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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 21, Issue 12, 2015

Case Presentation

Sarcoidosis

We present a 28-year-old man with a one-year history of cutaneous lesions in old scars and tattoos with concomitant subcutaneous nodules and myopathy. A skin biopsy specimen showed cutaneous sarcoidosis. We discuss the multiple aspects of this case, which represent unique presentations of this systemic disease as well as review isomorphic and isotopic responses.

Acrokeratoelastoidosis

Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers.

Possible photoactivated dermatitis with features of post-inflammatory pigmentary alteration (PIPA) and rosacea

Cutaneous flushing and facial erythema are common dermatologic conditions that elicit a wide differential diagnosis that includes rosacea, seborrheic dermatitis, photodermatitis, connective-tissue diseases, carcinoid syndrome, and mastocytosis. Herein we present an usual case of a mask-like rosacea-PIPA overlap that occurred in a patient with prior history of rectal carcinoid tumor and a negative systemic evaluation.

Squamous-cell carcinoma in situ

We present a 30-year-old woman with atopic dermatitis and ichthyosis vulgaris and a one-year history of an erythematous, scaly plaque on the dorsal surface of her right hand, which developed three years after an accidental exposure to prolonged ultraviolet C (UVC) radiation in a laboratory accident. The plaque, which was initially treated as eczematous dermatitis, was eventually identified histopathologically as squamous-cell carcinoma in situ. Although causation is not definitive, this case is the first to describe development of non-melanoma skin cancer (NMSC) in an area of skin known to be acutely exposed to UVC radiation. As UVC radiation becomes a more frequently used anti-microbial technology, UVC radiation may become a more commonly identified risk factor in the development of NMSC.

Angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia is a rare, benign, vascular proliferation that presents as dome-shaped, light-pink-to-red-brown papules or subcutaneous masses that lack distinguishing surface changes. The condition typically presents as a single lesion or multiple lesions that involve contiguous areas. The pathogenesis is poorly understood. Angiolymhpoid hyperplasia with eosinophilia has been associated with antecedent trauma, T-cell proliferation, infection, and hormone imbalance. This report details a case of widespread angiolymphoid hyperplasia with eosinophilia that flared while the patient was pregnant.

Extragenital bullous lichen sclerosus

Lichen sclerosus (LS) is a chronic, inflammatory dermatosis that is characterized by pruritic, white, atrophic plaques that classically affect the anogenital region of postmenopausal women. Extragenital involvement also may occur with several reported morphologic variants. Extragenital bullous LS is a rare variant, which presents as flaccid bullae that favor the trunk and proximal aspects of the extremities. The treatment of extragenital bullous LS is similar to that of genital LS. However, extragenital LS is often less responsive and may present a therapeutic challenge. We describe a 65-year-old woman with a two-year history of vulvar and extragenital LS, who developed a bullous eruption within a pre-existing patch of lichen sclerosis on the breast. We review the clinical and histopathologic features of extragenital bullous LS and discuss current treatment options, which include those for recalcitrant cases.

Benign familial pemphigus ( Hailey-Hailey disease)

A 56-year-old man presented with a 15-year history of scaly red plaques on the trunk and axillae. Skin biopsy was consistent with Hailey-Hailey disease. His condition was refractory to multiple therapies, which included topical and oral antibiotics and topical, intralesional, and oral glucocorticoids. Treatment with subcutaneous botulinum toxin type A at the axillae and on the back led to a nearly complete resolution of plaques in those areas. Botulinum toxin type A should be considered in patients with extensive Hailey-Hailey disease who are fail traditional therapies.

Piloleiomyomas in multiple cutaneous and uterine leiomyoma syndrome (hereditary leiomyomatosis and renal cell cancer or Reed syndrome)

Multiple cutaneous and uterine leiomyoma (MCL), or Reed syndrome is an uncommon condition that includes cutaneous piloleiomyomas and internal neoplasms of the uterus and kidney. Clinical findings include clusters of variably-painful, skin-colored-to-red-brown papules. Genetic testing shows germline mutations in the gene encoding fumarate hydratase. We describe a patient with MCL who presented with subtle cutaneous findings. We believe it is important that dermatologists consider a diagnosis of MCL when presented with patients with small painful dermal papules.

