Variable Response to Naltrexone in Patients With Hailey-Hailey Disease

Cao, Severine; Lilly, Evelyn; Chen, Shuo

doi:10.1001/jamadermatol.2017.5463

Cited by 27 publications

(29 citation statements)

References 3 publications

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“…Unexpectedly, our results showed a remarkable discrepancy compared to the results of the patients with Hailey-Hailey disease who were successfully treated with low-dose naltrexone in previous studies. [6][7][8][9] A possible explanation for this discrepancy could be the disease severity, as this was not fully clarified in the published cases of patients with Hailey-Hailey disease who were successfully treated with naltrexone. Our patients with a mild to moderate form of Darier disease also showed a satisfactory response.…”

Section: Base Linementioning

confidence: 99%

“…Previously, however, several studies reported success with lowdose naltrexone in treating refractory patients with Hailey-Hailey disease. [6][7][8][9] This prompted us to evaluate low-dose naltrexone (LDN) as a treatment option in Darier disease. The dosage in the published cases was very heterogeneous ranging from 3.5 to 50 mg. As LDN is dosed at 5 mg [p.o.]…”

Section: Introductionmentioning

confidence: 99%

“…Magnesium was added due to the fact that the SERCA-2 pump is magnesium-dependent and successful treatment with this had been published in the past. [6][7][8][9] Materials and methods Six patients (Table 1) with biopsy-proven Darier disease started off-label therapy with low-dose naltrexone [5 mg per os (p.o.)] and magnesium [200 mg per os (p.o.)]…”

Section: Introductionmentioning

confidence: 99%

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Variable response to low‐dose naltrexone in patients with Darier disease: a case series

Boehmer

Eyerich

Darsow

et al. 2019

Acad Dermatol Venereol

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Background Darier disease is a rare autosomal-dominant genodermatosis with a loss of function of a Ca 2+ -ATPase pump (SERCA2-pump). Clinically, the disease is characterized by red-brown keratotic papules mainly in seborrhoeic areas and has only limited and unsatisfactory treatment options. Previously, low-dose naltrexone was described as a successful treatment option in Hailey-Hailey disease, a genodermatosis with a genetic mutation coding for a similar loss of function of a Ca 2+ -ATPase pump (hSPCA1-pump).Objective To assess the efficacy of low-dose naltrexone as a treatment option in Darier disease. Methods Six patients with biopsy-proven Darier disease (four had severe, one had moderate and one mild clinical manifestations). The patients received off-label therapy with naltrexone [5 mg per os (p.o.)] and magnesium [200 mg p.o.]. Patients were followed up every 4 weeks for minimally 12 weeks. Upon clinical presentation, the disease severity and subjective pain and itch scores were assessed, and standardized photographs were obtained. ResultsThe clinical response to naltrexone varied after 12 weeks. The four patients with severe Darier disease showed worsening after initial improvement during the first 4 weeks, whereas the two patients with a mild to moderate clinical manifestation clearly improved, showing almost full remission after 12 weeks with complete flattening of the keratotic papules.Conclusion Low-dose naltrexone did not have an effect on severe Darier disease compared to Hailey-Hailey disease, but it was beneficial in mild to moderate forms of the disease. Further studies are needed to confirm these observations of variable responses.

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Section: Base Linementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Variable response to low‐dose naltrexone in patients with Darier disease: a case series

Boehmer

Eyerich

Darsow

et al. 2019

Acad Dermatol Venereol

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“…Three to fifty millgrams doses have been safely reported for an adequate response (Cao, Lilly, & Chen, ; Ibrahim et al, ), to our knowledge this is the first case treated with naltrexone 6.25 mg per day. If required, adjunctive agents such as acitretin and systemic antibiotics could be added (Cao et al, ; Kollman & Bass, ). Additional studies are needed to determine optimal dosage regimens and long‐term side effects.…”

Section: Resultsmentioning

confidence: 65%

“…Three to fifty millgrams doses have been safely reported for an adequate response (Cao, Lilly, & Chen, 2018;Ibrahim et al, 2017), to our knowledge this is the first case treated with naltrexone 6.25 mg per day. If required, adjunctive agents such as acitretin and systemic F I G U R E 1 Erythematous, fissured, macerated, symmetrical, and well-defined plaques were found involving both axillae F I G U R E 2 Three weeks after, erosions began to heal and improvement of erythema was observed F I G U R E 3 Complete resolution of the lesions 8 months after initiating treatment with low-dose naltrexone…”

