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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 20, Issue 1, 2014

Case Report

Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma

Background:  Anakinra is a recombinant form of interleukin-1 receptor antagonist.  It is the drug of choice for Schnitzler syndrome and cryopyrin-associated periodic syndromes.  It has also recently been demonstrated to have activity in the treatment of the non-Langerhans cell histiocytosis known as Erdheim-Chester disease.

Purpose:  To describe the activity of anakinra in a patient with co-existing lichen planus and Erdheim-Chester disease.

Methods:  A 43-year-old woman with progressive Erdheim-Chester disease presented for management of her night sweats and chills, systemic skeletal bone pain, and neurologic (diabetes insipidus) manifestations.  She also had widespread cutaneous lichen planus.  Anakinra, 100 mg subcutaneously daily, was initiated for the treatment of her Erdheim-Chester disease.

Results:  Within 2 days of starting anakinra, there was prompt resolution of her Erdheim-Chester disease-related symptoms.  Subsequently, her bone pain resolved and her diabetes insipidus improved.  Also, the lichen planus-associated pruritus rapidly ceased and most of the skin lesions improved.

Conclusions:  Our experience confirms the efficacy of anakinra for the treatment of Erdheim-Chester disease.  The concomitant improvement of her lichen planus on anakinra suggests that this agent warrants additional study in this disorder.

Imatinib-induced postoperative periorbital purpura: GASP (Gleevec-Associated Surgical Purpura) in a woman with imatinib-treated chronic myelogenous leukemia

Background:  Imatinib mesylate is a selective tyrosine kinase inhibitor used in the treatment of chronic myelogenous leukemia.  Ocular side effects of imatinib include periorbital edema, which may become so severe as to obstruct the visual field.

Purpose: The purpose of this case study is to describe the clinical characteristics of imatinib- induced postoperative periorbital purpura.

Materials and methods: We retrospectively reviewed the medical literature using PubMed, searching the terms edema, Gleevec, imatinib, periorbital, postoperative and purpura.  Patient reports and previous reviews of the subject were critically assessed and the salient features are presented.

Results: Three patients have undergone surgery to reduce the imatinib-induced periorbital edema; two of these individuals have developed imatinib-induced postoperative periorbital purpura.

Conclusion:  We recommend discontinuing imatinib usage one week prior to periorbital surgery and not resuming therapy until the eighth postoperative day.

Increasing recognition of dermatomyositis with subcutaneous edema – is this a poorer prognostic marker?

Subcutaneous edema as a presenting feature of dermatomyositis has infrequently been described and is thought to signify a more aggressive disease course. We report a case involving a 38-year-old man who presented with significant subcutaneous edema involving his neck and upper body; he later developed clinical features and biopsy results consistent with dermatomyositis. Only sixteen previous cases of dermatomyositis with subcutaneous edema involving adults have been published in the literature and we aim to review disease progression, prognosis, and optimal treatment of the condition.

Case Presentation

Alpha-1-antitrypsin deficiency-associated panniculitis: a case report

Panniculitis is a recognized, but rare complication of α1-antitrypsin (A1AT) deficiency. Less than 60 cases have been reported, mostly in the homozygous PiZZ variant. We report the case of a 55-year old woman with A1AT panniculitis associated with the heterozygous phenotype PiMS and discrete reduction of A1AT serum levels. In addition, the pathophysiology of the disease, clinical and histopathological features, and current treatment possibilities are briefly reviewed.

Eruptive purpuric papules on the arms; a case of chemotherapy-induced inflammation of actinic keratoses and review of the literature

Chemotherapy-induced inflammation of actinic keratosis can present in patients with subclinical actinic keratoses that become erythematous and pruritic within weeks of initiating systemic chemotherapy. The reaction is limited to sun-exposed areas and, classically, histologic findings of parakeratosis and epidermal necrosis with keratinocyte nuclear pleomorphism are present. Exuberant reactions with extensive epidermal necrosis may lead to subepidermal vesiculation. We report a case of a 67-year-old man with a history of chronic hepatitis B virus infection and recently diagnosed squamous cell carcinoma of the lung who was noted to have progressive asymptomatic violaceous papules on the extensor forearms and distal upper arms while hospitalized for possible sepsis following initiation of chemotherapy. A dermatology consulatation was requested to rule out possible vasculitis. It is important to recognize chemotherapy-induced inflammation of actinic keratoses in predisposed patients; it may be managed successfully with topical corticosteroids and does not necessitate discontinuation of the offending chemotherapeutic agent.

Primary amyloidosis-induced nail dystrophy

Primary amyloidosis is caused by a monoclonal proliferation of plasma cells and is capable of producing cutaneous lesions. A 56-year-old male was admitted to the hospital for evaluation of chronic back pain and acute lower extremity weakness. On examination, he was noted to have subungual verrucous plaques with overlying nail dystrophy on his bilateral thumbs. A biopsy of one subungual lesion showed a deposition of amorphous material in the dermis that stained with Congo red and crystal violet. These cutaneous lesions ultimately led to the diagnosis of plasma cell dyscrasia and primary amyloidosis. Based on the literature search, primary amyloidosis presenting with this degree of subungual thickening and overlying nail dystrophy has not been previously reported.

