Immunosuppression-induced porokeratosis of Mibelli: complete regression of lesions upon cessation of immunosuppressive therapy

Porokeratosis developing subsequent to immunosuppressive therapy is a common and well recognized phenomenon. A previously reported case of porokeratosis showed complete remission of the lesions following discontinuation of immunosuppression. A second example of porokeratosis is presented, in which lesions appeared 2 years after bone marrow transplantation, and completely regressed after immunosuppressive therapy was stopped.     

Atypical Porokeratosis Developing Following Bone Marrow Transplantation in a Patient with Myelodysplastic Syndrome

Porokeratosis is an abnormal disease of keratinization of epidermis. It is clinically characterized by margins covered with keratin layer and it typically has an atrophied macule with a protruded, circular form. Histopathologically, it shows the findings of cornoid lamella. Risk factors for its development include organ transplantation, long-term use of corticosteroids, immunocompromised status, including AIDS, and exposure to ultraviolet light. We herein report a case of atypical porokeratosis in a 38-year-old man who developed porokeratosis involving multiple sites following bone marrow transplantation for myelodysplastic syndrome.     

Imiquimod and Photodynamic Therapy Are Useful in the Treatment of Porokeratosis in Children with Bone Marrow Transplantation

Porokeratosis is an uncommon disorder that affects keratinization. Immunosuppression may favor the development of porokeratotic lesions. Patients who receive allogenic transplants represent a therapeutic challenge to dermatologists. We report two cases of porokeratosis in children with bone marrow transplant and their excellent response to imiquimod and photodynamic therapy.     

Facial and bilateral acral porokeratosis with nail dystrophy: A case report

Porokeratosis is a rare disorder of keratinization with unknown etiology. It exhibits both sporadic and autosomal dominant inheritance. The lesions are sharply demarcated, hyperkeratotic, and annular with distinct keratotic edges. The cornoid lamella is the histological hallmark of porokeratosis. Porokeratosis lesions on the face may have a superficial or a destructive nature. To our knowledge there are only a few cases of facial porokeratosis that have been reported. We report a sporadic form of facial and bilateral acral porokeratosis with nail dystrophy.     

Eruptive angiokeratomas and porokeratosis in the setting of sclerodermatous graft‐vs.‐host disease

Porokeratosis and angiokeratomas are both seen in the setting of chronic graft‐vs.‐host disease (GVHD), but rarely occur together. We present a case of a patient with lichen planus‐like (LPL) and sclerodermatous chronic GVHD manifesting after allogeneic bone marrow transplant with concomitant eruptive angiokeratomas and porokeratosis.     

Disseminated superficial porokeratosis in a patient with AIDS

We report the case of a 50-year-old male homosexual suffering from AIDS, who developed diffuse annular hyperkeratotic lesions on the arms and legs. Histopathological examination revealed typical features of porokeratosis, which clinically was of the disseminated superficial type. Ultrastructural examination showed a paucity of keratohyalin granules and lamellar bodies. Immunohistochemical studies showed an almost complete absence of Langerhans cells in lesional epidermis. Involucrin and filaggrin expression were altered in areas of cornoid lamella formation, whereas basal keratinocytes in these areas expressed PCNA/cyclin and, to a lesser degree, p53 protein. Porokeratosis may affect immunocompetent patients, but has also been reported in the setting of immunosuppression following organ transplantation. As far as we are aware, the development of porokeratosis during the course of HIV infection has not been reported previously.     

POROKERATOSIS IN IMMUNOSUPPRESSED AND NONIMMUNOSUPPRESSED PATIENTS

Porokeratosis is an uncommon, inherited, autosomally dominant disorder. In the last decade association of porokeratosis and immunosuppression has been observed. In this study we carried out a comparative study between immunosuppressed and nonimmunosuppressed porokeratosis cases. We found that 9 out of 20 cases of porokeratosis were associated with organ transplantation/immunosuppression. Clinicopathologic study revealed that the pattern of disease is alike both in immunosuppressed and nonimmunosuppressed patients. Our observations indicate that immune modulation could be a factor in the genesis of porokeratosis.     