Section: Discussionmentioning

confidence: 74%

Use of low‐dose naltrexone in the treatment of severe Hailey–Hailey disease: One case report

Garayar-Cantero

Martín

García

et al. 2019

Dermatologic Therapy

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Hailey–Hailey disease (HHD) or chronic benign familial pemphigus is an autosomal dominant genodermatosis with complete penetrance characterized by painful vesicles, erosions, and macerated intertriginous skin. We present a 66‐year‐old woman with a personal 35‐year history of pruritic recurrent vesicles and erosions in both axillae and inguinal folds. HHD was confirmed by cutaneous biopsy. Past treatments had failed, including topical corticosteroids, antibiotics and oral doxycycline, minocycline, dapsone, and acitretin. Phototherapy and intralesional injection of toxin botulinum A was performed in the axillae. The patient was started on naltrexone 6.25 mg nightly. Six weeks later, complete clearing was observed. At typical doses, naltrexone blocks μ and δ opiod receptors, thereby blocking the union of β‐endorphins at those sites. Paradoxically, at low doses, the partial binding to those receptors leads to a homeostatic increase of opioid receptors and an upregulation of endogenous opioids. Low‐dose naltrexone (LDN) may also exert an anti‐inflammatory action through its antagonist effect on toll‐like receptor 4 found on macrophages. We consider that LDN is an effective and safe alternative for the HHD, representing an important progress in the management of this disease with limited therapeutic options.

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Utility of Naltrexone Treatment for Chronic Inflammatory Dermatologic Conditions

et al. 2019

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IMPORTANCEDermatology is encountering increasing rates of autoimmune disease manifesting in primary skin conditions that are difficult to treat without a risk of immunosuppression. Naltrexone is an orally active opioid antagonist that influences a variety of systemic pathways, including the immune system, in low doses of 1.5 to 4.0 mg/d. This phenomenon has piqued the interest of researchers and practitioners in regard to low-dose naltrexone's potential in the treatment of several autoimmune conditions. OBJECTIVE To review the existing literature on naltrexone treatment for dermatologic conditions.EVIDENCE REVIEW A primary literature search was conducted using PubMed in April 2018 for all articles published from 1971 to April 2018. Search terms consisted of naltrexone or low dose naltrexone or low-dose naltrexone and dermatology or skin or hair or nails. Reviews, animal studies, and nondermatologic and pharmacologic studies were excluded.FINDINGS From 1037 articles, 22 were deemed to be appropriate for inclusion in this review for a qualitative synthesis. The 22 articles included randomized clinical trials, case reports, and series. There were 7 articles on low-dose naltexone, 1 on topical naltrexone, and 14 on high-dose naltrexone use in dermatology. In high, low, and topical doses, naltrexone was effective in treating pruritus attributable to atopic dermatitis, prurigo nodularis, cholestatsis, burn injury, systemic sclerosis, Hailey-Hailey disease, and lichen planopilaris. High-dose naltrexone was ineffective in treating flushing and uremic pruritus most likely because of the lack of opioid involvement in the pathophysiologic mechanisms of these conditions. CONCLUSIONS AND RELEVANCEThe findings suggest that low-dose naltrexone is safe and effective in the treatment of Hailey-Hailey disease and lichen planopilaris and both low-and high-dose naltrexone successfully treat pruritus attributable to various pathologic conditions; however, more adverse effects occurred in those taking high doses. Low-dose naltrexone has the potential for the treatment of chronic inflammatory skin conditions; however, additional evidence is needed for dosing and long-term treatment guidelines.

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Variable Response to Naltrexone in Patients With Hailey-Hailey Disease

Cited by 27 publications

References 3 publications

Variable response to low‐dose naltrexone in patients with Darier disease: a case series

Variable response to low‐dose naltrexone in patients with Darier disease: a case series

Use of low‐dose naltrexone in the treatment of severe Hailey–Hailey disease: One case report

Utility of Naltrexone Treatment for Chronic Inflammatory Dermatologic Conditions

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