Disseminated ulcerating lupus panniculitis emerging under interferon therapy of hairy cell leukemia: Treatment‐ or disease‐related?

We report a 43‐year‐old woman, who underwent therapy with interferon‐α for hairy cell leukemia. During interferon‐α therapy she developed multiple subcutaneous swellings, accompanied by fever and fatigue. A skin biopsy revealed lobular, T‐cell lymphocytic panniculitis. In conjunction with the clinical and immunological findings, the diagnosis of lupus panniculitis was made and interferon‐α therapy stopped. Initially, she responded well to oral prednisone and hydroxychloroquine, but after several months she became resistant to it. Her condition worsened, she developed skin ulcers in the inflamed regions. Only with the leukemia‐targeted therapy using cladribine and rituximab her skin condition could be controlled, suggesting hairy cell leukemia as an additional trigger of the lupus panniculitis. Our report is the first one to show induction of lupus panniculitis under interferon therapy of hairy cell leukemia and its presumable sustentation by the latter.     

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms is presented. A 43‐year‐old woman presented with repeatedly occurring aphthous stomatitis for several years. She had been treated for absence seizures, epilepsy and schizophrenia since she was 9 years old. She presented with multiple aphthous stomatitis on her gingiva, erythema nodosum‐like symptoms on the right lateral aspect of her leg and genital ulcers on her perianal area. She also showed polyarthritis. Laboratory examinations revealed elevated C‐reactive protein, elevated neutrophil counts, decreased serum Fe and elevated serum Cu. Histological examination showed perivascular neutrophil and mononuclear cell infiltrates and eosinophilic change of the vessel wall in the lobules of subcutaneous fat tissue. Six weeks after the oral prednisolone therapy, she showed resolution of aphthous stomatitis, folliculitis‐like eruption and genital ulcer. She experienced severe abdominal pain after the start of treatment of Behçet's disease. Plain computed tomography revealed edematous change in the appendix, and ascending and transverse colon. These results led to the diagnosis of entero‐Behçet's disease acute exaggeration. Treatment with infliximab (300 mg/once) was started. Eight weeks after the start of infliximab, her abdominal pain disappeared and C‐reactive protein decreased, followed by the successful change to adalimumab infusion therapy.     

Post‐steroid panniculitis in an adult

Post‐steroid panniculitis is known to be very rare and most of the reported cases have been in children after corticosteroid therapy. We present a case of post‐steroid panniculitis occurring in a 60‐year‐old man after massive, long‐term administration of corticosteroids for acute exacerbation of chronic obstructive pulmonary disease (COPD). Histopathological examination of a nodule revealed a patchy area of fat necrosis, several multinucleated giant cells containing needle‐shaped clefts. The lesions subsided completely in approximately 12 weeks without any treatment. We suggest that post‐steroid panniculitis is not confined to childhood but also occurs in adulthood.     

Metabolic panniculitis: alpha‐1 antitrypsin deficiency panniculitis and pancreatic panniculitis

Panniculitis can be the initial presentation of both alpha‐1 antitrypsin deficiency and pancreatic disease. They can both present with abscess‐like draining nodules, but may present like other forms of panniculitis with erythematous nodules. It is important to consider these in the differential diagnosis of patients presenting with panniculitis. Alpha‐1 antitrypsin deficiency is a relatively common disorder mainly affecting the lungs and liver. It frequently goes undiagnosed, yet critical interventions can be made to minimize disease progression. Panniculitis associated with alpha‐1 antitrypsin deficiency can be difficult to treat. Pancreatic panniculitis occurs in less than 3% of patients with underlying pancreatic disease and is often associated with arthritis. Diagnosis and treatment of the underlying pancreatic disease is imperative.     