Generalized granuloma annulare in a folliculocentric distribution

We present a 69-year-old man with type 2 diabetes mellitus and a five-year history of an eruption of follicular pustules, papules, and nodules, which was identified histopathologically as folliculocentric granuloma annulare (GA). Folliculocentric generalized GA is a rarely reported variant of GA, in which the the palisading histiocytes form focal granulomas in a follicular pattern. In this case, the GA may represent an isotopic phenomenon, with lesions developing in hair follicles that were previously affected by a suppurative folliculitis.

Eosinophilic dermatitis of hematologic malignancy

Eosinophilic dermatosis of hematologic malignancy is a rare, paraneoplastic phenomenon that presents as a pruritic papular or vesicular eruption that is clinically and histopathologically similar to insect bites. We present a 56-year-old man with multiple relapses of diffuse large B cell lymphoma with a typical presentation of pruritic papules and vesicles on the extremities that correlate with a recent relapse of his lymphoma.

Periungual pyogenic granuloma formation in a patient with complex regional pain syndrome

Peripheral nerve injury has been associated with the development of periungual pyogenic granuolomas (PGs). We present the case of a 39-year-old woman with an eight-month history of periungual PGs in the setting of a four-to-five year history of a traumatic inciting event that produced symptoms consistent with complex regional pain syndrome (CRPS). Although recurrent, these periungual PGs have remitted after treatment with topical timolol maleate. This case exhibits an underappreciated association between peripheral neurologic abnormalities, which include CRPS, and cutaneous abnormalities. It also presents evidence that supports the concept that PGs that are not appropriate for surgical treatments may be treated with topical timolol maleate.

Paraneoplastic erythema annulare centrifugum eruption (PEACE)

Erythema annulare centrifugum (EAC) is a reactive erythema with distinct, annular, erythematous plaques with trailing scale. This condition has been associated with various etiologies, which include an associated malignant condition. EAC with cancers or paraneoplastic erythema annulare centrifugum eruptions (PEACE), is more likely to be associated with lymphoproliferative malignancies such as lymphomas and leukemias. Histopathologic features include a superficial and deep, lymphohistiocytic perivascular infiltrate. We present a patient with a history of diffuse large B cell lymphoma in remission for two years, who presented with a one-year history of EAC.

Photodistributed granuloma annulare

Annular elastolytic giant cell granuloma (AEGCG) is a controversial entity that is considered by many to be a variant of granuloma annulare (GA). The majority of cases of AEGCG occur in Caucasian women (3:2) between the ages of 40 and 70, with the distribution of the mostly annular lesions favoring exposed areas of skin and rarely involving covered skin. The most common systemic association has been with diabetes mellitus. We present a 52-year-old woman with an asymptomatic, annular, erythematous, photodistributed eruption of two-years duration. As part of her evaluation, it was detected that she had a hemoglobin A1C of 10.3% and a diagnosis of diabetes mellitus was made. We review the literature on the clinical and histopathologic features of GA and AEGCG and the overlap between these entities.

Multiple eruptive dermatofibromas

We report a 34 year-old woman with psoriasis, systemic lupus erythematosus (SLE), and recent anti-TNFa therapy, who presented with multiple, eruptive dermatofibromas (MEDF). Although the pathogenesis of MEDF remains unknown, there is substantial evidence that this phenomenon represents an aberrant immune response. Like the more common presentation of solitary dermatofibromas, these lesions are benign, and no treatment is required. However, MEDF is increasingly recognized as a sign of immune dysregulation and an appropriate work-up should be initiated to identify an underlying cause in patients without a known trigger.

Keratolysis exfoliativa

Keratolysis exfoliativa (KE) is a palmoplantar eruption of air-filled bullae on an erythematous base, which results in lamellar peeling with hallmark superficial collarettes of scale. It is distinct from other diseases of volar skin, such as dyshidrosis, contact dermatitis, tinea, epidermolysis bullosa, and acral skin peeling. We present a 55-year-old woman with extensive disease on the hands and feet, who failed to respond to standard topical therapy but showed a marked dose-response improvement with the use of oral acitretin. Recent histopathologic and molecular studies have linked KE to premature corneo-desmosomal disruption. Acitretin has previously been used to treat diseases of abnormal corneocyte desquamation, for example Netherton’s disease. To the best of our knowledge, this report is the first that documents the efficacy of the use of systemic acitretin in KE.

