Dermoscopy and reflection confocal microscope features of pigmented prurigo.

BACKGROUND: Pigmented prurigo (PP) is a chronic and recurrent inflammatory skin disease. PP is not common clinically, but it is easily misdiagnosed because of its diversified clinical manifestations in different stages. MATERIALS AND METHODS: We retrospectively analyzed the clinical, histopathological, dermoscopy, and reflectance confocal microscopy (RCM) features of 20 patients diagnosed as PP. RESULTS: The female predominance ratio was revealed with male to female of 1:4. Seven female patients were on a diet (without staple food) and one patient had a history of diabetes. Eight cases were suffered in spring, six cases in winter, three cases in summer, and three cases in autumn. Multiple sites were involved in 13 cases. Four patients had urticarial papules and plaques. Nineteen patients had erythematous papules with reticular distribution, of which 14 cases accompanied reticulate hyperpigmentation, four cases with papulovesicle, and two cases accompanied with pustules. One patient only showed reticulate hyperpigmentation. In the early lesions, dermatoscopy showed pink oval lesions, punctate or linear vessels, and pale yellow rings around the skin lesions. RCM is characterized by spongiosis, spongy vesicle, neutrophils scattered in the epidermis, which was consistent with epidermis spongiosis, neutrophils infiltrating into the upper epidermis and necrotic keratinocytes in histopathology. In the fully developed lesions, dermatoscopy showed pink lesions with brown pigment granules in the center and linear vessels in the edge. RCM showed that demarcation of epidermis and dermis is not clear, and inflammatory cells can be seen in the upper dermis and histopathologically lesions assumed a patchy lichenoid pattern, and the inflammatory cells infiltrating the dermis were dominated by lymphocytes. In the late lesions, dermatoscopy showed grainy grayish-brown or yellowish-brown pigmentation surrounding the hair follicle merging with each other. RCM showed that pigment granules were increased on the ring of basal cells, inflammatory cells were sparsely infiltrated in the dermal papilla and superficial layer, and epidermis slightly hyperplastic, with melanophages and a few lymphocytes infiltrating the superficial dermis in histopathology. CONCLUSION: PP is easily misdiagnosed and not always occurs in those on a restrictive diet. A combination of dermatoscopy and RCM is helpful for its diagnosis of PP.

Post bariatric surgery prurigo pigmentosa in an adolescent.

Prurigo pigmentosa is an inflammatory skin condition that is characterized by the presence of pruritic reticulated hyperpigmented papules or vesicles of the upper trunk. Previously, this condition was rarely encountered until the rise in popularity of weight reduction methods. Even with the increased prevalence, most affected patients are still adults. We present the case of an adolescent who was successfully treated with doxycycline and encouraged to resume eating.

Prurigo Pigmentosa: Demographics and Characteristics in 14 Caucasian Patients.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. LIMITATION: This was a retrospective study in a tertiary referral hospital. CONCLUSIONS: 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.

[Prurigo pigmentosa during pregnancy]. / Prurigo pigmentosa au cours de la grossesse.

INTRODUCTION: Prurigo pigmentosa is a rare inflammatory dermatosis characterized by pruritic and reticulate papules on the trunk leaving hyperpigmentation. This dermatosis has been rarely described outside Asia. The pathophysiology remains obscure. CASE REPORT: We report the case of a 21-year-old North-African woman presenting with a highly pruritic eruption with numerous erythematous macules and papules coalescing in a reticular pattern on the trunk. The eruption occurred during the first trimester of pregnancy, which was marked by severe vomiting resulting in weight loss of 13kg with ketonemia and ketonuria. Taking into account the characteristic pattern of the eruption, the absence of differential diagnosis, and the histological examination, we concluded on a diagnosis of prurigo pigmentosa. Progression of the disease exhibited phases of decreased inflammation and of pruritus alternating with episodes of inflammatory flares triggered by relapses of ketonemia. The symptoms finally resolved during the 2nd trimester of pregnancy after vomiting ceased. Secondary reticulated hyperpigmentation was observed. CONCLUSION: Although rare, the highly evocative clinical presentation of the eruption should help clinicians in diagnosing prurigo pigmentosa. While pathophysiology remains undetermined, prurigo pigmentosa was reported in cases of intense fasting, anorexia, type-1 diabetes, and in two other cases of pregnancy with severe vomiting. Our case underlines the need to screen for and treat underlying ketonemia to achieve control of the inflammatory flares of prurigo pigmentosa.

Prurigo pigmentosa in a fair-skinned European woman: Dramatic improvement with doxycycline.

