Netherton's syndrome: successful treatment with isotretinoin

Abstract

A case of rituximab-related urticarial reaction in cutaneous B-cell lymphomaEditor Only a few cases of rituximab-related urticarial reaction have been reported in patient treated for B-cell lymphoma. This phenomenon may be related to the killing of tumoral CD20 B cells in the site of the tumour. We reported another case of urticarial reaction during rituximab infusion in a patient with cutaneous lymphoma.A 43-year-old man was referred to our hospital because of unusual erythematous skin patches involving the dorsal right infraaxillar regions. Biopsies confirmed the diagnosis of cutaneous follicular B-cell lymphoma. Complete evaluation did not reveal systemic disease. After an unsuccessful treatment with topical steroids, external radiotherapy was delivered with complete remission. However, a cutaneous relapse occurred 2 years later with thoracic and dorsal skin lesions. The patient was treated with four weekly intravenous infusions of 375 mg/m 2 of rituximab, the first infusion over a 6-h period, and the three consecutives over 1 h. One hour after the start of the first infusion and despite pre-medication with diphenhydramine, acetaminophen and steroid, the patient developed an urticarial reaction at the site of tumour patches and excision scars. The infusion was stopped for 2 h and the lesions spontaneously resolved. The patient could therefore complete the planned treatment with no side-effects. Surprisingly, the three last infusions of rituximab were well tolerated despite an accelerated infusion over 1 h. 1 Evaluation after the four cycles showed a complete remission status. The patient is still in complete remission with a 12-month follow-up.To date, few cases of localized rituximab-related urticarial reactions have been described. 2,3 These cases show urticarial lesions only located on the tumour sites. Moreover, this effect was observed during the first infusion which was realized according to the manufacturer guidelines with infusion times of 6 h and not during fast infusion of 1 h. 1 This could favour a more antitumoral effect rather than an anaphylactic mechanism. It is not known if this kind of reaction represents an indirect sign of antitumoral effect of rituximab on malignant CD20 B cells. Of note, the three reported patients experienced a complete remission after rituximab monotherapy. It is possible that this cutaneous reaction could be associated with a better response to rituximab as suggested for rash induced by epidermal growth factor receptor inhibitors 4 or bortezomib-induced cutaneous vasculitis. 5 Interestingly, the three reported cases only concerned primary cutaneous lymphomas. Maybe, the high concentration of malignant B cells into cutaneous lesions in primary cutaneous B-cell lymphoma may explain why this reaction has not been reported in systemic lymphoma. Additionally, all the reported cases concerned primary cutaneous B-cell lymphoma of follicular type. 2,3 The sensitivity of follicular B cells to rituximab may explain why the cases described actually are restricted to these lympho...

Improvement in Hair Loss and Better Hair Quality with Vitamin Therapy in Monilethrix

“…The condition also occurs in ichthyosis linearis circumflexa and Netherton's syndrome which is a triad of ichthyosiform erythroderma (inflamed, red, scaly skin), short, brittle, lustreless hair and atopic diathesis (predisposition to allergy problems) [21][22][23]. Reports have mentioned low dose retinoid therapy is reported to benefit Trichorrhexis invaginata [22,23]. This hair shaft disorder too improves on nutritional correction with caution to use low dose or be careful to avoid excess use of retinoids [22].…”

Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome

“…The use of systemic retinoids remains controversial, and the administration of low‐dose oral isotretinoin has been proposed by some authors as a steroid‐sparing treatment 6,7 . A recent report described successful treatment with oral isotretinoin 6 .…”

“…5 The use of systemic retinoids remains controversial, and the administration of low-dose oral isotretinoin has been proposed by some authors as a steroid-sparing treatment. 6,7 A recent report described successful treatment with oral isotretinoin. 6 Low-dose oral isotretinoin (0.2 mg ⁄ kg ⁄ day) failed in our patient, who had worsening of her condition, leading us to stop the medication 2 months later.…”

“…6,7 A recent report described successful treatment with oral isotretinoin. 6 Low-dose oral isotretinoin (0.2 mg ⁄ kg ⁄ day) failed in our patient, who had worsening of her condition, leading us to stop the medication 2 months later. Although UVA1 phototherapy has been reported as a treatment for NS, 8 there are no previous reports of NB-UVB successfully used in this condition, to our knowledge.…”

Picture of the Month—Diagnosis

“…Topical corticosteroids are inconsistently successful. Other treatments include lactate lotion, 12%, 4 calcineurin inhibitors (tacrolimus and pimecrolimus), 5 and isotretinoin. 6 Patients with Comèl-Netherton syndrome are likely more susceptible to systemic absorption of medication and are potentially at increased risk to experience adverse reactions to topical therapies.…”