Mycobacterium avium complex infection

We report the case of a 30-year-old woman with a three-year history of papules, nodules, and multiple ulcers of the left lower extremity. A skin biopsy specimen showed dermal fibrosis and patchy granulomatous inflammation in the upper-to-mid dermis that was associated with a perivascular, lymphohistiocytic infiltrate along with granulomas and necrosis in the subcutaneous fat. Tissue culture showed Mycobacterium avium complex infection. Clinical manifestations of cutaneous nontuberculous mycobacterial infections are discussed as well as their potential association with pedicures.

IgA vasculitis (Henoch-Schönlein purpura)

We report the first case of direct immunoflourescence-proven immunoglobulin A (IgA) vasculitis associated with influenza infection in an adult patient. IgA vasculitis, which was previously known as Henoch-Schönlein purpura, is the most common systemic vasculitis in children but rarely occurs in adults. Disease onset often occurs after upper respiratory tract infections that are caused by adenovirus or enterovirus. The American College of Rheumatology defines IgA vasculitis by the presence of any two of the following four criteria: age ≤ 20 years at disease onset, palpable purpura, acute abdominal pain, and a biopsy specimen that shows granulocytes in the walls of small arterioles or venules. Purpura, abdominal pain, and arthralgia comprise the classic triad. Renal involvement may be severe, especially in adults. Treatment is most often supportive but glucocorticoids and/or immunosuppressive agents are recommended in severe or refractory cases.

Lupus miliaris disseminatus faciei

Lupus miliaris disseminatus faciei (LMDF) is a rare, inflammatory condition, which is characterized by red-brown and yellow-brown papules on the face, with characteristic involvement of the eyelids and with histopathologic findings of suppurative and granulomatous folliculitis and dermatitis. The etiology of this disease is not known, but retinoids, anti-inflammatory, immunosuppressive, and antimicrobial medications are utilized to treat the condition with variable results. We present the case of a patient with LMDF that has thus far been refractory to treatment.

Chronic actinic dermatitis occurring in an adult with atopic dermatitis

Chronic actinic dermatitis (CAD) is a photosensitivity disorder that is characterized by a persistent eczematous eruption in sun-exposed sites. The hallmark of CAD is a reduced minimal erythema dose (MED) to ultraviolet B (UVB), ultraviolet A (UVA), and/or to visible light, which makes phototesting the essential diagnostic investigation. The uncommon subgroup of patients with atopic dermatitis (AD) that are affected by CAD has primarily been described in young patients in the United Kingdom. We present an atopic adult women with CAD who was diagnosed years after symptoms began. We believe it is important that dermatologists perform phototests on AD patients with features of a photoaggravated dermatitis in order to avoid delay in diagnosis of a true photosensitivity condition and provide appropriate management.

Nodular amyloidosis

Nodular amyloidosis is the rarest form of primary cutaneous amyloidosis. We report the case of a 74-year-old woman with an eight-year history of asymptomatic, hyperpigmented plaques on the pretibial areas. A skin biopsy specimen showed deposits of amorphous eosinophilic material that extended throughout the dermis with apple-green birefringence with a Congo-red stain, which established a diagnosis of nodular amyloidosis. Patients with nodular amyloidosis should be evaluated for systemic disease and followed appropriately due to a small risk of progression to systemic amyloidosis.

Primary diffuse macular amyloidosis

We present a 53-year-old woman with diffuse macular amyloidosis. We discuss the clinical manifestations, pathophysiologic mechanisms, and associations of cutaneous macular amyloidosis.

Linear atrophoderma of Moulin

We present a 40-year-old woman with asymptomatic, linear, hyperpigmented atrophic plaques in a Blaschkoid distribution on the right back and right upper extremity that is consistent with a diagnosis of linear atrophoderma of Moulin. Clinical lesions developed with a biphasic pattern in late adolescence and in adulthood. The pathogenesis of this acquired, progressive Blaschkolinear dermatosis may hold insight into the pathogenesis of this rare dermatologic condition, as well as other dermotoses, which include those resulting from post-zygotic genetic mosaicism.