Clinical manifestations and treatment outcomes in prurigo pigmentosa (Nagashima disease): A systematic review of the literature

Abstract

Background: Prurigo pigmentosa (PP) is a rare inflammatory dermatosis characterized by pruritic erythematous papules that coalesce to form a reticulate pattern. PP is often misdiagnosed, and patients are treated with ineffective therapies. Although the majority of reports about PP are from East Asia, patients of all backgrounds can be affected.Objectives: To perform a systematic review of reported PP cases with the purpose of summarizing the clinical presentation and treatment of PP.Methods: MEDLINE and Embase were searched for original articles describing PP. We identified 115 studies from 24 countries representing 369 patients to include in the analysis.Results: Of the 369 patients included in the analysis, the mean age was 25.6 years (range: 13-72 years) with 72.1% (266 of 369) female. Risk factors or aggravating factors were described in 52.3% (193 of 369) of patients and included dietary changes (25.5%, 94 of 369), friction (8.4%, 31 of 369), sweat (7.6%, 28 of 369), and ketonuria (5.1%, 19 of 369). Of those patients who experienced PP following dietary changes, 40.4% (38 of 94) started a ketogenic diet. Minocycline monotherapy was the most frequently prescribed treatment for PP (20.9%, 77 of 369), achieving complete resolution in 48.1% (37 of 77) of patients.Conclusions: PP is sometimes associated with ketogenic diets and can be effectively managed with oral tetracyclines.

Prurigo pigmentaire accompagnant l’apparition récente d’une acidocétose diabétique

“…On a recensé des cas de prurigo pigmentaire lors de jeûne intensif, d’anorexie mentale, d’hyperémèses, de chirurgie bariatrique, de diabète de type 1 et, plus récemment, de régimes cétogènes 1 , 3 . Les facteurs de risque au développement ou à l’aggravation des lésions comprennent des changements au régime alimentaire, le frictionnement, la sudation, la cétonurie et des changements hormonaux 3 .…”

“…On a recensé des cas de prurigo pigmentaire lors de jeûne intensif, d’anorexie mentale, d’hyperémèses, de chirurgie bariatrique, de diabète de type 1 et, plus récemment, de régimes cétogènes 1 , 3 . Les facteurs de risque au développement ou à l’aggravation des lésions comprennent des changements au régime alimentaire, le frictionnement, la sudation, la cétonurie et des changements hormonaux 3 . Les lésions sont prurigineuses et présentent habituellement des papules réticulées érythémateuses et des plaques situées sur le dos, le torse et le cou, épargnant le visage, les bras, les jambes et les muqueuses.…”

Prurigo pigmentosa with new-onset diabetic ketoacidosis

“…Prurigo pigmentosa has been reported with intense fasting, anorexia nervosa, hyperemesis gravidarum, bariatric surgery, type I diabetes mellitus and, more recently, ketogenic diets. 1 , 3 Risk factors for development or worsening of lesions include dietary changes, friction, sweat, ketonuria and hormonal changes. 3 The lesions are pruritic and usually present with erythematous reticulated papules and plaques on the back, chest and neck areas, sparing the face, arms, legs and mucosa.…”

“… 1 , 3 Risk factors for development or worsening of lesions include dietary changes, friction, sweat, ketonuria and hormonal changes. 3 The lesions are pruritic and usually present with erythematous reticulated papules and plaques on the back, chest and neck areas, sparing the face, arms, legs and mucosa. Mottled hyperpigmentation occurs as lesions resolve.…”

“…Although not a classic finding, a predominance of eosinophils has been reported with prurigo pigmentosa, and necrotic keratinocytes are not always present. 3 , 4 …”