analysis revealed positivity for neuroendocrine markers:synaptophysin (Fig. 1c) and chromogranin. Thus, thediagnosis of carcinoid tumour was established. Thepatient received chemotherapy with four cycles of cisp-latin and etoposide. Erythema nodosum disappearedshortly after the first cycle. The follow-up abdominalCT carried out 5 months after chemotherapy showedpartial response. No recurrence of erythema nodosumhas been found 13 months after chemotherapy.

The association of erythema nodosum with carcinoidtumour has never been reported. Malignancy and ery-thema nodosum has been described with lymphoma,1

acute myelocytic leukaemia,2 cervical carcinoma3 andmetastatic pancreatic carcinoma.4 The dermatosis typic-ally runs a parallel course with the cancer, similar to thefindings in our case. In addition, the increased levels ofLDH and CRP in our patient probably reflected theunderlying tumour burden and the inflammatory process,respectively.

Paraneoplastic dermatoses or immunological diseasessuch as sarcoidosis,5 neurofibromatosis,6 pemphigus7 andmembranous glomerulonephropathy8 are associated withmalignant carcinoid tumour. The underlying mechanismof these cases is unclear but is most likely due to thedeposition of immune complexes. The autoimmune glome-rulonephritis that occurs with carcinoid tumour typicallyresolves after the resolution of the carcinoid tumour.8

Interestingly, a similar mechanism was also suggestedrecently in patients with erythema nodosum and Hodgkin’sdisease.1 The mechanism underlying the association oferythema nodosum with carcinoid tumour may alsoinvolve paraneoplastic mediators secreted by tumour tissueor immune dysregulation.9 Carcinoid tumour associatedwith erythema nodosum might be rare, but nevertheless itshould be considered in the spectrum of malignanciesassociated with erythema nodosum.

J.-T. Lin, P.-M. Chen, D.-F. Huang,* W.-K Kwang,† K. Lo‡

and W.-S. Wang

Divisions of Medical Oncology, *Immunology and Rheumatology,

Department of Medicine and Departments of �Pathology and

�Dermatology; Taipei Veterans General Hospital and National

Yang-Ming University School of Medicine, 201, Sec. 2,

Shi-Pai Road, Shi-Pai, Taipei, Taiwan.

Accepted for publication 3 October 2003

References

1 Bonci A, Di Lernia V, Merli F et al. Erythema nodosum

and Hodgkin’s disease. Clin Exp Dermatol 2001; 26:

408–11.

2 Thwe M. Acute myeloblastic leukaemia presenting with

erythema nodosum. Postgrad Med J 1966; 42: 1–53.

3 Altomare GF, Capella GL. Paraneoplastic erythema nodo-

sum in a patient with carcinoma of the uterine cervix. Br J

Dermatol 1995; 132: 667–8.

4 Durden FM, Variyam E, Chren MM. Fat necrosis with

features of erythema nodosum in a patient with meta-

static pancreatic carcinoma. Int J Dermatol 1996; 35:

39–41.

5 Weltfriend S, Harth Y, Katz I. Subcutaneous sarcoidosis in a

patient with malignant carcinoid tumor of the colon. J Am

Acad Dermatol 1989; 20: 507–86.

6 Dawson BV, Kazama R, Paplanus SH. Association of carci-

noid with neurofibromatosis. South Med J 1984; 77: 511–3.

7 Shirahama S, Nham NX, Yagi H et al. Pemphigus associated

with metastatic carcinoid. Dermatology 1994; 189 (Suppl.

1): 97–8.

8 Ashman N, Steele JP, Sheaff M et al. Membranous nephro-

pathy resolving with treatment of bronchial carcinoid

tumor. J Am J Kidney Dis 2001; 36: E15.

9 Naschitz JE, Rosner I, Rosenbaum M et al. Rheumatic

syndromes: clues to occult neoplasia. Sem Arthritis Rheum

1999; 29: 43–5.

Acute lipodermatosclerosis in a 15-year-old girl?

