|Year : 2019 | Volume
| Issue : 2 | Page : 157-159
Pyoderma gangrenosum in childhood acute lymphoblastic leukemia
Ajeitha Loganathan1, Arathi Srinivasan1, Julius Xavier Scott1, Ramkumar Ramamoorthy2
1 Department of Pediatric Oncology, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
2 Department of Pediatric Dermatology, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||29-Mar-2019|
Dr. Arathi Srinivasan
Department of Pediatric Oncology, Kanchi Kamakoti Childs Trust Hospital, 12 A, Nageswara Rao Road, Nungambakkam, Chennai - 600 034, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which occurs either idiopathically or associated with various systemic diseases and malignancies. Although the association of PG with myeloid malignancies is well known, its association with lymphoid malignancy especially in children is extremely rare. We describe PG in association with acute lymphoblastic leukemia in a 4-year-old child, an occurrence which has been reported just once previously to the best of our knowledge.
Keywords: Leukemia, neutrophilic dermatosis, pyoderma gangrenosum
|How to cite this article:|
Loganathan A, Srinivasan A, Scott JX, Ramamoorthy R. Pyoderma gangrenosum in childhood acute lymphoblastic leukemia. Indian J Paediatr Dermatol 2019;20:157-9
|How to cite this URL:|
Loganathan A, Srinivasan A, Scott JX, Ramamoorthy R. Pyoderma gangrenosum in childhood acute lymphoblastic leukemia. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Dec 14];20:157-9. Available from: http://www.ijpd.in/text.asp?2019/20/2/157/255209
| Introduction|| |
Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which may be associated with systemic diseases such as ulcerative colitis, polyarthritis, diabetes mellitus, myelodysplastic syndrome, and myeloid leukemia. Less information is available about the association of PG with lymphoid malignancies. Here, we report a case of childhood precursor B-cell acute lymphoblastic leukemia (ALL) associated with PG who failed to respond to broad-spectrum antibiotics but responded to steroid based chemotherapy.
| Case Report|| |
A four year old female child presented with intermittent fever, progressive pallor, lethargy, significant weight loss of one month duration associated with ulcerated skin lesion in the lower lip of ten days' duration. The lesion had initially started as a small erythematous lesion which progressed to a necrotic ulcer. She had been treated with oral antibiotics for the skin lesion with no improvement. On physical examination, she had marked pallor with hepatosplenomegaly. She had no bleeding manifestations or lymphadenopathy. Cutaneous examination revealed a necrotic ulcer in the lower lip with dusky, edematous overhanging borders and a surrounding margin of erythema [Figure 1]a. Underneath the necrotic debris was a deep ulcer with a clean base and violaceous borders [Figure 1]b. She had a hemoglobin of 3.4 g/dl, white blood cell count of 800/mm3, and platelet count of 24,000/mm3. Dermatology consultation was obtained for the lip ulcer, and a probability of PG was considered. She was started on broad-spectrum antimicrobials in view of febrile neutropenia. Bone marrow aspiration done in view of pancytopenia was suggestive of ALL and flow-cytometry revealed expression of CD10, CD19, CD22, thus confirming the diagnosis of acute lymphoblastic leukemia associated-positive B-cell ALL. Cultures from the swab of ulcer showed normal skin microbial flora. She was started on prednisone based induction chemotherapy. The skin lesions that had earlier failed to respond to broad-spectrum antibiotic (piperacillin-tazobactam with amikacin) showed signs of improvement in Four days after chemotherapy. In view of the classical morphology of the lesion and the patients' failure to respond to antibiotics, but the complete response to steroid-based chemotherapy, the diagnosis of PG was made after dermatological consultation. She was in complete bone marrow remission at the end of the induction chemotherapy. Her ulcers healed completely with minimal scarring [Figure 2].
|Figure 1: (a) A necrotic ulcer in the lower lip with dusky, edematous overhanging borders with a surrounding margin of erythema. (b) A deep ulcer with clean base and violaceous borders underneath the necrotic debris|
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| Discussion|| |
PG is a rare skin lesion which occurs in all age groups; approximately 4% of patients are infants and children. There are five clinical variants of PG: ulcerative/classical, bullous, pustular, peristomal, and vegetative. The most common is the ulcerative variety which begins as small discrete pustules or fluctuant nodules. These lesions enlarge, and the overlying skin begins to necrose. Typical ulcerative PG lesion has multiple deep necrotic ulcers with violaceous, ragged, undermined edges. Our patient had this typical ulcerative PG.
PG of the head and face appears to be common in children, as in our patient., In children, it usually occurs in association with inflammatory bowel disease, immunodeficiency, immunosuppression, and HIV infection. In a case series of 46 children and infants with PG, ulcerative colitis was the most common underlying disease, seen in 74% of patients.
Maldonado et al. first reported the occurrence of PG with leukemia. Leukemia is the most frequently associated malignancy, with acute myeloid leukemia being the most common form.,,, PG in lymphoid leukemia is rare. Our patient had this rare association. To the best of our knowledge, this is the only second report of PG associated with pediatric ALL.
Diagnosis mainly depends on recognition of the evolving clinical features and is only supported by histopathology. Massive neutrophilic infiltration in the absence of vasculitis and granuloma formation is typical of PG. Although PG is a neutrophilic skin lesion, it can occur in neutropenic conditions such as autoimmune neutropenia and leukocyte adhesion defect type 1. A clinical criterion has been proposed for PG. Our patient fulfills these criteria.
No specific therapy is universally effective for patients with PG. Effective control of the underlying disease may be linked to a control of the cutaneous process as well. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective. Our child responded to oral prednisolone which formed a part of her treatment of ALL. Other agents used are immunosuppressive agents such as cyclosporine, tumor necrosis factor-alpha blockers such as infliximab, adalimumab, and biologics such as efalizumab and alefacept. We report our child having PG associated with ALL to make pediatricians and dermatologists aware of the association to avoid unnecessary tests and biopsy.
Informed consent from the parents obtained for publishing the photographs of the child.
Declaration of patient consent
These authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]