Successful treatment of refractory idiopathic angio‐oedema with omalizumab: review of the literature and function of IgE in angio‐oedema

The management of angio‐oedema is quite complicated because of the frequent poor response to usual treatments, including H1 antihistamines, systemic corticosteroids, immunosuppressant drugs, antileucotriene drugs or tranexamic acid. Omalizumab, an anti‐immunoglobulin (Ig)‐E antibody, has shown good resuts in chronic spontaneous urticaria, and might also be a good option for angio‐oedema. To our knowledge, there have been eight relevant case reports published in the English literature. We report two further cases (a 55‐year‐old man and a 36‐year‐old woman) with severe chronic recurrent angio‐oedema affecting the face, pharynx, hands, feet and hips. Both patients were refractory to H1 antihistamines, systemic corticosteroids and tranexamic acid. We decided to start with subcutaneous omalizumab 375 mg every other week and tapering doses up to 375 mg every 4 weeks. Both patients showed a very good response to the therapy, with clinical improvement in the first week. This response remained stable more than 3 years later.     

Vemurafenib‐Induced Neutrophilic Panniculitis in a Child with a Brainstem Glioma

Neutrophilic panniculitis is a rare adverse effect of therapy with selective BRAF inhibitors. We report a case of neutrophilic panniculitis in a 15‐year‐old girl receiving treatment with vemurafenib for a brainstem glioma. Clinicians should be aware of this rare but important side effect of vemurafenib. This is the first report of neutrophilic panniculitis in a child treated with vemurafenib.     

Gouty panniculitis in a 68‐year‐old man: case report and review of the literature

Background Gouty panniculitis is a very rare disease. To date, only eight patients have been reported. In each case, time to diagnosis was lengthy. We describe a 68‐year‐old man with a 6‐month history of tender, erythematous plaques and nodules involving the anterior aspects of the legs Methods and results Histologic examination led to the diagnosis of gouty panniculitis. Conclusion Review of the literature and the present case suggests that hyperuricemia is a necessary but probably not sufficient condition for gouty panniculitis. Why gouty panniculitis very seldom arises as a manifestation of gout is puzzling. In almost all reported patients, uricostatic or uricosuric therapy leads to clinical improvement of the disease.     

Acquired idiopathic lipoatrophic panniculitis in a 12‐month‐old infant

Lipoatrophic panniculitis is a rare condition affecting mainly children, often associated with connective tissue disease. We report a healthy 12‐month‐old girl with no clinical or laboratory features of connective tissue disease who presented with the progressive appearance of annular atrophic plaques beginning at the left arm. A histopathological analysis revealed lobular panniculitis, with fat necrosis and an associated inflammatory infiltrate supporting the diagnosis of lipoatrophic panniculitis. Lipoatrophic panniculitis should be considered in infants and young children with clinical features of panniculitis and fat atrophy even without clinical or serologic evidence of connective tissue disease.     

Ponatinib‐induced neutrophilic panniculitis

Ponatinib is a bcr‐abl tyrosine‐kinase inhibitor (TKI) used to treat resistant and refractory chronic myeloid leukemia and Philadelphia chromosome‐positive acute lymphoblastic leukemia that express bcr‐abl. Neutrophilic panniculitis has been described in rare cases of patients on other TKIs in the same class as ponatinib. We present the first case of neutrophilic panniculitis following treatment with ponatinib and summarize the other cases of panniculitis caused by TKIs.     

Sjögren's syndrome and granulomatous panniculitis

An 83‐year‐old Cambodian woman presented with multiple painful erythematous nodules on the lower legs, forearms and wrists. Subsequent history and investigations revealed a granulomatous lobular panniculitis associated with Sjögren’s syndrome. This is a rare cutaneous association of Sjögrens syndrome with only three similar case reports in the literature.     

Dermatomyositis panniculitis: A case report

Dermatomyositis‐related panniculitis is a rare cutaneous manifestation of dermatomyositis. There are few reported cases in the medical literature. We present the case of a 60‐year‐old woman with a 2‐year history of dermatomyositis and recent biopsy‐confirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.     

