Fig 1. Pityriasis lichenoides et varioliformis acuta. Viola-ceous papules with central crusting and eschars.

Fig 2. Pityriasis lichenoides et varioliformis acuta. Vacuolarinterface lymphocytic vasculitis (asterisks) with exocytosisof lymphocytes, necrotic keratinocytes (arrows), parakera-tosis, and extravasated erythrocytes. (Hematoxylin-eosinstain; original magnification:3200.)

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hemorrhagic vacuolar interface dermatitis with apredominance of CD8+ cytotoxic/suppressor T cellsand lymphocytic vasculitis (Fig 2). Tissue culturerevealed no infectious organisms. The histopatho-logic differential included PLEVA and Degos disease.In combination with the clinical picture, a diagnosisof PLEVA was made.

Oral tetracycline (1000 mg twice a day) andprednisone (0.5 mg/kg/d) was started in the patient.Tetracycline was discontinued shortly after becauseof shortness of breath. The eruption persisted overseveral months, until the patient vacationed inFlorida and stopped all medications out of frustra-tion. He noted rapid improvement of his skin lesionsand pruritus. He restarted SCIg 1 month later andexperienced recurrence of the necrotic papulareruption by the third infusion, confirming a diagno-sis of SCIg-induced PLEVA. The medication wasdiscontinued and prednisone (0.5 mg/kg/d) wasrestarted along with phototherapy (narrowbandultraviolet B 3 times weekly) with resolution of hissymptoms during the next 3 weeks.

Erythema and rash at the injection site are themost commonly reported adverse cutaneous sideeffects of SCIg. Five (10%) patients in the phase IIIclinical trial reported a rash, but no further descrip-tion was provided.5 Because of the relatively limitedexperience with SCIg, clinicians treating patientswith primary immunodeficiency should be awareof a potential association with PLEVA. Additionalcases are needed to confirm or refute theimmunoglobulin-PLEVA association.

Mac Machan, MD,a Rebecca Loren, BS,b GarthFraga, MD,c and Deede Liu, MDa

University of Kansas Medical Center, Division ofDermatologya and Department of Pathology,c

and University of Kansas School of Medicine,b

Kansas City

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Deede Liu, MD, University ofKansas Medical Center, Division of Dermatology,3901 Rainbow Blvd, MS 2025, Kansas City, KS66160

E-mail: dliu@kumc.edu

REFERENCES

1. Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert

WC. Pityriasis lichenoides et varioliformis acuta: a disease

spectrum. Int J Dermatol 2010;49:257-61.

2. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes.

J Am Acad Dermatol 2006;55:557-72.

3. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophys-

iology, classification, and treatment. Am J Clin Dermatol

2007;8:29-36.

4. Jowkar F, Namazi MR, Bahmani M, Monabati A. Triggering of

pityriasis lichenoides et varioliformis acuta by radiocontrast

iodide. J Dermatolog Treat 2008;19:249-50.

5. Jolles S, Bernatowska E, de Gracia J, Borte M, Cristea V, Peter HH,

et al. Efficacy and safety of Hizentra((R)) in patients with primary

immunodeficiency after a dose-equivalent switch from intrave-

nous or subcutaneous replacement therapy. Clin Immunol

2011;141:90-102.

http://dx.doi.org/10.1016/j.jaad.2011.12.017

Necrotic ulcerations on the back of the handsin a patient with chronic renal failure: Anuncommon presentation of calciphylaxis

To the Editor: A 75-year-old man presented with a3-week history of erosions on the back of both handsthat had progressed to painful ulcerations. There wasno history of trauma. Over the previous few days thepatient reported the appearance of painful ulcera-tions on his penis. Noteworthy in his clinical historywas chronic renal insufficiency secondary to IgAglomerulonephritis treated with hemodialysis over

Fig 1. Calciphylaxis. A, Painful erosions on back of bothhands; no blisters were previously present. Erosionsprogressed to necrotic ulcerated plaques after 3 weekson back of both hands (A) and penis (B).

Fig 2. Calciphylaxis. Calcification of media and subintimalfibroplasia of dermal capillaries and thrombotic occlusionof vessels (hand). (Hematoxylin-eosin stain; original mag-nification: 34.)

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the previous 8 years and two kidney transplants, 24and 21 years before. Comorbidities included para-thyroidectomy, diabetes mellitus, large cell lungcarcinoma treated surgically 9 years previously, andsevere ischemic cardiomyopathy. The patient wasstill on dialysis 5 times per week.

Physical examination revealed necrotic ulcera-tions with well-defined borders on the back of bothhands and penis (Fig 1). Peripheral pulses werepresent.

Laboratory analyses revealed high creatininelevels (6.2 mg/dL), normal blood calcium (8.8mg/dL), and elevated phosphorous (7.1 mg/dL)with a slightly elevated calcium-phosphorus productlevel (62.48 mg2/dL2) and a parathyroid hormonelevel of 223 pg/mL. Cryoglobulins and antiphospho-lipid antibodies were negative.

A skin biopsy was performed on the lesions onthe hand and penis. Histopathologic analysis re-vealed the presence in the dermis and hypodermis ofareas of fibrosis accompanied by extensive ischemicnecrosis with vessel thrombosis. Epidermal denuda-tion and thrombosed vessels with calcifications inthe shape of concentric lamellae were present(Fig 2). Similarly, there were intensely basophilicareas in the dermis consistent with foci of calcifica-tion (Fig 2).

A diagnosis of calciphylaxis was made. Sodiumthiosulfate was started in our patient at a dose of 25 g3 times a week. However, he died a few days later as

a result of multiorgan failure secondary to a respira-tory infection.