The pathogenesis of lipodermatosclerosis (LDS) has notbeen clearly elucidated. While a strong association withvenous insufficiency is well established, other mechanismsare almost certainly involved.1 Chronic LDS presents withindurated, typically nonpainful and nontender plaques andvariably hyperpigmented skin, located predominantly onthe medial aspect of the lower leg. Because of thesubcutaneous fibrosis and scarring of LDS, the mid-portionof the leg may become constricted, bound-down, andnarrowed, giving the �inverted bottle� appearance.2,3 AcuteLDS also presents as plaques over the medial aspect of theleg, and is generally inflamed and painful.1 The painful redplaques are frequently mistaken for cellulitis. The degree ofvenous insufficiency necessary for the development ofacute LDS may be mild at first, although most of thesepatients do exhibit more extensive venous disease later intheir clinical course.1,4 Acute LDS can also be a feature ofchronic lymphoedema.5 Previous case studies of thiscondition describe middle age to be the commonest ageof presentation, although it has been described in elderlywomen. The authors of this report have been unable tofind any report of LDS presenting in a teenager.2 Anotherwise healthy 15-year-old girl presented to the Divi-sion of Dermatology, Groote Schuur Hospital, Cape Town,South Africa in November 2002 with pain, skin changesand swelling of both lower legs. The symptoms had beenpresent for 3 years. She had also experienced episodes ofmore severe pain and redness and had been treated forpresumed cellulitis several times at the local clinic withmild temporary benefit. On examination, the lower legswere found to be diffusely tender and indurated, this beingmost marked over the medial aspects of both ankles.Proximally, there was an �active� red, indurated border,

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showing a sharp cut-off from the normal skin. Both legsshowed marked oedema and the Stemmer sign was positive(thickened cutaneous fold of all toes)6 (Fig. 1a). There wereno well-defined individual nodules or areas of ulcerationand there was no evidence of cellulitis, phlebitis, morphoeaor other forms of panniculitis. Our clinical diagnosis wasacute LDS. General examination was normal and therewere no findings to suggest other skin, vascular orsystemic disorders. A deep incisional biopsy was performedfrom the acutely inflamed red upper border of the affectedarea. The histological sample showed a mononuclearinflammation in the subcutaneous tissue with sparseneutrophils within the lobules of adipose tissue. Therewas no septal involvement. No granulomas or vasculitiswas seen. The findings were compatible with early LDS.Repeat biopsy, of a more established area, was notperformed, for fear of poor wound healing in this setting.Duplex sonography showed the presence of a normalsuperficial vein system, with no deep vein thrombosis.Dynamic lymphoscintigraphy was performed with injec-tion of technetium 99m nanocolloid into the first webspace of both feet. Patent lymphatic channels weredemonstrated on both sides, with the right appearing tobe normal and the left showing a more rapid clearance,suggestive of an enhanced pattern on the left leg. Thepatient was advised to exercise and to use a firmcompression bandage. After 5 weeks, she showed markedimprovement. The oedema of both legs had resolvedcompletely and the indurated areas were softer and largelynontender (Fig. 1b).

The diagnosis of LDS is largely based on clinical findings,with compatible histology. Initially the young age of thispatient caused a great deal of discussion, however, theclinical appearance was compelling and it is difficult toentertain any other diagnosis. Her clinical presentationwas characteristic of acute LDS and the histologicalfindings were compatible. The histological evidence mighthave been stronger if we had taken the biopsy from a morelong-standing area of inflammation, as only the chronicform shows the characteristic sclerosis with pericapillaryfibrin cuffing, increased number of capillaries, collagenproduction and lymphocytes in the septa.2 Acute lobularpanniculitis resembling LDS was considered in our differ-ential diagnosis, but there were no features to support thisand the condition had been present for 3 years at the timeof presentation. Other forms of panniculitis consideredwere erythema induratum and erythema nodosum. Therewere no discrete nodules and there was no ulceration orevidence of any underlying disorder. Recommended treat-

Figure 1 (a) Hyperpigmented indurated skin over the lower leg,

with oedema. The lesions are more prominent and bound-down

over the medial aspects of the legs. (b) After 5 weeks of compres-

sion the oedema and erythema has resolved, leaving shiny, firm,

hyperpigmented areas.

