rare inflammatory disease characterized by pustular eruptions beginning at the tips of fingers and toes and considered to be a localized form of pustular psoriasis (PP). ACH always involves the nail apparatus; therefore, alternative diagnoses such as PPP need to be considered when there is no nail involvement [7]. Plaque-type psoriasis often occurs in association with Barber’s PPP [6], which may be either psoriasis-related or a localized type of PP only seen in Western countries. Ingram [8] suggested that recalcitrant pustular eruptions of the extremities and Barber’ s PPP [6] exist within a spectrum of PP, and concluded that PPP was a localized type of PP; many subsequent studies have accepted this hypothesis. However, Andrews et al. described recalcitrant pustular eruptions on the palms and soles, where vesicles progress to form pustules [9,10]. This was interpreted as a response to a bacterial product (bacterid). Some reports suggest that antibiotics or surgical treatment of focal infections (such as tonsillectomy) may be helpful, but this is not always the case [11,12]. In Europe, whether PP and PPP differ remains the subject of debate [8,13]. Lever [14] introduced the terms PP and PPP. PPP presents with clinical features that are not seen in psoriasis patients, but cooccur with psoriasis vulgaris and/or a positive family history of psoriasis. PPP and psoriasis are closely related, and can in fact be indistinguishable [15].
Treatment of acrodermatitis continua of hallopeau with ixekizumab
Published in Journal of Dermatological Treatment, 2021
Austinn C. Miller, Timothy E. Holland, David J. Cohen
Acrodermatitis continua of hallopeau (ACH) is a rare inflammatory variant of pustular psoriasis often restricted to the distal portions of the hands and feet. This disease is characterized by its chronic relapsing nature and recalcitrance to multiple topical and systemic anti-psoriatic therapies. With its low prevalence and high resistance to treatments, no clinical guidelines for ACH currently exist. Fortunately, biologic medications formulated to target psoriatic pathways have demonstrated effectiveness for ACH.