Camilla Vassallo

Muco-cutaneous changes during long-term therapy with hydroxyurea in chronic myeloid leukaemia

Hydroxyurea is an antimetabolite agent used in the treatment of myeloproliferative disorders and sickle cell anaemia. Although hydroxyurea is relatively well tolerated, adverse effects often involve skin and mucous membrane during long‐term therapy. A group of 510 patients affected by chronic myeloid leukaemia from 1977 to 1998 has been considered. Only 158 patients were treated with hydroxyurea and fulfilled inclusion/exclusion criteria of this study. A spectrum of severe cutaneous and mucosal changes (inflammatory and neoplastic) was seen in about 13% of patients (21 patients out of 158) and was studied in detail. Cutaneous and mucosal atrophy were observed in all 21 patients. Skin atrophy was often characterized by numerous telangiectases, especially on legs and on sun‐exposed sites (16/21). Cutaneous, mucosal and nail hyperpigmentation was evident, albeit with variable extent, in 10 of the 21 patients. Severe stomatitis and glossitis with flattening of papillae were another common finding. Five patients, who received a particularly long treatment with hydroxyurea, developed squamous‐cell neoplasms on sun‐exposed sites (both squamous‐cell carcinomas and keratoacanthomas). Acral changes were characteristic and constant, including acral erythema (21/21), dermatomyositis‐like changes on the dorsa of hands (7/21), ulcers localized on acral areas of legs, on genitalia and oral mucosae (20/21). The frequency and the variety of these muco‐cutaneous changes are reported and the mechanisms by which hydroxyurea may induce this muco‐cutaneous syndrome‐like group of changes, are proposed.

Radiation recall dermatitis, panniculitis, and myositis following cyclophosphamide therapy: histopathologic findings of a patient affected by multiple myeloma.

Radiation recall dermatitis is one of the skin sequelae that may affect oncology patients. It occurs in a previously irradiated field, when subsequent chemotherapy is given. The eruption may be elicited by chemotherapy, even several months after radiotherapy. Its mechanism is poorly understood, and the histopathologic findings have received, to date, only sketchy descriptions. A 55-year-old male affected by multiple myeloma received radiation therapy both on his left coxofemoral area, and lumbar region (D11-L1). After cyclophosphamide administration, he developed 2 well defined square-shaped, infiltrated erythematoviolaceous plaques in the prior irradiated fields. Histopathologic findings revealed a diffusely fibrosclerosing process, involving deep dermis, hypodermis, as well as the underlying muscle, while sparing the epidermis and superficial-mid dermis. Histopathology was indistinguishable from deep radio-dermatitis, panniculitis, and myositis. This is the first case providing clear evidence of the causative role of cyclophosphamide in inducing a cutaneous and subcutaneous radiation recall reaction.

Reactive angioendotheliomatosis in an infant

Reactive cutaneous angioendotheliomatosis (RCA) is an uncommon benign disease characterized by intravascular proliferation of endothelial cells. The observation of RCA in infants is exceedingly rare. We describe a case of RCA in a 3-month-old infant. The lesions were characterized by six small purpuric papules (1-2 mm in diameter), distributed on the thighs and neck. The general condition of the patient was good, with no lymphadenopathy, systemic involvement, or fever. The histopathologic features of a papule were characterized by the presence of cohesive aggregates of large mononucleated cells protruding into the lumina of dilated vessels and filling some of them completely. Neither an inflammatory infiltrate nor a proliferation of pericytes were present around blood vessels. Intravascular proliferating cells demonstrated positive staining for Ulex europaeus agglutinin 1 (UEA-1) and for Factor VIII-RA and CD34 antigens. The course of the disease was unremarkable with persistence of the lesions for 8 months; no treatment was started.

Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study

Background  Epidermolysis bullosa acquisita (EBA) is a rare autoimmune mucocutaneous bullous disease caused by autoantibodies against type VII collagen, a component of anchoring fibrils that stabilizes dermoepidermal adherence. Type VII collagen is composed of a collagenous domain linked by the noncollagenous (NC)1 and NC2 domains.

IgA anti‐epidermal transglutaminase autoantibodies: a sensible and sensitive marker for diagnosis of dermatitis herpetiformis in adult patients

Background  Dermatitis herpetiformis (DH) is a rare gluten‐sensitive blistering itchy skin disease, strictly related to coeliac disease (CD). Direct immunofluorescence, demonstrating IgA granular deposits localized either in the dermal papillae or along the dermo‐epidermal junction, is currently the gold standard for diagnosis of DH. It has been shown that DH immunocomplexes contain epidermal transglutaminase (eTG) and that sera from patients with DH contain antibodies specifically directed against eTG.

Isomorphic cutaneous graft‐versus‐host disease reaction after ultraviolet exposure: clinical, histological and direct immunofluorescence studies of four allo‐transplanted patients

Background  Acute and chronic graft‐versus‐host disease (GVHD) continues to be a major limitation to successful haematopoietic stem cell transplantation. If experimental studies and clinical observations could partially elucidate the pathophysiology of acute GVHD, the biology of chronic GVHD is still much less well understood.

Hair depigmentation and vitiligo-like lesions in a leukaemic paediatric patient during chemotherapy with dasatinib.

© 2012 The Authors. doi: 10.2340/00015555-1289 Journal Compilation

Wegener granulomatosis in a child: cutaneous findings as the presenting signs.

Abstract: Wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described without renal involvement. We report a 14‐year‐old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cutaneous lesions were associated with upper and lower respiratory tract involvement, low‐grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagnosis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infiltrate and a late‐stage small vessel vasculitis in the dermis. Histopathology of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patients clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an “acneiform” presentation is a rare finding in WG.

Photoinduced dermatitis and oral lichenoid reaction in a chronic myeloid leukemia patient treated with imatinib mesylate

Imatinib mesylate (IM) is a phenylaminopyrimidine that represents the first‐line treatment for chronic myeloid leukemia (CML), Philadelphia chromosome‐positive. It acts as a potent and selective inhibitor of the bcr‐abl fusion protein by a competitive inhibition at the adenosine triphosphate‐binding site of the enzyme, which leads to the inhibition of tyrosine phosphorylation of the proteins involved in bcr‐abl signal transduction. IM is generally well tolerated and usually provokes only mild side effects consisting of nausea, myalgia, edema and muscle cramps.

Keratoacanthoma in vitiligo lesion after UVB narrowband phototherapy

The treatment of vitiligo is still a challenge. Among various therapeutic modalities, phototherapy with UVB narrowband (UVB‐NB) is presently considered a treatment of choice for this skin disease.