Paraneoplastic pityriasis rubra pilaris: case report and literature review

Pityriasis rubra pilaris (PRP; MIM 173200) is an uncommon papulosquamous inflammatory dermatosis. Only a few cases of PRP associated with an underlying malignancy have been documented. We investigated a 59‐year‐old patient presenting with a fulminant form of PRP recalcitrant to systemic retinoid therapy, in whom the skin disease heralded a diagnosis of cholangiocarcinoma. We searched the MEDLINE database to find articles reporting on similar associations of PRP with malignancies. We identified 10 studies linking PRP and malignancies, but an association between PRP and cholangiocarcinoma has not yet been reported.     

Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up

Pityriasis rubra pilaris (PRP) is an idiopathic papulosquamous disease that clinically presents with palmoplantar keratoderma and follicular hyperkeratotic papules that coalesce into scaly erythematous plaques. We report a unique case of atypical PRP beginning at 1 year of age with associated severe arthropathy and osteoporosis. We further discuss the clinical and histopathologic aspects of PRP, its possible etiology, and other associated conditions.     

Pityriasis rubra pilaris in association with laryngeal carcinoma

Pityriasis rubra pilaris (PRP) is a rare group of hyperkeratotic, papulosquamous diseases that can be acquired or inherited. Cases of PRP associated with malignancy have been rarely reported. We report a case of 46‐year‐old man who presented with rapidly progressing PRP as a possible initial cutaneous symptom of a previously undiagnosed laryngeal carcinoma. Microlaryngoscopy was performed because of the patient’s hoarseness, and this revealed leucoplakia on the left vocal cord. Histopathological examination led to the diagnosis of squamous cell carcinoma in situ. After surgical treatment, the clinical signs of PRP began to resolve, and the patient was free of skin lesions at follow‐up. This case represents a rare coexistence of PRP with malignancy, and indicates that PRP can occur as paraneoplastic dermatosis associated with laryngeal cancer.     

Pityriasis rubra pilaris as the initial manifestation of internal neoplasia

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder of unknown aetiology. There are only three cases reported occurring coincident with internal neoplasia We report a case of this disorder presenting as the initial manifestation of a previously undiagnosed malignancy with metastases in the liver. The primary tumour was not detected. The patient had a complete remission of his PRP with topical steroids alone, but died of the malignancy.     

Erythema gyratum repens associated with pityriasis rubra pilaris

Erythema gyratum repens (EGR) is a rare cutaneous eruption characterized by serpiginous morphology and a migrating scaly border. It is one of the most specific cutaneous paraneoplastic phenomena, and is associated with malignancy in most cases. We report a 46‐year‐old Afro‐Caribbean man with the unequivocal clinical and histological features of pityriasis rubra pilaris (PRP). However, despite improvement on oral acitretin, the morphology of the eruption evolved into the striking serpiginous rash of EGR. The histology findings, although nonspecific, were in keeping with the diagnosis of EGR. No evidence of malignancy was found. Only four cases of PRP evolving into EGR have been reported in the literature, and none was associated with malignancy. All previously reported cases of EGR have been described in white patients, making our case the first reported exception, to our knowledge. The possible role of retinoids in altering the rash of PRP to that of EGR is discussed.     

Adult onset pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) has always been an intriguing topic ever since its inception. It is a group of chronic disorders characterized by reddish orange plaques with pityriasiform scaling showing follicular keratoses, palmoplantar keratoderma, and sometimes, erythroderma. It occurs all over the world but with racial variations. Its incidence might vary and the age at onset, behavior, clinical appearance, and prognosis are considered to be very important for its classification. It may manifest either as Type I classical adult onset PRP, Type II atypical adult (onset) PRP, or Type VI PRP (HIV-associated PRP pityriasis rubra pilaris) in contrast to classical juvenile (Type III) and circumscribed juvenile (Type IV) encountered among children. Its diagnosis is largely clinical with microscopic pathology being a useful supplement, but it continues to be a therapeutic dilemma. We review the epidemiology of adult onset PRP here and take stock of the prevalent treatment options.     

Atypical dermatitis herpetiformis in two patients with internal malignancy

Two patients with bullous dermatoses indistinguishable from dermatitis herpetiformis clinically, but with atypical immunological and histological features are reported. Both died within a few months of the initial dermatologic presentation of disseminated malignancy involving the bronchus in one case and uterine endometnum in the other. The significance of these observations and the possible association of bullous disease and malignancy is discussed.     

