Neutrophilic Panniculitis in a child with MYSM1 deficiency

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.     

Pancreatic Panniculitis Caused by l-Asparaginase Induced Acute Pancreatitis in a Child with Acute Lymphoblastic Leukemia

Abstract:   Panniculitis caused by l -asparaginase induced acute pancreatitis is an unusual condition. We report a case of a 7-year-old Thai boy with underlying acute lymphoblastic leukemia who developed pancreatic panniculitis caused by l -asparaginase administration. During the second week of induction chemotherapy, the patient developed acute pancreatitis with septic shock. Two weeks later he developed multiple subcutaneous nodules on the abdominal wall. Skin excisional biopsy showed the typical histopathological features of pancreatic panniculitis. Acute pancreatitis improved after 3 weeks of conservative treatment. The skin lesions resolved spontaneously within 1 month.     

Probable Griseofulvin‐Induced Drug Reaction with Eosinophilia and Systemic Symptoms in a Child

A 9‐year‐old boy presented with fever, rash, anterior cervical lymphadenopathy, high liver enzymes, atypical lymphocytosis, and eosinophilia (drug reaction with eosinophilia and systemic symptoms [DRESS]). His history was notable for having taken griseofulvin for 3 weeks prior to onset of these findings. He improved after treatment with oral prednisone. We present a rare case of probable DRESS secondary to griseofulvin.     

Vemurafenib‐induced hyperkeratosis of the areola treated with topical adapelene

We present a rare condition, hyperkeratosis of the areola, induced by vemurafenib. Only a few papers have described an association of BRAF inhibitors with hyperkeratosis of the areola and/or nipple. Vemurafenib is a selective BRAF inhibitor used in patients with unresectable or metastatic melanoma who are positive for the V600 mutation. This drug has been associated with numerous cutaneous side effects, both benign and malignant. We report a male patient with vemurafenib‐induced hyperkeratosis of the areola managed successfully with a topical retinoid, and describe for the first time a treatment for this side effect.     

Vemurafenib‐induced toxic epidermal necrolysis: possible cross‐reactivity with other sulfonamide compounds

Vemurafenib is a newly licensed target‐directed medication. It has been proven to improve the survival of patients with metastatic melanoma and the BRAF^V600E mutation; however, adverse cutaneous reactions are frequent. Few cases of life‐threatening severe cutaneous adverse reactions (SCARs) induced by vemurafenib have been reported. Dabrafenib, another selective BRAF inhibitor, has been licensed recently as an alternative drug with the same indications. From a molecular point of view, both vemurafenib and dabrafenib contain a sulfonamide group; cross‐reactivity to sulfonamide compounds has been reported in allergic patients. We report on a patient with vemurafenib‐induced toxic epidermal necrolysis (TEN). In vitro analysis of lymphocyte reactivity to vemurafenib showed positive results, confirming drug causality. In addition, lymphocytes from the patient reacted to dabrafenib and to the antibiotic sulfonamide drug sulfamethoxazole. Moreover, lymphocytes from two patients with cutaneous adverse reactions to sulfamethoxazole also reacted to vemurafenib and dabrafenib in vitro. These data strongly suggest that there might be clinical cross‐reactivity between BRAF inhibitors and sulfonamides in some patients. Thus, precautions should be taken to avoid sulfonamide drugs as much as possible in patients showing serious hypersensitivity reactions to vemurafenib and vice versa.     

Palisaded Neutrophilic Granulomatous Dermatitis in a Child with Juvenile Idiopathic Arthritis on Etanercept

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare neutrophilic dermatosis that may be associated with significant morbidity because of associated pruritus and pain. It is typically seen in adults with collagen vascular diseases, but may be associated with medications as well. PNGD is rarely reported in children. We describe a girl with polyarticular juvenile idiopathic arthritis who developed PNGD after administration of etanercept and demonstrated rapid and significant improvement upon treatment with topical triamcinolone 0.1% ointment.     

Vemurafenib‐induced interface dermatitis manifesting as radiation‐recall and a keratosis pilaris‐like eruption

Vemurafenib is a specific inhibitor of the V600E mutated BRAF protein kinase used for the treatment of unresectable or metastatic melanoma harboring this mutation. Multiple predictable side effects have been described with use of this targeted therapy, and implicate BRAF and mitogen activated protein kinase (MAPK) signaling pathways in their pathogenesis. Herein, we report the novel finding of an interface dermatitis in radiation recall and a keratosis pilaris‐like clinical reaction in a patient treated with vemurafenib.     

Guanfacine‐Induced Lichenoid Drug Eruption in a Child with Autism and Attention Deficit Hyperactivity Disorder

Lichenoid drug eruptions (LDEs) have a variety of medication causes. We report a case of a 5‐year‐old boy with autism and attention deficit hyperactivity disorder treated with guanfacine who developed pruritic lesions consistent with LDEs. Rechallenge was not attempted. There are several clinical and histopathologic clues that may distinguish LDEs from lichen planus.     

Pancreatic Panniculitis in an 18‐Month‐Old with Complete DiGeorge Syndrome

Pancreatic panniculitis, characterized by tender, erythematous subcutaneous nodules occurring most commonly on the lower extremities, occurs in 2% of cases of pancreatic disease. We present a rare case of pancreatic panniculitis in a child with complete DiGeorge syndrome.     

Crohn's Disease Presenting as a Neutrophilic Dermatosis in a 5‐Month‐Old Boy

A 5‐month‐old boy with a previous history of failure to thrive and poor feeding was admitted to the hospital with failure to thrive, oral ulcers, and a generalized vesiculopustular rash that demonstrated a subcorneal pustule and neutrophilic infiltrate on histology. Esophagogastroduodenoscopy and flexible sigmoidoscopy biopsies demonstrated chronic active colitis with granulomas, consistent with the diagnosis of Crohn's disease. Our case represents, to our knowledge, the youngest person reported with this condition in association with Crohn's disease.     

Panniculitis in a patient with dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.     

Panniculitis in a 3‐year‐old child with Fanconi anemia‐associated bone marrow hypoplasia heralds transformation to acute myeloid leukemia

Fanconi anemia is a rare, autosomal recessive genomic instability disorder characterized by congenital limb anomalies, panmyelopathy and a high risk of malignancy, principally acute myeloid leukemia. Hematologic malignancy presenting with acute febrile neutrophilic dermatosis (Sweet syndrome), both deep and superficial forms, is well described in Fanconi anemia patients but is a rare phenomenon in otherwise healthy children. We present a case of panniculitis (presumptive subcutaneous Sweet syndrome) heralding transformation to acute myeloid leukemia in a 3‐year‐old boy with a severe Fanconi anemia phenotype.     

Panniculitis in Juvenile Dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. We present a 14-year-old African American boy with a 4-year history of dermatomyositis referred for evaluation of tender, indurated plaques and nodules on the trunk and proximal extremities. A biopsy specimen revealed epidermal and dermal changes consistent with dermatomyositis. Although calcification was absent, a striking lobular panniculitis was observed. A total of seven cases of clinical panniculitis in association with dermatomyositis have been published. As in our patient, it typically presents as indurated, tender plaques and nodules on the arms, thighs, and buttocks. Although in the vast majority of cases panniculitis found in association with dermatomyositis is subclinical and represents an incidental histopathologic finding, the case we present further emphasizes the need to recognize panniculitis as a rare, but clinically relevant pathologic feature seen in certain lesions of juvenile dermatomyositis.