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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 22, Issue 12, 2016

Case Presentation

Skin-limited relapse of systemic anaplastic large-cell lymphoma

Anaplastic large-cell lymphomas (ALCLs) are agroup of CD30-positive non-Hodgkin lymphomasthat are linked by common morphologic andimmunophenotypic features but have varyingclinical and genetic characteristics. The World HealthOrganization classification currently recognizes threesubtypes of ALCL: systemic anaplastic lymphomakinase-positive ALCL, systemic anaplastic lymphomakinase-negative (ALK-) ALCL, and primary cutaneousALCL. Here we present a 42-year-old man with ahistory of systemic ALK- ALCL, who was in remissionfor six months before relapsing with skin-limitedanaplastic large-cell lymphoma.

Idiopathic scleredema

Scleredema, which also is known as scleredemaadultorum of Buschke, is an uncommonsclerodermiform condition that is characterizedby progressive thickening and hardening of theskin due to excessive dermal mucin and collagendeposition. The clinical course is variable, andprogression of disease may lead to functionalimpairment with limitations in mobility. The etiologyand pathogenesis are unknown although severalwell-known associations include streptococcalinfection; diabetes mellitus, particularly withmetabolic syndrome; and monoclonal gammopathy.We present a case of scleredema in a 52-year-oldman with no identifiable associated condition,who experienced improvemement with UVBphototherapy.

Acquired aquagenic papulotranslucent acrokeratoderma

Aquagenic papulotranslucent acrokeratoderma isa rare condition with the development of white-totransluscentpapules and plaques after exposureto water. While the first report was described asan autosomal dominant hereditary condition,there have since been acquired cases reported inassociation with cystic fibrosis, with prior exposureto a drug, or as an idiopathic condition. We presenta 24-year-old man with acquired aquagenicpapulotranslucent acrokeratoderma that has beenpresent since infancy, without a family history,without prior drug exposure, and without anypersonal or family history of cystic fibrosis. Thus fartreatment with urea cream, calipotriene ointment,vitamin E cream, and clobetasol ointment hasbeen ineffective. Our patient will be treated withbotulinum toxin.

Kaposi’s sarcoma in an HIV-negative patient

We report an HIV-negative, 55-year-old manwith recurrent Kaposi’s sarcoma (KS) of the lowerextremities, who does not fit into any of thefour previously described variants of KS: classicKS, AIDS-related KS, iatrogenic KS, and AfricanendemicKS. There are reports in the literature ofchildhood-onset KS, which is thought to be dueto an inherited immune deficiency that confers ahigher susceptibility to human Herpesvirus-8 (HHV-8), which is the virus that is known to cause KS. Ourpatient may be affected with an inherited immunedeficiency that has predisposed him to KS, and thismutation also may account for his prostate andbladder cancer.

Lichen planus pigmentosus

Lichen planus pigmentosus (LPP) is a type oflichenoid dermatitis with superficial dermalmelanophages that presents as symmetrical,hyperpigmented macules and patches that aredistributed over the forehead, temples, cheeks, andneck. The condition most often occurs in darkerskinned individuals and is frequently resistant totreatment. Here we present a patient of Egyptiandecent with a lacy reticulated LPP eruption on theface.

Multiple pilomatricomas in the setting of myotonic dystrophy

The association between multiple pilomatricomasand the autosomal dominant neurodegenerativedisorder myotonic dystrophy has been described inthe literature. Although the mechanism is unknown,it is hypothesized that the dystrophia myotonicaprotein kinase mutation in myotonic dystrophyaffects intracellular calcium levels, which alterproliferation and terminal differentiation that leads tocells that are observed in pilomatricomas. We presenta patient with multiple, symptomatic pilomatricomasand myotonic dystrophy, with a strong family historyof both of these rare disorders.