Sweet's syndrome with subcutaneous involvement associated with pegfilgrastim treatment: first reported case

Neutrophilic panniculitis is an infrequent entity, considered by most authors as part of the ‘neutrophilic dermatosis’ spectrum. Few cases have been reported to be related with granulocyte colony‐stimulating factor (G‐CSF); we report a case of neutrophilic panniculitis and Sweet's syndrome lesions related with pegfilgrastim, a long‐acting G‐CSF. A 77‐year‐old woman with M2 acute myeloid leukemia was treated with chemotherapy as well as broad‐spectrum antibiotics and antifungal drugs because of febrile neutropenia. Ten days after a single dose of pegfilgrastim, she developed a limited number of purple plaques on the neck, left leg, both arms and several indurated and slightly mobile nodules on her forearms. Skin biopsy of a plaque showed a diffused dermal neutrophilic infiltrate with dermal edema. Biopsy of a nodule showed a lobular neutrophilic panniculitis without vasculitis. No foreign material was found in those biopsies. No organisms were detected in blood, urine or tissue cultures. She was started with prednisolone 40 mg once a day, with dramatic improvement within the next 2 days. This case is noteworthy for the simultaneous appearance of Sweet's syndrome and neutrophilic panniculitis and it is the first case of neutrophilic panniculitis associated with this drug, pegfilgrastim.     

A case of Wong‐type dermatomyositis treated with rituximab and IVIG

A 37‐year‐old woman presented to our rheumatology–dermatology clinic with a rash, muscle weakness and fatigue. She has had prior diagnoses of cutaneous lupus and lichen planus based on skin biopsies. She did not respond to topical steroids, hydroxychloroquine and dapsone. Clinically, she had sharply demarcated photo‐distributed erythema over the upper back, chest and upper arms, along with hyperkeratotic follicular papules on bilateral upper arms, shoulders, posterior neck, behind the ears, chest including breasts, abdomen and right buttock. Investigations revealed a high titre ANA, elevated creatinine kinase, aldolase and positive anti‐MJ/nuclear matrix protein 2 (NXP‐2). A skin biopsy showed findings of connective tissue disease. The diagnosis of Wong‐type dermatomyositis was made. She responded to therapy with mycophenolate mofetil, rituximab and IVIG.     

Equestrian chilblain: another outdoor recreational hazard

Herein, we describe two cases and review 14 cases of equestrian chilblain or ‘equestrian cold panniculitis’ in the literature. The first, a 23‐year‐old healthy female horse trainer, presented with burning nodular swelling on her lateral thighs. The second was a 34‐year‐old healthy woman with recurrent nodular eruption on the lateral thighs after horseback riding in the winter. Physical examination of both patients revealed erythematous to violaceous nodules with eczema craquelé‐like changes. Laboratory workup for systemic and autoimmune connective tissue disease was negative. Punch biopsies from both patients showed a superficial and deep perivascular and periadnexal lymphocytic infiltrate with focal extension into the subcutaneous fat. Parakeratosis, subtle spongiosis and increased pandermal interstitial mucin were also present. Previously reported cases generally showed a similar clinical course and similar histopathologic findings. In contrast, our cases revealed increased pandermal interstitial mucin, resembling tumid lupus erythematosus. We aim to better characterize the histopathologic findings of equestrian chilblain and discuss its relationship to other cold‐induced skin injuries and autoimmune connective tissue disease, namely lupus erythematosus.     

Composite cutaneous lymphoma of diffuse large B‐cell lymphoma‐leg type and subcutaneous panniculitis‐like T‐cell lymphoma

Composite lymphoma (CL) is a rare disease defined by the occurrence of two distinct lymphomas within a single tissue at the same time. We present the case of an 89‐year‐old male with a clinical history of immunoglobulin M monoclonal gammopathy of undetermined significance. The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of two components. The first one consisted of large B‐cells with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitic manner, and was CD3+/CD8+/granzyme B+/TCRβF1+. The final diagnosis was CL of primary cutaneous large B‐cell lymphoma‐leg type (PCLBCL‐leg type) and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL). We report and characterize for the first time coexistent PCLBCL‐leg type and SPTCL in a patient.     