Facial porokeratosis characterized by destructive lesions

Porokeratosis is a rare group of keratinizing diseases. It is inherited as an autosomal dominant disease with variable penetrance, although sporadic cases are often reported. Porokeratosis has as its histological hallmark the typical cornoid lamella. Porokeratosis lesions localized on the face can vary from superficial to destructive in nature. Only 12 cases have been reported in the literature to date. We report one more unusual case of destructive facial porokeratosis.     

Mibelli's porokeratosis after bone marrow transplantation]

A 24-year old male patient developed, on both legs, lesions typical of Mibelli's porokeratosis 22 months after bone marrow transplantation, under treatment with cyclosporin A. He denied any family history. Mibelli's porokeratosis seldom develops after an immunosuppressive treatment, and to our knowledge it has exceptionally been described after bone marrow transplantation. A possible complication of Mibelli's porokeratosis is the development of Bowen's disease, basal or squamous cell carcinomas. Immunosuppressive treatment might facilitate the degeneration. For this reason, these subjects should be periodically and carefully examined.     

Porokeratosis in Singapore: an Asian perspective

Porokeratosis is a rare disorder of skin keratinisation characterised by a cornoid lamella. We reviewed its associations with immunosuppression and phototherapy, as well as the risks of malignant progression. This is a retrospective review on all cases of porokeratosis seen at the National Skin Centre, Singapore, between 2000 and 2010. A total of 94 patients were reviewed. Clinical and histological diagnoses were confirmed in 63% patients. Most patients were Chinese (89%) with a mean age of 51.6 years. The male to female ratio was 1.4:1. The four main clinical variants were classical porokeratosis of Mibelli (56%), disseminated superficial actinic porokeratosis (DSAP) (18%), disseminated superficial porokeratosis (DSP) (11%), and linear porokeratosis (13%). Phototherapy-induced porokeratosis, seen in three patients, is rare. Seven cases of porokeratosis occurred in patients who were immunosuppressed. Progression of porokeratosis to malignancy is uncommon and was observed in three patients. The most common treatments included cryotherapy (26.5%) as well as topical steroids or retinoids (38.1%). A good response, defined as clear or almost clear lesions, occurred in 16% patients. The most common presentation of porokeratosis in our review was a middle-aged male patient with an asymptomatic lesion of porokeratosis of Mibelli over the extremities. No particular immunosuppressive drug was implicated. Porokeratosis associated with ultraviolet phototherapy or malignancy is rare. Progression of porokeratosis to malignancy arose in the disseminated variants, with a possible correlation with age. This is the largest institutional retrospective review of porokeratosis to date and highlights the major epidemiological characteristics of this condition.     

Case for diagnosis

Porokeratosis is a primary keratinizing disorder of unknown etiology. This disorder is characterized by the presence of centrifugally enlarging hyperkeratotic plaques, associated with the histopathological hallmark of cornoid lamellae. Genital porokeratosis is extremely rare. No more than thirty cases have been reported in the literature, including only one case of linear porokeratosis confined to the genital area. This case report describes a patient with genital linear porokeratosis, who was successfully treated with cryotherapy. Over two years of follow-up, the lesion improved and there was no evidence of recurrence or signs of malignant transformation. Nevertheless, there is a need for long-term follow-up data on recurrence and malignant transformation.     

Efficacy of Acitretin for Porokeratosis in a Child with Chronic Cutaneous Graft Versus Host Disease

Abstract: Porokeratosis is a rare disorder of epidermal keratinization that is regarded as a precancerous. Recipients of hematopoietic stem cell transplantation (HSCT) have a greater risk of skin cancer; chronic graft versus host disease (GVHD) is an additional risk factor. A 16‐year‐old boy who had received HSCT for acute myelogenous leukemia was referred to us for sclerodermoid chronic cutaneous GVHD. Two years later, he developed disseminated porokeratosis with a few atypical lesions. Despite cryotherapy, numerous lesions of porokeratosis recurred rapidly. Acitretin resulted in good clinical response and reduced the rate of onset of new lesions.     