Primary cutaneous smoldering adult T-cell leukemia/ lymphoma

HTLV-1 is a virus that is endemic in southwesternJapan and the Caribbean and has been implicatedin the development of ATLL. ATLL, which is anuncommon malignant condition of peripheralT-lymphocytes, is characterized by four clinicalsubtypes, which include acute, lymphomatous,chronic, and smoldering types, that are based onLDH levels, calcium levels, and extent of organinvolvement. We present a 52-year- old woman withpruritic patches with scale on the buttocks and withtender, hyperpigmented macules and papules oftwo-years duration. Histopathologic examinationwas suggestive of mycosis fungoides, laboratoryresults showed HTLV-I and II, and the patient wasdiagnosed with primary cutaneous ATLL. We reviewthe literature on HTLV-1 and ATLL and specifically theprognosis of cutaneous ATLL. The literature suggeststhat a diagnosis of ATLL should be considered amongpatients of Caribbean origin or other endemicareas with skin lesions that suggest a cutaneousT-cell lymphoma, with clinicopathologic features ofmycosis fungoides. Differentiation between ATLLand cutaneous T-cell lymphoma is imperative as theyhave different prognoses and treatment approaches.

Gyrate erythema in the setting of tinea pedis

Gyrate erythema, which also is known as erythemaannulare centrifugum (EAC), is a reactive dermatitisthat is thought to occur in response to an underlyingtrigger. The superficial form is characterized bythe typical, centrifugally-expanding, annular,erythematous patches or plaques with a distincttrailing scale. The deep form also is a centrifugallyexpanding,erythematous plaque but with induratedborders and absence of scale. These cutaneousfindings are thought to be reactive, most often inresponse to infections or drugs and, less likely, tounderlying malignant conditions.

Acral keratoses and squamous-cell carcinomas likely associated with arsenic exposure

Chronic arsenic exposure is known to inducepunctate keratoses with an increased risk ofprimary squamous-cell carcinoma. Drinking wateris currently the major source of arsenic exposureworldwide and is considered one of the mostsubstantial environmental carcinogens. We describethe case of a 61-year-old Hungarian woman withscattered, acral, hyperkeratotic papules and a historyof five palmoplantar squamous-cell carcinomasas well as two other extremity non-melanomaskin cancers. Prior to immigration, she had livedin a county of Southern Hungary that is known tohave elevated concentrations of inorganic arsenicin the drinking water above the World HealthOrganization’s current maximum threshold forsafety. To date, this report is the first to describethe phenomenon of palmoplantar squamouscellcarcinomas in a patient from this region andunderscores the importance of vigilant screening inthose individuals who have spent substantial time inhigh-risk regions internationally and domestically.

Cowden syndrome presenting with trichilemmomas

Cowden syndrome (CS) is a genetic cancerpredisposition syndrome that is associated withgermline mutations in the phosphate and tensinhomologue deleted on chromosome ten (PTEN)tumor suppressor gene. It is characterizedby the formation of benign and malignanttumors. Characteristic benign tumors includetrichilemmommas, acral keratoses, mucocutaneousneuromas, and oral papillomas. The most commonmalignant condition include breast, thyroid, andendometrial cancers. We present a case of a30-year-old woman with CS, who initially presentedwith trichilemmomas that were misdiagnosed ascomedonal acne. Recognition of the presentingfeatures of CS is important to ensure proper referral,management, and treatment for these patients.

Silicone granulomas with ulcers

We present a 42-year-old transgender womanwith woody induration over her buttocks andlower extremities as well as persistent ulcers of thebuttocks. The lesions developed ten years prior to herpresentation and approximately five years after shereceived illegal silicone injections to her buttocks.Histopathologic examination was consistent witha silicone granuloma. Silicone granuloma is a notan uncommon side effect of silicone injections andmore often occurs when the filler is administeredby non-physician practitioners, as is the case in thispatient. Ulcerative silicone granulomas, however,rarely are reported. In this case, the patient’shemodialysis treatments, which required longperiods of weight bearing on her buttocks, may havepredisposed her to ulcers in this area, and the ulcersmay have been in part due to poor vascular supplyas well as physical pressure. Treatment of this patientis relatively challenging, owing to her multiplecomorbidities that include end-stage renal diseaseand congestive heart failure.

Herpes simplex virus in erythrokeratoderma variabilis

We report a 48 -year-old woman witherythrokeratoderma variabilis, which is a rarehereditary disorder of keratinization, who developednew, painful, blisters within her skin lesions. Thediagnosis of herpes simplex virus infection was madebased on the clinical history and histopathologicfeatures. She was successfully treated withprophylactic valacyclovir, and her herpetic outbreakshave halted. This case serves as a reminder thateven among the most rare skin disorders, commonsecondary complications may be easily overlooked.

