Prurigo Pigmentosa: Demographics and Characteristics in 14 Caucasian Patients

Abstract

<b><i>Background:</i></b> Prurigo pigmentosa (PP) is an inflammatory dermatosis typically reported in young Asian women and characterized by recurrent papulovesicular lesions resolving with a postinflammatory reticulated hyperpigmentation. <b><i>Methods:</i></b> We have included all consecutive patients with PP diagnosed in our tertiary center between 2013 and 2020. The clinical information was retrospectively collected from medical records. <b><i>Results:</i></b> We identified 14 patients with PP. The median age at the time of diagnosis was 29.5 years (range 17–43 years), while the female-to-male ratio was 13:1. Diagnostic delay varied from 10 days to 10 years (mean of 25 months). Light microscopy studies consistently showed presence of a lymphohistiocytic infiltrate in combination in 40% of cases with neutrophils. Interface changes were found in 60% of cases. In 6 (43%) of 14 cases, there was a chronological relationship between the starting of a new diet and the development of the first flares. Treatment with doxycycline in 9 patients resulted in a complete regression of the lesions. <b><i>Limitation:</i></b> This was a retrospective study in a tertiary referral hospital. <b><i>Conclusions:</i></b> Our observations indicate that PP is not so rare in Europe as previously thought and is often diagnosed after a long delay. Demographics, clinical characteristics and triggering factors in Caucasian patients are similar to those described in the Asian population. Diagnosis is based on the peculiar recurrent course and distinctive clinicopathological features. Tetracyclines represent the first-line therapy in PP.

Clinical Features and Follow-Up of Prurigo Pigmentosa: A Case Series

“…As for management, resolution of PP has been reported with diet modification alone (as patient 7 in our series) [ 12 , 13 ] or treatment of hyperglycemia and dehydration [ 14 ]. Medically, oral doxycycline is considered to be the first-line treatment of PP [ 5 ], and administration of doxycycline was successful in all of the medically treated patients in our series. The plausible mechanism of action of doxycycline is the inhibition of neutrophil chemotaxis [ 2 ].…”

“…In our series, this association was striking with history of diet changes prior to development of PP in 75% of the patients. In a series of PP patients from Swiss, atopy was also found to be a predisposing factor [ 5 ]. However, in our series, none of the patients reported atopic diathesis.…”

Symmetrical pruritic lesions on the trunk in a young woman – a rare case of non‐associated Prurigo pigmentosa in Germany

“…Nevertheless, there is recent evidence that a relevant proportion of PP patients were on a special diet when skin lesions first developed. 2,[8][9][10] In most cases, PP occurs in the 2nd or 3rd decade of life, with women being affected about 2-3 times more often than men. PP often starts in spring or summer 6 and shows an acute onset with intensely pruritic, erythematous urticarial papules, papulo-vesicles or vesicles with a symmetrical reticular pattern of distribution.…”

Prurigo pigmentosa

“…Die Prurigo pigmentosa wurde erstmals 1971 von Nagashima in Japan beschrieben. Seither kamen die meisten Fallberichte aus dem asiatischen Raum, in den letzten Jahren wurde jedoch auch zunehmend über europäische Fälle berichtet [1,2]. Es handelt sich um eine in Schüben auftretende Dermatose unklarer Ätiologie.…”

“…Diese gehen meist in eine dann asymptomatische postinflammatorische Hyperpigmentierung über, welche weiterhin eine retikuläre Anordnung erkennen lässt. Frauen sind häufiger betroffen als Männer [1]. Wiederholt wurde ein Auftreten der Prurigo pigmentosa bei ketogener Stoffwechsellage berichtet, bspw.…”