INTRODUCTION: Prurigo pigmentosa is a rare inflammatory, pruriginous skin disease seen predominantly in young Asian women, with average age of onset in the mid-20s. OBSERVATION: A 25-year-old fair-skinned European woman presented with a two-year history of pruriginous skin lesions recurring fortnightly. The initial lesions were inflammatory papules, which first emerged on the back of the neck before spreading to the shoulders, below the breasts and the back. The papules resolved leaving a reticular hyperpigmented network that gradually worsened after each episode. The clinical presentation and histopathological findings were consistent with a diagnosis of prurigo pigmentosa. Doxycycline 200mg/day was initiated, with rapid resolution, absence of any further flare-ups and gradual regression of the reticular pigmentation. DISCUSSION: Prurigo pigmentosa is a skin disease of stereotypical presentation marked by frequent inflammatory flare-ups involving the trunk that are followed by periods of remission with residual hyperpigmentation. Herein we report a case observed in a fair-skinned French female subject. It is important that dermatologists are able to recognize it and distinguish it from other forms of pruriginous papular dermatosis, owing to the dramatic efficacy of tetracyclines in controlling the inflammatory flares and in reducing the adverse aesthetic impact of hyperpigmentation.

Folliculitis in prurigo pigmentosa: a proposed pathogenesis based on clinical and pathological observation.

BACKGROUND: Prurigo pigmentosa is a rare inflammatory dermatosis whose exact etiology is not understood yet. The purpose of this study was to provide evidence of hair follicle involvement in the pathogenesis by analyzing its clinicopathologic features. METHODS: Patients who fulfilled both the clinical and histological diagnostic criteria of prurigo pigmentosa were recruited. Their histopathologic findings, clinical features and medical histories were analyzed. RESULTS: A total of 32 confirmed patients were enrolled from 2002 to 2013. Their ages ranged from 11 to 79 years with a female predominance. Patient lesions were primarily reddish-brown and located on the back. A total of 25 patients (78%) had pathological involvement of hair follicles, either bacterial colonies in the hair follicles (21/32, 66%), folliculitis (8/32, 25%) or perifolliculitis (15/32, 47%). There was a significantly higher proportion of patients with hair follicle involvement compared with control groups with either noninflammatory (5/43, 12%, p < 0.001) or inflammatory skin diseases (12/32, 38%, p = 0.002) on the back. Minocycline was an effective antibiotic treatment either singly or in combination with steroids. CONCLUSIONS: The frequent presence of bacterial colonies along with sequelae of inflammatory changes on biopsy provides new evidence to support the theory that prurigo pigmentosa is a reactive inflammation associated with bacterial folliculitis.

The efficacy of minocycline in inflammatory dermatoses: a case of prurigo pigmentosa of prepubescent onset in Western world.

We present a 21-year-old Italian girl with an 8-year history of missed diagnosed prurigo pigmentosa (PP) successfully treated with short monotherapy with minocycline. PP is an inflammatory disease characterized by recurrent pruritic erythematous papules followed by reticular hyperpigmentation usually located on the trunk. About 300 cases of PP have been described mainly in Japan, whereas only few cases have been reported in Italy. This report shows that minocycline is rapidly effective probably through its ability to scavenge reactive oxygen species and to inhibit the chemotaxis and neutrophil function. Other than its ethnic rarity, this case is very interesting because it is the third case of PP in Caucasian patient with prepubescent onset.

Prurigo Pigmentosa Successfully Treated with Oral Minocycline: A Case Report.

Purpose: We aimed to report a case of prurigo pigmentosa (PP) with blisters. Patients and Methods: A 21-year-old male patient presented to our hospital with erythema, papules, and blisters all over the front chest and back. Densely distributed round and oval edematous erythema was noted on the neck, front chest, and back of the patient. Histopathologic examination of the skin lesions (blisters) on the chest revealed epidermal cell hyperkeratosis. He was diagnosed with PP and was treated with oral minocycline. Results: After 1-week minocycline administration, the patient's itching was relieved, and the blisters dried up. His skin lesions recurred following self-withdrawal from the medication; subsequently, the patient was administered oral minocycline treatment again. After 1-month continuous oral treatment, the drug was discontinued. No recurrence of symptoms was noted. Conclusion: Oral minocycline is effective for the treatment of PP.

A Rare Case of Prurigo Pigmentosa in a Danish Sibling Couple.

Prurigo pigmentosa (PP) is probably underdiagnosed due to lack of awareness. Previously, it was assumed that PP primarily affected Japanese females; however, more cases are reported worldwide, and the pathogenesis is still not completely understood. In this case report, we present two healthy Danish siblings, who developed PP approximately 2 weeks after starting a ketogenic diet, suggesting that both increased levels of ketone bodies in the blood together with a genetic predisposition might play a role in the development of PP.