White fibrous papulosis of the neck

White fibrous papulosis of the neck is a rare diagnosisthat typically presents with white, 2-to-3-mmpapules on the posterior aspect of the neck. Wepresent an elderly woman with diffuse involvementon her abdomen and back.

Darier-White disease

We present a 58-year-old woman with bipolardisorder and with a longstanding history of yellowbrown,hyperkeratotic papules in a seborrheicdistribution and nail changes. Her father andpaternal grandmother had similar eruptions and alsohad psychiatric disease. Histopathologic examinationshowed acantholysis and dyskeratosis, which wasconsistent with Darier-White disease. Darier-Whitedisease is a rare, hereditary disorder that is inheritedin an autosomal dominant manner and that usuallypresents in adolescence or early adulthood dueto mutations in the SERCA2 calcium pump. Theresultant alterations in calcium homeostasis affectdesmosome assembly and lead to acantholysis andapoptosis, which creates the characteristic eruption.Genetic linkage analysis of affected families showsthat a susceptibility locus for bipolar disorder cosegregateswith genetic markers that are found inthe Darier-White disease region. Treatments, whichinclude topical emollients, mild keratolytics, andtopical or oral retinoids, are aimed at improvingthe appearance of skin, relieving symptoms, andpreventing or treating infectious complications.

Gardner-Diamond syndrome

Gardner-Diamond syndrome, which also is knownas autoerythrocyte sensitization disorder, is a raresyndrome of inflammatory, edematous papulesthat evolve into painful ecchymoses on the trunkand lower legs after a period of stress with no priorhistory of trauma. This syndrome usually occurs inwomen with a history of psychiatric disorders, themost common one being depression. Although theexact mechanism of injury is not well understood,it is hypothesized that these patients haveautoantibodies to phosphatidylserine, which is aphospholipid membrane component in erythrocytes.Treatment for this disorder includes symptomatictherapies and psychotropic medications to treat theunderlying psychiatric disorder.

Lichen planus pigmentosus and lichen planopilaris

Lichen planus is an inflammatory skin conditionwith characteristic histopathological findings thathas many clinical variants. Recently, there have been25 cases reported in the literature of lichen planuspigmentosus (LPPi) that preceded the developmentof frontal fibrosing alopecia (FFA), which is a variantof lichen planopilaris (LPP). We review the literatureregarding LPPi, LPP, and FFA and present a case of a40-year-old African American woman with LPPi andLPP.

Sarcoidosis with prominent necrosis on histopathology

Sarcoidosis is a multiorgan inflammatory diseasewith variable clinical presentations and the commonhistopathologic finding of noncaseating granulomas.The etiology of the disease is not known, butevidence suggests both environmental and geneticcontributions to the pathogenesis. Depending onthe severity of cutaneous disease and extent ofextracutaneous involvement, therapies range fromtopical and intralesional glucocorticoids to systemicimmunomodulatory and immunosuppressiveagents. We present the case of a patient withcutaneous sarcoidosis with prominent necrosis onhistopathologic examination in the setting of severepulmonary sarcoidosis.

Frontal fibrosing alopecia in a 46-year-old man

Frontal fibrosing alopecia is a scarring alopecia thatis characterized by recession of the frontotemporalhairline with the frequent loss of eyebrows. Itpredominantly affects postmenopausal womenand only rarely affects men. We report the caseof a 46-year-old man with a ten-year history of anerythematous patch with perifollicular erythemaat the superior aspect of the forehead andfrontotemporal hairline. A skin biopsy specimenshowed a perivascular, lymphocytic infiltrate withperiinfundibular fibrosis. These findings establisheda diagnosis of frontal fibrosing alopecia. Thepathogenesis of this condition is poorly understoodbut may be hormonally-mediated.