Calciphylaxis is a rare calcifying vasculopathy ofsmall vessels leading to ischemia of the skin and softtissues. It occurs mainly in patients with chronicrenal failure. The incidence has been estimated as 1%to 4% of hemodialyzed patients and mortality isbetween 60% and 80%.1 A high percentage (32%-38%) of patients with calciphylaxis have receivedkidney transplants, including normal-functioninggrafts.2

The mechanisms of vasculopathy in calciphylaxisare unclear but established risk factors include renalfailure, kidney transplantation, treatment with sys-temic corticosteroids and vitamin D, and calcium-phosphorus product in excess of 70 mg2/dL2.

The diagnosis is based on histopathologic find-ings and clinical correlation. Clinically the disordermanifests as a painful retiform purpura that pro-gresses to necrotic ulcerations. Histopathologicanalysis usually reveals calcification of the mediaof small vessels of the dermis and subcutaneoustissue, with thrombotic occlusion and extravascularcalcium deposits.3 The differential diagnosis in-cludes pyoderma gangrenosum, vasculitis, heparin-and warfarin-induced skin necrosis, and fungalinfections.

There is no evidence-based medicine treatmentoption available for patients with calciphylaxis.Sodium thiosulfate is an inorganic salt that hasantioxidant and calcium-chelating properties.Several studies have described that more rapidwound granulation may occur with sodium thiosul-fate administration.4,5

Calciphylaxis is commonly found on the lowerextremities and rarely in distal areas. According to acombined MEDLINE and EMBASE search, this is thefirst report of calciphylaxis involving the back of

Fig 1. A, Lichen planus (LP) pemphigoides: punch biopsyspecimen from initial eruption showing epidermal hyper-plasia, hyperkeratosis, Civatte bodies, and bandlike papil-lary dermal lymphocytic infiltrate with vacuolar changeconsistent with LP. B, Punch biopsy specimen from edge ofblister showing subepidermal detachment, necrotic kerat-inocytes, numerous melanophages, and perivascularlymphocytic infiltrate in papillary dermis. (Hematoxylin-eosin stain; original magnifications: A and B, 333.)

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hands without distal limb necrosis. Moreover, penilecalciphylaxis is a very unusual disease. To ourknowledge, only 43 cases of penile calciphylaxishave been identified in the literature.

This case demonstrates the clinical diversity of thisdisease and illustrates an unusual location of calci-phylaxis that caused a significant delay in thediagnosis.

Maider Pretel Irazabal, MD, MPH,a Laura MarquesMartin, MD,a Miguel �Angel Idoate Gastearena,MD, MPH,b Tania Labiano Miravalles, MD,b andNuria Garc�ıa Fern�andez, MD, MPHc

Departments of Dermatology,a Pathology,b andNephrology,c University Clinic of Navarra, Schoolof Medicine, Pamplona, Spain

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Laura Marques Martin, MD,Department of Dermatology, University Clinic ofNavarra, Av. P�ıo XII, n836, 31008, Pamplona,Spain

E-mail: lmarques@unav.es

REFERENCES

1. AngelisM,Wong LL,Myers SA,Wong LM. Calciphylaxis in patients

on hemodialysis: a prevalence study. Surgery 1997;122:1083-9.

2. Budisavljevic MN, Cheek D, Ploth DW. Calciphylaxis in chronic

renal failure. J Am Soc Nephrol 1996;7:978-82.

3. Kuzela DC, Huffer WE, Conger JD, Winter SD, Hammond WS.

Soft tissue calcification in chronic dialysis patients. Am J Pathol

1977;86:403-24.

4. Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in

prevention, diagnosis, and treatment. Semin Dial 2002;15:172-86.

5. Hayden MR, Goldsmith D, Sowers JR, Khanna R. Calciphylaxis:

calcific uremic arteriolopathy and the emerging role of sodium

thiosulfate. Int Urol Nephrol 2008;40:443-51.

http://dx.doi.org/10.1016/j.jaad.2011.12.022

Lichen planus pemphigoides in a 2-year-oldgirl: Response to treatment with methotrexate

To the Editor: Lichen planus (LP) pemphigoides(LPP) is a heterogeneous autoimmune diseasecharacterized by subepidermal blisters occurring inassociation with LP. It is hypothesized that thelichenoid inflammation in LP, in the absence ofadequate immune surveillance, may result in epitopespreading and production of autoantibodies against180-kd bullous pemphigoid (BP) antigen (BP180).1

Pediatric LPP is rare, with only 14 cases documentedin the literature.2-4 We describe a child with thecharacteristic findings of LPP who was successfullytreated with methotrexate. Decreasing levels of

serum anti-BP180 autoantibodies correlated withher clinical improvement.

A 2-year-old girl presented with a 3-week his-tory of intensely pruritic, violaceous scaly plaquesin a widespread distribution. Only her palms,soles, oral mucosa, and nails were not involved.Histopathologic examination of a skin biopsyspecimen confirmed the clinical diagnosis of LP(Fig 1, A). She was treated with triamcinolone 0.1%ointment under occlusion with wet wraps, andwith oral hydroxyzine, but showed minimal im-provement. Two weeks later, she developed nu-merous tense blisters and large erosions within theviolaceous plaques of LP and on previously unin-volved skin (Fig 2, A and B). Biopsy specimenswere obtained for histopathology and direct im-munofluorescence. Histopathologic examinationrevealed a subepidermal blister with a dermalmononuclear infiltrate composed of lymphocytes,plasma cells, and numerous melanophages (Fig 1,B). Direct immunofluorescence showed linear de-position of IgG and complement component 3 at