Granulocyte colony‐stimulating factor‐induced granulomatous dermatitis with enlarged histiocytes clinically manifesting as painful edematous nodules with high fever similar to Sweet's syndrome

A 72‐year‐old woman with a history of diffuse large B cell lymphoma and recent recurrence visited our department complaining of several painful edematous nodules with blisters on her face. She had iteratively developed cutaneous eruptions after every treatment with granulocyte colony‐stimulating factor (G‐CSF) for neutropenia, and each time the eruption improved after the cessation of the G‐CSF treatment. The blisters became crusty and the skin lesions slightly improved, but on the 24th hospital day, the eruption formed painful erythematous nodules with erosion, and the patient also developed a high fever of up to 38°C. A biopsy specimen showed a dermal infiltrate of increased and enlarged plump histiocytes, some of which indicated karyomitosis with a small number of lymphocytes. No increase in the number of eosinophils or neutrophils was noted. These eruptions lasted for 15 days and disappeared with the recovery of the peripheral blood count and attendant cessation of G‐CSF. We diagnosed this case as G‐CSF‐induced granulomatous dermatitis with enlarged histiocytes. Several cases with maculopapular rash and dermal inflammatory infiltrate composed of interstitially arranged large histiocytes have been reported. However, to the best of our knowledge, this is the first case report of G‐CSF‐induced granulomatous dermatitis with enlarged histiocytes clinically manifesting as painful edematous nodules with a high fever, similar to Sweet's syndrome. We speculated that the infiltrating cells were not neutrophils but histiocytes, presumably because of agranulocytosis.     

Successful therapy of pemphigus vulgaris with immunoadsorption using the TheraSorb™ adsorber

Pemphigus vulgaris (PV) is caused by autoantibodies to desmogleins. Standard immunosuppressive therapy may be limited by concomitant diseases or ineffective.In these cases, removal of circulating antibodies by immunoadsorption can induce remission. An 87‐year‐old woman with PV and considerable co‐morbidities suffered from extensive mucous membrane erosions and bouts of skin blistering refractory to treatment with methotrexate,as well as mycopheno‐late mofetil and corticosteroids even when combined with plasmapheresis and intravenous immunoglobulin. Adjuvant immunoadsorption therapy with the TheraSorb? columns‐first weekly, than monthly‐induced a complete remission, as well as a parallel decrease in PV antibody titers. Continued therapy for 18 months kept the patient in remission but for one infection‐related relapse, and remission has been stable over two further years on low‐dose methotrex‐ate monotherapy. This case confirms the clinical efficacy of immunoad‐sorption in PV and adds another type of adsorber column to the armamentarium, which is useful to the dermatologist who has to cooperate with local lipid apheresis or dialysis units.     

Low‐dose combination therapy of severe digital ulcers in diffuse progressive systemic sclerosis with the endothelin‐1 receptor antagonist bosentan and the phosphodiesterase V inhibitor sildenafil

Digital ulcers in progressive systemic sclerosis (PSS) are often refractory to therapy. A frequently chronic aggressive course can lead to the loss of acral limbs involved. A 73‐year‐old woman developed a dramatic worsening of her ulcerations despite maximum conventional therapy. Switching therapy to bosentan and sildenafil, both in low‐dose regimens, resulted for the first time in ten years in a complete healing of the ulcers. If substantiated in a series of patients, the additive and perhaps synergistic clinical benefits of combining bosentan and sildenafil may be a valuable option for the treatment of acral ulcers in PSS.     

Ulcerative necrobiosis lipoidica responsive to colchicine

Necrobiosis lipoidica is an uncommon granulomatous disease of unknown aetiology. Few treatments have emerged with consistent efficacy and the ulcerated form of necrobiosis lipoidica can be particularly difficult to treat. A 56‐year‐old non‐diabetic woman with chronic ulcerative necrobiosis lipoidica unresponsive to other therapies was commenced on colchicine treatment. Complete resolution of the ulcers was observed after 2 months therapy with colchicine 500 µg twice daily.