Refractory pityriasis rubra pilaris treated with etanercept,adalimumab, or ustekinumab: A retrospective investigation

Pityriasis rubra pilaris (PRP) is a rare, difficult to treat papulosquamous disorder that responds variably to retinoids and immunosuppression. Successful use of biologics for treating PRP has been described in the literature by case reports and a limited number of case series. To provide additional data, we retrospectively analyzed cases of PRP treated with biologics at our institution. We identified seven patients with a clear diagnosis of PRP treated with adalimumab, etanercept, and/or ustekinumab at our institution from January 1, 2014 to April 1, 2017. Six of seven patients had type I, adult acquired PRP, and one had type V atypical juvenile PRP. In response to tumor necrosis factor (TNF)‐α inhibition, two patients had marked responses (>75% improvement in involved body surface area), while three patients failed to show any improvement on a TNF‐α inhibitor. In two cases of PRP refractory to TNF‐α inhibition, ustekinumab resulted in a partial response (<75% improvement) in one patient and no response in the other. Compared to other published data, our cohort was substantially more resistant to treatment with biologics, a finding which may provide valuable perspective for dermatologists managing refractory PRP in the future.     

Extracorporeal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare papulosquamous skin disease of unknown aetiology that has been categorized into five clinical types based on age at onset, cutaneous features and prognosis. We present a patient with chronic exanthematic type II atypical adult PRP, whose skin status was significantly improved with monthly extracorporeal photochemotherapy (ECP). Various therapeutic regimens including narrow-band UV-B, bath PUVA therapy, systemic fumaric acid esters and systemic cyclosporin had failed. Oral retinoids could not be administered due to a type IIa hyperlipoproteinemia with profound hepatic steatosis and elevated liver transaminases. The observed clinical benefit may encourage future clinical studies analysing the effectiveness of ECP in otherwise unresponsive cases of type II PRP.     

Sweet's syndrome with bullous lesions

We present a case of Sweet's syndrome with atypical lesions, characterized by erythematous plaques, vesicles and bullous lesions. Skin lesions in patients with an underlying malignancy are more frequently atypical and with vesicular, bullous or even ulcerative characteristics, in addition to the typical plaques and nodules. However, the case presented is not associated with malignancy, despite the fact that these processes, particularly hematologic ones, should be suspected.     

Herpetiform Sweet's syndrome and leukaemia

A patient who developed atypical vesicular Sweet's syndrome (SS) resembling herpes simplex virus infection, during relapse of acute myeloid leukaemia is presented. The morphologic and histologic spectrum of SS is discussed, alerting dermatologists to atypical varients. The association with malignancy, particularly of myeloprliferative origin, is highlighted, as are features of SS that suggest the presence of an underlying malignancy.     

Platelet‐rich plasma as a novel treatment for lichen planopillaris

Treatment of lichen planopillaris (LPP) remains a significant challenge due to the irreversible damage inflicted on hair follicles combined with the low efficacy of existing treatments. We hypothesized that growth factors released by the use of platelet‐rich plasma (PRP) may arrest the development of LPP. To test our hypothesis, we treated an LPP patient that has failed previous treatments with a new PRP regimen. Following PRP treatment and six months follow‐up, the patient experienced complete regression of itching and hair shedding. To the best of our knowledge, this is the first report of successful treatment of LPP with a PRP regimen.     

Serum sickness disease in a patient with alopecia areata and Meniere’ disease after PRP procedure

Background: Platelet rich plasma procedure (PRP) is considered to be one of the safest aesthetic procedures. Adverse reactions after PRP administration are extreme rare. Purpose: We present the patient with serum sickness disease (SSD) after PRP procedure. Objective and methods: 41 years old female suffers from alopecia areata for 5 years with frequent relapses and she has been suffering from Menier's disease recurrent symptoms for 6 years. The patient developed SSD after third PRP rejuvenating procedure and she has also noticed new alopecia areata lesions, but without Menier's disease symptoms. After SSD, 4 months later, she developed severe symptoms of Menier's disease with an episode of sudden sensorineural hearing loss. It alleviated only after intravenous administration of methylprednisolone. In our opinion, significant contraindication of PRP procedure is an autoimmune disease in the active phase.     

Acute cutaneous graft‐versus‐host disease resembling type II (atypical adult) pityriasis rubra pilaris

We present a case of cutaneous acute graft‐versus‐host disease (aGVHD) with confluent erythematous perifollicular hyperkeratosis and ichthyosiform scale in the clinical pattern of type II (atypical adult) pityriasis rubra pilaris (PRP), which developed 26 days after allogeneic peripheral blood stem cell transplant. Skin histology confirmed features of both aGVHD and PRP. The skin lesions were refractory to oral prednisolone and cyclosporine and only partially responsive to a combination of i.v. methylprednisolone, oral tacrolimus, oral mycophenolate mofetil, and infusions of anti‐thymocyte globulin and the tumour necrosis factor‐α inhibitor, etanercept.     