Disseminated giant porokeratosis and porokeratosis of Mibelli in Bankura and Bardhaman districts,West Bengal,India

Porokeratosis of Mibelli (PM) is a rare genodermatosis. It is caused by proliferation of abnormal clones of epidermal cells in response to several stimuli, the most important of which is sunlight. Giant porokeratosis is thought to be a variant of PM. We report two cases of disseminated PM and one case of disseminated giant porokeratosis from the Bankura and Bardhaman districts of West Bengal in India; presenting to a single observer in the summer of 2012. Interestingly we have noted that the majority of cases of porokeratosis in India have been reported from West Bengal. Few patients with a single lesion of giant porokeratosis have been reported in world literature. As far as we know, this is the first case report of disseminated giant porokeratosis in world literature.     

Fatal squamous cell carcinoma arising from transplant-associated porokeratosis

Porokeratosis is a disorder of epidermal keratinization characterized by variably sized plaques with central depression and a well-demarcated keratotic border. Associations of porokeratosis with immunosuppression and of porokeratosis with malignancy have been observed. The authors report a case of fatal metastatic squamous cell carcinoma arising from porokeratosis in an immunosuppressed patient.     

Giant porokeratosis

Porokeratosis is a disorder of keratinization characterized by annular plaques with an atrophic center surrounded by a raised, keratotic wall. It has several clinical forms including a porokeratosis of Mibelli, giant porokeratosis, linear porokeratosis, disseminated superficial actinic porokeratosis, palmoplantar porokeratosis and punctate porokeratosis. We report a patient with the rare condition of giant porokeratosis. Several variants of porokeratosis coexist in our patient.     

Focal porokeratosis of nuchae: case report

Porokeratosis is the common name of several diseases of unknown pathogenesis, which are similar in clinical appearance. It was first described in 1893 and was thought to be a disorder of sweat glands, hence the name porokeratosis. The lesion that can be found in all cases is annular, with atrophic center, and hyperkeratotic outer ring. Cases of porokeratosis have been linked with genetic heritage, excessive ultraviolet exposure, kidney failure, and state of immunosuppression, but no definitive link has been established. As a rare condition, its main feature is that the correct diagnosis can be delayed for a significant period of time. Porokeratotic lesions have a high incidence of malignant transformation and are considered premalignant. We present a case where a porokeratotic lesion was unsuccessfully treated as a psoriatic lesion for more than a year in a patient with previously diagnosed psoriasis. A skin biopsy was performed at our department, which revealed classic cornoid lamella and thus led to the correct diagnosis. The lesion was excised. Additional diagnostic tests revealed normal kidney function and intact immune system. A follow-up protocol was established for the patient, ensuring timely diagnosis of any future porokeratotic lesions. Porokeratosis, especially when there are only few lesions, is not difficult to manage - once it is diagnosed. A diagnosis of porokeratosis may also aid in identifying a serious systemic disease such as kidney failure. Importantly, a misdiagnosis of porokeratosis may lead to development of skin cancer.     

Linear Porokeratosis Presenting as Erosions in the Newborn Period

Abstract: The classic appearance of porokeratosis is characterized by a hyperkeratotic annular rim that expands peripherally, leaving an atrophic center. Linear porokeratosis is a variant with collections of such lesions arranged in a linear fashion, usually corresponding to a dermatome or Blaschko's lines. Ulcerations have rarely been reported in patients with porokeratosis. We report an unusual case of linear porokeratosis at birth, with erosions and Ulcerations of the face and lower extremity, that eluded diagnosis for nearly a year. Porokeratosis should be considered in the differential diagnosis of erosions in the newborn period.     

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft‐versus‐Host Disease

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides–associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft‐versus‐host disease after an allogeneic bone marrow transplantation.     

Multicentric squamous cell carcinoma over lesions of porokeratosis palmaris et plantaris disseminata and giant porokeratosis

Porokeratosis is a specific disorder of keratinization that has five clinical types and shows a characteristic 'cornoid lamella' on histopathology. Malignant degeneration has been described in all forms of porokeratosis. To the best of our knowledge, this is the first Indian report of multicentric squamous cell carcinoma complicating porokeratosis.