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ment for LDS has included elastic compression and,particularly in the acute phase, stanozolol.4,7 Our patient’scondition improved rapidly, with the introduction ofcompression and exercise. LDS is usually associated withchronic venous insufficiency1,2 and the people com-monly affected are middle-aged to elderly, predominantlywomen.2 We have been unable to find any previous reportof LDS occurring in a 15-year-old. However, the clinicalfeatures presented in this case do suggest this diagnosis.What is the underlying cause of the patient’s LDS? It isunusual in this age group to find an underlying venousinsufficiency and this was supported by our investigations:the duplex-Doppler showed a functioning deep venoussystem and no venous abnormalities. However, this doesnot exclude the possibility of a subtle abnormality in eitherthe venous or the fibrinolytic systems. One possible causefor acute LDS in this age group could be chroniclymphoedema.5 Lymphoedema does remain a possibleexplanation even in the presence of normal lymphoscin-tigraphy, since the false negative rate is > 10% inlymphoedema.7,8 Lymphoedema can be primary orsecondary.9 Our patient had tender hyperpigmented indu-ration of the lower legs, an onset of oedema at the age of12 years, and no history of trauma or lymphangitis. Shecould belong to the Meige type of hereditary lymphoede-ma9 although her marked improvement with conventionalcompression renders this diagnosis unlikely. Acute pan-niculitis resembling LDS was also considered in ourdifferential diagnosis, but there were no features to supportthis and the condition had been present for 3 years atpresentation. The clinical findings were not those oferythema induratum or erythema nodosum. Her promptresponse to compression, elevation and exercise wasfurther supportive evidence of a problem of underlyingvenous insufficiency.

K. Hartmann and S. Jessop

Division of Dermatology, Department of Medicine, Groote Schuur

Hospital and University of Cape Town, Cape Town, South Africa

Accepted for publication 19 November 2003

References

1 Greenberg AS, Hasan A, Montalvo M et al. Acute lipoder-

matosclerosis is associated with venous insufficiency. J Am

Acad Dermatol 1996; 35: 566–8.

2 Kirsner RS, Pardes JB, Eaglstein WH et al. The clinical

spectrum of lipodermatosclerosis. J Am Acad Dermatol 1993;

28: 623–7.

3 Falanga V. Venous ulceration. J Dermatol Surg Oncol 1993;

19: 764–71.

4 Helfman T, Falanga V. Stanozolol as a novel therapeutic

agent in dermatology. J Am Acad Dermatol 1995; 33:

254–8.

5 Gniadecka M. Localization of dermal edema in lipoder-

matosclerosis, lymphedema, and cardiac insufficiency.

High-frequency ultrasound examination of intradermal

echogenicity. J Am Acad Dermatol 1996; 35: 37–41.

6 Stemmer R. Stemmer’s sign – possibilities and limits of

clinical diagnosis of lymphoedema. Wien Med Wochenschr

1999; 149: 85–6.

7 Burnand K, Clemenson G, Mortland M et al. Venous lipo-

dermatosclerosis: treatment by fibrinolytic enhancement

and elastic compression. Br Med J 1980; 280: 7–11.

8 McMullin GM, Watkin GT, Coleridge Smith PD et al. Efficacy

of fibrinolytic enhancement with stanozolol in the treatment

of venous insufficiency. Aust N Z J Surg 1991; 61: 306–9.

9 Carpentier PH. Physiopathology of Lymphedema. Rev Med

Interne 2002; 23 (Suppl. 3): 371–4.

Acrokeratosis paraneoplastica (Bazex syndrome)associated with breast cancer

A 62-year-old Iranian woman presented with an 8-month-history of pruritic red skin lesions on the hands and feet.She had a past medical history of hypertension, cholecys-tectomy for gallstones, hypertriglyceridaemia and osteo-arthritis. Her medications included diclofenac sodium andmethyldopa. She had been treated with topical betameth-asone valerate, but without benefit. Examination revealedseveral well-defined slightly erythematous psoriasiformplaques involving the central portion and medial borderof the soles (Fig. 1). Neighbouring plaques had becomeconfluent in the centre of the affected areas. There weresimilar plaques on the palmar surface of some fingers. Thedorsum of the hands showed nonscaling slightly redpapules resembling granuloma annulare, and some sharplydefined flesh-coloured papules with a rough surfaceresembling seborrhoeic keratoses. The palms, dorsa of thefeet, nails, nail folds, mucous membranes and other parts of

Figure 1 Erythematous psoriasiform plaques involving the central

portion and medial border of the sole.

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