HIV-associated pityriasis rubra pilaris responsive to triple antiretroviral therapy

HIV-associated pityriasis rubra pilaris (PRP) or PRP type VI designates a new distinctive entity reported in HIV patients. It is characterized by cutaneous lesions of PRP and variable association with lesions of acne conglobata, hidradenitis suppurativa and lichen spinulosus. We report a patient with HIV-associated PRP which was treated by triple antiretroviral therapy (zidovudine, lamivudin and saquinavir) with complete response. The patient has remained free from symptoms for 20 months of follow-up. We review the clinical features, pathology, evolution, treatment and possible aetiology of this recently described entity.     

The effect of platelet rich plasma on hair regrowth in patients with alopecia areata totalis: A clinical pilot study

Autologous rich plasma (PRP) is blood plasma with enhanced concentration of platelets and is enriched with several growth factors which stimulate tissue regeneration. The current study aimed to investigate the effect of PRP on hair regrowth in patients with alopecia areata (AA) totalis. Ten subjects (28.9 ± 6.28 years; five males and five females) with clinically diagnosed AA totalis for at least 3 years who had not received any treatment within 3 months prior to the study were recruited. Blood sample was collected in thrombocyte harvesting tubes. The PRP was separated via centrifugation. The patients' scalp was divided sagittally into two approximately equal parts. In each patient, 4 mL of PRP was injected intradermally into the left or right side of the scalp; in each point, 0.1 mL of PRP was injected. Each patient was followed up monthly for 4 months. No hair regrowth was seen in eight patients and in two patients only <10% hair regrowth was observed. Totally, no significant effect was found for PRP on hair regrowth (p > .05). There was no side effect during treatment. Single dermal PRP injection did not prove to have any effect on hair regrowth in these patients.     

Atypical pyoderma gangrenosum as a manifestation of childhood acute lymphoblastic leukemia

Pyoderma gangrenosum is a neutrophilic dermatosis that may occur idiopathically or in association with various systemic diseases and malignancy. Although the association of this entity with myeloid malignancies is well known, its association with lymphoid malignancy is extremely rare. We describe atypical pyoderma gangrenosum in association with acute lymphoblastic leukemia in a 2-year-old child, an occurrence not reported before.     

Atypical cellular neurothekeoma: A potential diagnostic pitfall for benign and malignant spindle cell lesions in skin

Cellular neurothekeomas (CNTs) are rare, benign cutaneous tumors that arise primarily on the head and neck, with a slight female predominance. CNTs with atypical features have been described, including those with an infiltrative growth pattern. Although CNTs with atypical features are benign, recognition of this entity can pose diagnostic challenges. Here, we report a case of CNT with an unusual clinical presentation on the left second digit, and with atypical histological features including an infiltrative growth pattern, which could have been mistaken for features of malignancy.     

Intralesional injection of platelet-rich plasma versus steroid in the treatment of oral lichen planus

Background

Oral licen planus (OLP) is a chronic inflammatory disease and may have immunological background. Both intralesional injection of PRP and steroids succeeded in treating and decreasing recurrence of the disease.

Patients and Methods

Twenty-four participants with clinically diagnosed as OLP were enrolled in this study. We separated the patients in 2 groups, 12 patients in group A were treated by intralesional PRP every two weeks for 2 months or stopped if healing occurred earlier. Group B (12 patients) treated by intralesional Triamcinolone Acetonide (TA) (20 mg) every two weeks for 2 months or may be less if healing occurred earlier. The response of OLP lesions to treatment was evaluated by reduction of lesional areas, REU scores, and NRS scores. The patients with complete response (CR; 80%–100% reduction in the lesion area) were followed for 3 months biweekly.

Results

There was a statistically significant decrease in REU and pain score in both groups after treatment compared to before. There was a statistically increase in frequency of side effects among patients received PRP especially pain compared to those treated by steroid. Also, recurrence of the disease after treatment during follow-up for 3 months was more significant among patients treated by PRP.

Conclusion

Intralesional PRP is a good and safe modality for treatment of OLP and intralesional TA. However, there were some side effects and recurrence of disease after follow-up for three months in patients treated by PRP more than those treated by TA.     

Unresponsive eczematous dermatitis: a case of pancreatic cancer masquerading as cutaneous T cell lymphoma.

Skin diseases may be the presenting sign of malignancy, but strict criteria are required to make the diagnosis of a paraneoplastic syndrome. Common dermatoses may also herald an underlying malignancy without meeting these criteria. We report the case of an elderly man with an unresponsive eczematous dermatitis whose evaluation eventually revealed a pancreatic carcinoma. This case is presented to alert the clinician that a common dermatosis may be the first sign of an occult malignancy, especially if it behaves in an atypical or aggressive manner or is recalcitrant to standard therapies.