A case of perniosis

Perniosis is a localized, inflammatory reaction that ischaracterized by erythematous papules and nodulesthat often are located on the acral surfaces in youngwomen. The lesions of perniosis are thought to bedue to cold-induced vasoconstriction that leadsto hypoxemia and inflammation of the vessel wall.Histopathologic and laboratory studies are indicatedfor patients with suspected perniosis to distinguishbetween idiopathic perniosis and secondaryperniosis. Treatment includes adequate heating andclothing, proper food intake, nifedipine, ultravioletA1 phototherapy, topical glucocorticoids, andvasodilators.

Cutis verticis gyrata

Cutis verticis gyrata that involves only the face isa rare presentation of this even rarer cutaneousanomaly. We present a 61-year-old man, whodeveloped primary essential progressive cutis verticisgyrata of the face.

Diffuse sebaceous-gland hyperplasia

Diffuse sebaceous-gland hyperplasia is a rarevariant of sebaceous-gland hyperplasia that isdistinct from the well-known circumscribed type.The term presenile sebaceous hyperplasia has beenutilized to describe this entity that is distinguishedby specific features, which include confluence oflesions that results in the formation of large plaqueson the face, the sparing of periorificial regions, andhighly functional glandular hyperplasia that resultsin excessive sebaceous secretion. We present a43-year-old woman with monomorphous, skincoloredand yellow, smooth 1- to-3-mm papules,some with central umbilication, that spare theperiorificial zones. Histopathologic examination wassuggestive of diffuse sebaceous-gland hyperplasia.Differential diagnosis of this condition is broad andincludes syndromes that are associated with multiplefacial papules and malignant conditions, such asMuire-Torre syndrome and Cowden syndrome. Itis important to be aware of this condition in orderto consider appropriate treatment options, such asisotretinoin and to avoid unnecessary diagnostictests.

Primary cutaneous marginal-zone lymphoma

Primary cutaneous B cell lymphomas (PCBCL) are thesecond most common type of primary cutaneouslymphoma. The three main types of PCBCL areprimary cutaneous marginal-zone lymphoma(PCMZL), primary cutaneous follicle-centerlymphoma, and primary cutaneous diffuse largeB-cell lymphoma, leg type. PCMZL has an indolentcourse with a five-year survival rate approaching99%. Lesions most often present on the trunk or armas erythematous-to-violaceous papules, plaques, ornodules. Approximately one-half of patients havesolitary skin lesions. Treatment options includesurgery, radiation, and topical, intralesional orsystemic therapy. We present the case of a 33-yearoldHispanic woman with firm, pruritic, pink papuleson the forehead and cheeks, who was diagnosedwith PCMZL.

Eosinophilic dermatosis of hematologic malignancy

We report a 68-year-old woman with chroniclymphocytic leukemia, who developed numerous,pruritic, edematous, and vesicobullous skin lesionsof the face and extremities over the course of severalmonths. The diagnosis of eosinophilic dermatosis ofhematologic malignancy (EDHM) was made basedon the clinical history and histopathologic features.Owing to the possible link between EDHM and amore aggressive underlying CLL, she was startedagain on chemotherapy. This case serves as areminder that, although the precise pathogenesis ofEDHM remains unclear, the paraneoplastic disorderis the result of immune dysregulation. Patientswho develop EDHM should undergo prompthematologic/oncologic evaluation.

Dermatomyositis, clinically presenting with cutaneous ulcers, with histopathologic evidence of perforating collagenosis

Dermatomyositis is a systemic, autoimmune diseasewith a variety of clinical features that often includemyositis and characteristic cutaneous findings. Asubset of patients with dermatomyositis developcutaneous ulcers, often in the setting of vasculitis orvasculopathy. We present a case of dermatomyositiswith cutaneous ulcers that show perforatingcollagenosis on histopathologic examination.Acquired reactive perforating collagenosistypically occurs in the setting of diabetes mellitus,chronic renal failure, and other pruritic conditions,and this case represents a rare association withdermatomyositis, which may ultimately be helpful inelucidating the pathophysiology of this perforatingdisorder.

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The University of California Davis, School of Medicine, Department of Dermatology is recruiting for a full-time position at the Associate or Full Professor level in the Clinical X Series or Health Sciences Clinical Professor (HSCP) Series. The successful candidate is nominated to be the holder of the Frederick G. Novy, Jr. M.D Endowed Professorship. Appointees to the ClinX series are expected to conduct independent research. Both series require significant participation in teaching and University/public service.

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