Systematized linear epidermolytic hyperkeratosis

A 5-year-old boy presented with widespread asymptomatic hyperpigmented verrucous plaques since 3 months of age. The lesions were distributed in a linear manner along Blaschko's lines on trunk and extremities and were accentuated in flexures and around joints. There was no history of blistering or redness and no other family member was affected. Ichthyosis hystrix (of Curth and Macklin) and generalized linear/mosaic epidermolytic hyperkeratosis (EHK) were considered in the differential diagnosis. Biopsy from both trunk lesion and lesion on knee revealed characteristic epidermolytic hyperkeratosis, thereby clinching the diagnosis of systematized linear EHK.

Acute onset of a vesiculopustular rash in an ICU patient.

A 63 year-old woman with hyperthyroidism was admitted to the Medical Intensive Care Unit for ARDS following damage to her lungs from propylthiouracil.  She was placed on 250 mg SSKI PO TID as an alternative therapy until thyroidectomy could be performed. Four days after admission, she abruptly developed an acneiform rash on her face, shown to be iododerma.  The eruption rapidly resolved after discontinuation of the SSKI.

Treatment of subcutaneous sarcoidosis with hydroxychloroquine: Report of 2 cases

Although the therapeutic benefit of oral antimalarials in various types of sarcoidosis is well described, their reported use specifically in subcutaneous sarcoidosis (Darier-Roussy type sarcoidosis) is limited. We describe the cases of two patients with subcutaneous sarcoidosis treated with hydroxychloroquine, suggesting that it may represent an alternative first-line treatment for patients in whom steroid-sparing therapy is desired.

Congenital malalignment of the great toenail. Report of two cases.

Congenital malalignment of the great toenail is characterized by lateral deviation of nail plates, which are not parallel to the distal phalanx. We report two cases of congenital malalignment of the great toenail in two otherwise healthy children. Because this entity is probably underdiagnosed, a high index of suspicion is required to prevent from diagnostic errors and unnecessary treatments.

Traumatic neuroma of the penis

Traumatic neuromas are tumors produced by a reactive process to regenerate injured nerves that result in a disordered proliferation of nerve bundles.  These tumors are usually related to previous surgery or trauma. We describe a case of traumatic neuroma on the penis of a 24-year-old man; the tumor was initially suspected to be a condyloma.  A shave biopsy was both diagnostic and curative.

Verruciform xanthoma

A 50-year-old man presented with a several month history of a polypoid papule on the scrotum. A dense accumulation of macrophages with foamy cytoplasm was exhibited in the biopsy specimen leading to a diagnosis of verruciform xanthoma.

Getting to the point: a case of a sewing needle retrieved from the thigh

Foreign bodies are rarely retained in the skin after puncture wounds or impalement injuries and are even less commonly initially detected several months after penetration. Sewing needles are most frequently reported in the literature as foreign bodies in cases of ingestion, inoculation of the cranium and heart, and penetration of the knee. Herein we describe a case of a middle-aged man who presented to the outpatient dermatology clinic with an 8-month history of a nodule in his left thigh; he had noted recent onset of mild pain. On examination he was found to have a sharp needle-like point palpable below the skin of his left lateral thigh. Plain radiographs of the left thigh showed a fractured sewing needle overlying the same area. During local incision, two fragments of a sewing needle were removed from the lateral thigh.

Photo Vignette

A case of eosinophilic dermatosis of hematologic malignancy in a patient with multiple myeloma

A 50-year-old man with eosinophilic dermatosis of hematologic malignancy is presented. His dermatosis cleared after chemotherapy produced improved control of his multiple myeloma.

Surgical Pearl

A simplified minimally invasive technique for the treatment of venous lakes

A simplified approach to treat venous lakes of the vermillion lip is presented.  Our method involves the use of a 30 gauge hypodermic needle to deliver a low-powered, high-frequency electrical current from a hyfrecator power source into the venous lake lesion.

Letter

Generalized morphea successfully treated with extracorporeal photochemotherapy (ECP).

A patient is presented with generalized morphea whose disease completely resolved after combination therapy with extracorporeal photopheresis and broad band UVA treatments.

Herpes zoster in a 2-year-old vaccinated against varicella

Herpes zoster is uncommon in the pediatric population. We report a case of herpes zoster in a 2-year-old boy who received the live attenuated varicella zoster virus vaccination at his 12-month pediatric visit. The child was treated with acyclovir and recovered without complications.

Fish odor syndrome: a case report of trimethylaminuria

Trimethylaminuria is a rare, autosomal recessive, metabolic disorder that results in accumulation of trimethylamine (TMA), which smells like rotten fish. The chemical is excreted in sweat and urine owing to a deficiency in the enzyme flavin monooxygenase 3 (FMO3). We report a case of trimethylaminuria in a 12-year-old girl. The patient failed treatment with diet and hygiene modification, but achieved symptomatic improvement after a four-month course of metronidazole.

Chemical burn caused by topical application of garlic under occlusion

Allium sativum (garlic) can cause an irritant contact dermatitis, secondary to naturopathic practices. We report an unusually severe case of chemical burn following garlic applied under occlusion.