Prurigo pigmentosa post-bariatric surgery: a case report.

Background: Prurigo pigmentosa (PP), also referred to as Nagashima disease, is an acquired dermatological condition that is characterized by pruritic erythematous eruptions including papules and plaques surrounded by a reticular pattern of hyperpigmentation. It was first described in 1971 when Japanese dermatologist Masaji Nagashima noticed similar dermatological manifestations among 8 patients of Asian descent. The disease has been strongly associated with ketosis and ketogenic diet; however, the underlying etiology is unknown. Effective treatment involves an oral antibiotic and a balanced diet. The disease classically affects young women of Asian descent, but cases affecting males and people of non-Asian ancestry have also been observed. We present an unusual case of PP in a Saudi male that started 18 days following bariatric surgery. Case Description: The patient is a 25-year-old morbidly obese Saudi male who complained of an intensely pruritic rash 3 weeks after undergoing bariatric surgery. His past medical history is positive for irritable bowel syndrome (IBS). A punch skin biopsy yielded the diagnosis, and a combination treatment consisting of minocycline and a topical corticosteroid was prescribed. The patient presented again 2 weeks after the initial visit with complete resolution of the rash. He reported adherence to the medication regimen. Pruritus had resolved. Upon examination, post-inflammatory hyperpigmentation in a reticular pattern was observed over the trunk, upper abdomen, and chest. Hydroquinone 4% cream was prescribed to treat the post-inflammatory hyperpigmentation. Conclusions: Nowadays, PP cases are being reported from all around the world, including cases of PP that appeared following bariatric surgery for weight loss without ketogenic dietary modification. Theoretically, the typical PP patient is a young adult female from Eastern Asia who has been adherent to a ketogenic diet. In contrast, we report a case of a Saudi male who did no dietary modifications, but underwent bariatric surgery. Treatment with minocycline resulted in successful complete resolution.

Prurigo Pigmentosa Induced by a Ketogenic Diet.

Prurigo pigmentosa is an important cause of reticular pruritic rash that has been under-reported in the United States. To ensure proper patient care, it is important for dermatologists to be aware of its presentations and associated factors. Here, we present an uncommon case of prurigo pigmentosa induced by a ketogenic diet and discuss the links between this condition and the state of ketosis, a relationship every provider should be conscious of.

Remission of Prurigo Pigmentosa after Breaking Ketogenic Diet and Resuming Regular Diet.

Prurigo pigmentosa (PP) is a rare skin disorder presenting as erythematous urticarial papules on the chest and leaving reticulated pigmentation. Although the etiology of PP is unknown, conditions associated with ketosis such as diabetes mellitus, ketogenic diet (KD), and anorexia nervosa are implicated. Herein, we report a 21-year-old woman who developed PP after adhering to a KD and responded to resuming a regular diet.

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

Introduction Prurigo pigmentosa (PP) is an underrecognized disease in the Western population. Our aim is to describe the clinical features and follow-up outcomes of Caucasian patients diagnosed with PP. Methods This case series was conducted in the dermatology outpatient clinic of a tertiary hospital. Patients with confirmed PP from May 2020 to June 2021 were included in the study. Patient demographics, clinical features, potential triggers, treatment and follow-up data were recorded. Results A total of eight patients with female predominance were identified. The mean age of the patients was 24.5. The duration of symptoms ranged from four days to six months. All patients presented with pruritic, papular or papulovesicular lesions. Net-like hyperpigmentation was also present at the initial visit in two patients, in whom the duration of the symptoms was the longest. Lesions were most commonly located on the chest and back. Six of eight patients reported alteration of diet that potentially led to ketosis. Doxycycline 200 mg daily for two weeks led to a complete response in all six medically treated patients. Duration of follow-up ranged from 1-14 months (mean: 7.2 months). In five patients with a follow-up duration of more than three months, postinflammatory hyperpigmentation was resolved without any treatment. Only one patient had a recurrence. Conclusion PP does not seem to be a rare disease. Young women are most commonly affected, and ketosis stemming from decreased calorie intake may be the etiological factor in the majority of the patients. Dermatologists should be familiar with early signs of PP in order to minimize unnecessary therapies, recurrences and long-lasting hyperpigmentation.

Prurigo Pigmentosa Following a Keto Diet and Bariatric Surgery.

Prurigo pigmentosa (PP) is a rare pruritic condition with idiopathic etiology that affects mostly females. It most commonly presents as a rash on the neck and trunk. We report the occurrence of PP in a young woman on two separate occasions; her first episode was following a ketogenic diet and second after undergoing a laparoscopic gastric sleeve surgery. This presents as a unique case because, to our knowledge, PP has only been reported in a small number of cases in the Western world. This presentation could be suggestive of a stronger relationship between PP and the metabolic state of the body. It also outlines the effectiveness of treatment options currently in use for treating PP.

