Abstract: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. methylprednisol… Show more
“…Itching and dysesthesia are commonly reported, but some patients remain asymptomatic. Atypical presentations mimicking pityriasis rubra pilaris, acquired ichthyosis, and psoriasis vulgaris-like eruption have been previously reported in the literature [ 44 – 46 ]. Fig.…”
Section: Clinical Manifestationsmentioning
confidence: 99%
“…Skin histology reveals interface dermatitis, vacuolar degeneration of the basal layers, dyskeratosis, and superficial infiltrate, which are characteristic of, but not specific to, acute GVHD. The combination of histopathological findings of GVHD and other entities can be a clue for diagnosis in atypical presentations mimicking well-known dermatological disorders [ 44 – 46 ]. Histopathological grading was defined by Lerner et al in 1974 and is currently used (Table 2 ) [ 55 ].…”
Graft-versus-host disease (GVHD) is an immunological reaction and a frequent complication following allogeneic hematopoietic stem cell transplantation. It is associated with high mortality rates and may have a significant negative impact on the patient’s quality of life, particularly in the chronic-stage setting. Many different organs can be involved, which leads to a wide range of clinical manifestations. In this context, dermatologists play a key role by diagnosing and treating GVHD from the outset since cutaneous features are not just the most common but are also usually the presenting sign. Several skin-direct therapies are available and may be indicated as monotherapy or adjuvant treatment in order to allow faster tapering and withdrawal of systemic immunosuppression. Treatment of steroid-refractory patients remains a challenge and, to date, no consensus has been reached for one single agent in second-line therapy. This article aims to review skin involvement as well as provide and update discussion on therapeutic options for both acute and chronic cutaneous GVHD.
“…Itching and dysesthesia are commonly reported, but some patients remain asymptomatic. Atypical presentations mimicking pityriasis rubra pilaris, acquired ichthyosis, and psoriasis vulgaris-like eruption have been previously reported in the literature [ 44 – 46 ]. Fig.…”
Section: Clinical Manifestationsmentioning
confidence: 99%
“…Skin histology reveals interface dermatitis, vacuolar degeneration of the basal layers, dyskeratosis, and superficial infiltrate, which are characteristic of, but not specific to, acute GVHD. The combination of histopathological findings of GVHD and other entities can be a clue for diagnosis in atypical presentations mimicking well-known dermatological disorders [ 44 – 46 ]. Histopathological grading was defined by Lerner et al in 1974 and is currently used (Table 2 ) [ 55 ].…”
Graft-versus-host disease (GVHD) is an immunological reaction and a frequent complication following allogeneic hematopoietic stem cell transplantation. It is associated with high mortality rates and may have a significant negative impact on the patient’s quality of life, particularly in the chronic-stage setting. Many different organs can be involved, which leads to a wide range of clinical manifestations. In this context, dermatologists play a key role by diagnosing and treating GVHD from the outset since cutaneous features are not just the most common but are also usually the presenting sign. Several skin-direct therapies are available and may be indicated as monotherapy or adjuvant treatment in order to allow faster tapering and withdrawal of systemic immunosuppression. Treatment of steroid-refractory patients remains a challenge and, to date, no consensus has been reached for one single agent in second-line therapy. This article aims to review skin involvement as well as provide and update discussion on therapeutic options for both acute and chronic cutaneous GVHD.
“…Histopathologic findings include alternating orthokeratosis and parakeratosis in horizontal and vertical directions, hypergranulosis, and acantholysis . Acute PRP‐like GVHD at day 40 has been reported once in the literature, but to our knowledge, chronic PRP‐like GVHD has not been reported …”
The graft-versus-host disease (GvHD) is frequent complication, it occurs in 50% of patients after allogeneic hematopoietic stem cell transplantation (HSCT) and it is one of the major causes of mortality not associated with disease recurrence. Skin lesion in the symptom complex of acute GvHD develops within the first 100 days after HSCT, and it is complicated diagnostic and therapeutic problem. Significant immunosuppressive status of children during the posttransplant period enhances and changes the course of dermatoses, infections, drug toxicity. Finally, it can lead to immunoallergic processes with possible development to generalized life-threatening diseases of the skin and mucous membranes. Meanwhile, toxic, allergic and infectious skin lesions can be present simultaneously or develop sequentially. The description of the clinical picture of skin lesions in acute GvHD is really crucial and has scientific and practical significance due to relatively small frequency of HSCT in children with oncology diseases. The article summarizes data on etiology, pathogenesis, clinical forms, diagnostic and treatment methods of cutaneous complications of the early post-transplantation period after HSCT.
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