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

Böhler, L; Dumann, Konstantin; Kunz, Manfred

doi:10.1111/jdv.18113

Cited by 3 publications

(5 citation statements)

References 7 publications

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“…Malnutrition, particularly when being on a ketogenic diet, as well as severe diarrhea contributing to a nutrition deficiency due to impaired absorption in the gastrointestinal tract might trigger the onset of PP. [8][9][10]14 In 20% of patients, affected skin was associated with external stimuli, which might be explained by Koebnerization.…”

Section: Discussionmentioning

confidence: 99%

“…This difference led to debates on a predisposition in Asian individuals, particularly in Japanese and Korean individuals . Although cases have recently been described outside of Asia, this disease may remain underdiagnosed in other regions…”

Section: Introductionmentioning

confidence: 99%

“…The cause of PP is still unknown, but ketosis, friction, and sweating are associated risk factors. [8][9][10] Until 2020, 76% of PP cases reported worldwide were in patients of Asian origin. 8 This difference led to debates on a predisposition in Asian individuals, particularly in Japanese and Korean individuals.…”

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confidence: 99%

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Clinical and Pathological Spectrum of Prurigo Pigmentosa in Central European Individuals

Michor-Tscharre,

D’Alì,

Cota

et al. 2023

JAMA Dermatol

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ImportanceBased on early studies, prurigo pigmentosa (PP) was considered a rare inflammatory dermatosis affecting primarily Asian individuals. However, several case reports subsequently showed that the disease is not restricted to those of Asian origin. Large studies on PP in central European individuals, on the other hand, are missing.ObjectiveTo increase awareness of PP by describing the clinical, histopathological, and immunohistochemical features in central European individuals.Design, Setting, and ParticipantsThis observational, retrospective case series analyzed clinicopathological features of 20 central European patients diagnosed with PP. Data collection was performed by means of archive material, including physician’s letters, clinical photographs, and histopathological records, at the Department of Dermatology at the Medical University of Graz in Austria from January 1998 to January 2022.Main Outcomes and MeasuresDemographic, clinical, histopathological, and immunohistochemical characteristics for patients diagnosed with PP were recorded.ResultsOf the 20 patients included, 15 (75%) were female, and the mean (range) age was 24.1 (15-51) years. The study cohort consisted entirely of European patients. The most common site of involvement of PP was the breast, followed by the neck and back. Other involved clinical sites were the abdomen, shoulders, face, head, axillae, arms, and genital region and groin. Clinically, lesions were characterized by a symmetric pattern in 90% (n = 18) of all cases. Marked hyperpigmentation was observed only in 25% (n = 5) of patients. In some cases, triggers such as malnutrition, long-term pressure, and friction were noted. Histologic findings revealed presence of neutrophils in all cases and necrotic keratinocytes in 67% (n = 16) of cases. Immunohistochemistry results showed predominance of CD8+ lymphocytes in the epidermis, as well as the presence of plasmacytoid dendritic cells and myeloid cell nuclear differentiation antigen–positive neutrophil precursors.Conclusions and RelevanceThis case series found that most clinical features observed in Asian patients were also observed in central European patients, but hyperpigmentation was primarily mild to moderate. Histopathological features were similar to those reported in the literature with the additional presence of myeloid cell nuclear differentiation antigen–positive precursor neutrophils. These results expand previous knowledge about PP in central European individuals.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical and Pathological Spectrum of Prurigo Pigmentosa in Central European Individuals

Michor-Tscharre,

D’Alì,

Cota

et al. 2023

JAMA Dermatol

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“…Dear Editor, With great interest we read the article by Böhler et al 1 who reported a German patient with Nagashima disease (prurigo pigmentosa [PP]). 2 We report another German female with unusual PP distribution mimicking textile contact dermatitis.…”

Section: E T T E R T O T H E E D I T O R Prurigo Pigmentosa (Nagashim...mentioning

confidence: 99%

Prurigo pigmentosa (Nagashima disease) in bathing suit distribution mimicking contact dermatitis

Gambichler

Doerler

Scheel

et al. 2022

Acad Dermatol Venereol

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“…Prurigo pigmentosa (PP), also called Nagashima disease or "keto-rash", is a rare inflammatory skin condition first described in 1971 [1]. PP usually occurs in Asian women of childbearing age, but in recent years it has been increasingly diagnosed in individuals of other non-Asian populations [2][3][4]. PP is characterized by pruritic papulovesicles and erythematous plaques, usually symmetrically distributed on the trunk and neck, confluent and healing with a reticulated hyperpigmentation.…”

Section: Introductionmentioning

confidence: 99%

Prurigo Pigmentosa Induced by the Ketogenic Diet

Irimie¹,

Irimie²

2022

BUT_Series_VI

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Prurigo pigmentosa, also called Nagashima disease or "keto-rash", is a rare inflammatory skin condition. The etiology is unknown, but conditions associated with ketosis (fasting, diabetes, postbariatric surgery) often accompany this rash. Clinical case: A 13-year-old patient with no significant pathological history, overweight, on a ketogenic diet, was consulted for maculopapular skin lesions and erythematous plaques, discretely pruritic, persistent, confluent, with centrifugal extension, located at the posterior cervical, axillary, later thoracic, epigastric and lumbar regions. Hematological, biochemical, and immunological investigations were normal. Initial therapy with medium potency topical corticosteroid and anti-H1 antihistamine was ineffective, instead, under treatment with doxycycline and giving up the ketogenic diet, the resolution of the lesions was obtained, residual hyperpigmentation with a reticulated pattern persisting. Conclusions: We present this case to highlight the increasingly common association between prurigo pigmentosa and the ketogenic diet. Dermatologists should identify the relationship between the timing of rash onset and resolution in relation to diet, with a view to early diagnosis and treatment with optimal patient outcomes.

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

Cited by 3 publications

References 7 publications

Clinical and Pathological Spectrum of Prurigo Pigmentosa in Central European Individuals

Clinical and Pathological Spectrum of Prurigo Pigmentosa in Central European Individuals

Prurigo pigmentosa (Nagashima disease) in bathing suit distribution mimicking contact dermatitis

Prurigo Pigmentosa Induced by the Ketogenic Diet

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