Prurigo pigmentosa clinically and immunologically mimicking autoimmune bullous disease: A case report.

A 15-year-old Japanese male noticed brown macules on his back 9 months ago. Initial examination revealed reticulated infiltrative erythema and pigmentation with blisters on the erythema of the back. Histopathology showed blisters with eosinophil infiltration in the epidermis, and direct immunofluorescence showed negative results for immunoglobulin (Ig) G, Ig A, Ig M, and C3 in the epidermal basement membrane zone. Immuno-serological tests revealed the presence of IgG antibodies against BP180, linear IgA disease antigen 1 (LAD-1), and laminin α3. The autoimmune bullous disease was suspected, and prednisolone at a concentration of 20 mg/day (0.3 mg/kg/day) was started. When the prednisolone dose was reduced to 10 mg/day, erythema and blisters recurred. The patient was diagnosed with prurigo pigmentosa based on clinical features and was treated successfully with oral doxycycline hydrochloride hydrate and topical tacrolimus ointment. This is the first case of prurigo pigmentosa with blisters in which autoantibodies to the epidermal basement membrane zone were found, which might be secondary non-pathogenic antibodies.

Prurigo Pigmentosa: A Case Report With Unusual Presentation.

Prurigo pigmentosa (PP) is an idiopathic cutaneous inflammatory disorder. Here we report a 50-year-old healthy male of Arabic descent who presented with a six-month history of very itchy persistent skin lesions on his back. Skin examination revealed multiple brownish non-scaly excoriated papules and patches in the midline of his lower back. The differential diagnosis includes lichen planus (LP), confluent and reticulated papillomatosis (CARP), and PP. Skin biopsy revealed acanthosis, spongiosis, and dyskeratotic keratinocytes in the epidermis. The dermis showed mild perivascular lymphocytic infiltrate. Based on the previous clinicopathological findings, the patient was diagnosed with PP. He was prescribed doxycycline 100 mg once daily (OD) for two months. Two months after treatment, all lesions disappeared completely. After one year at the follow-up, he presented with a recurrence of the same skin lesions at the same site. We restarted him on doxycycline treatment.

A Case of Exfoliative Vesiculobullous Prurigo Pigmentosa Cured by Doxycycline and Topical Tacrolimus.

Prurigo pigmentosa is a rare inflammatory skin disease characterized by an unexpected onset of diffuse erythematous papules and macules usually on the chest, neck, and back. These generally resolve, leaving reticular hyperpigmentation. Rarely, vesicular or bullous forms have been reported. We present a case of exfoliative vesiculobullous prurigo pigmentosa in a 13-year-old boy. He presented with symmetrical eruption of papules and vesicles on his back, neck, and chest in the last 10 days, causing pruritis and prickling sensation. Within a few days, the bullous lesions and all affected areas of the skin showed exfoliation. Histological study and clinical findings indicated the condition to be vesiculobullous prurigo pigmentosa with exfoliation. Treatment with doxycycline 200 mg/day and topical tacrolimus ointment showed a good response. The lesions resolved, leaving a light-brown reticulated hyperpigmentation. In conclusion, this was a case of exfoliative vesiculobullous prurigo pigmentosa in an adolescent man successfully treated with doxycycline and topical tacrolimus as an effective and safe treatment option.

Use of minocycline for the treatment of prurigo pigmentosa with intraepidermal vesiculation: a case report.

Pruritus pigmentosa is a skin disease mainly characterized by pruritus, inflammatory rash and reticular and macular pigmentation. The disease more commonly affects young women and may persist for several years. In this article, we report a case of a 20-year-old female patient who presented with erythema and blisters on the neck and trunk with pruritus for 20 days. Dermatological examination revealed a reticular distribution of erythema on the chest and abdomen and some areas of erythema covered with crusts. Additionally, blisters and bullae with clear fluid and negative Nikolsky's sign were noted. On the neck and back, erythema was also in a reticular distribution, and erythema secondary to erosion and/or crusts was present. In addition, histopathological analysis of the lesions showed hyperkeratosis and intraepidermal multilocular vesiculation and confirmed increased migration of inflammatory cells into the epidermis and infiltration of inflammatory cells, including lymphocytes, histiocytes and eosinophils, in the superficial dermis. The expression levels of IgG, IgM, IgA and C3 were all negative. This patient was diagnosed with prurigo pigmentosa, and the condition improved after treatment with minocycline.