884
Our case is particularly interesting because the patient showed
a rapid multiorgan involvement. Peculiar characteristics of
IVLBCL result in tumour cells involving all types of organs,
mainly bone marrow, the central nervous system and skin, with
insult to organs resulting from transient or permanent vascular
occlusion.4 Even if the lymphoma is usually disseminated exten-
sively at presentation and the overall mortality rate is thought to
be > 80%,5 in case of cutaneous involvement, such as our own,
skin biopsy is a quick, minimally invasive and highly informative
procedure. However, it is not possible to treat all cases ante-mor-
tem. Studies suggest that IVLBCL responds remarkably well to
rituximab-containing chemotherapy (R-CHOP).4,6 If possible,
also high-dose chemotherapy with autologous hematopoietic
stem cell rescue or aggressive combined therapy with high doses
of methotrexate should be considered.6 However, in our case, the
rapid course and aggressiveness did not allow for any treatments.
Funding source
The authors declare that there are no financial or personal
relationships that could inappropriately influence (or bias) the
author’s decisions, work, or manuscript.
Acknowledgements
None.
L. Feci,1* M. Pellegrino,1 V. Mancini,2 P. Taddeucci,1
E. Trovato,1 C. Miracco,2 M. Fimiani1
1
Dermatology Section, Department of Clinical Medicine and Immunology,
Siena University, 2Pathological Anatomy Section, Department of Human
Pathology and Oncology, University of Siena, Siena, Italy
*Correspondence: L. Feci. E-mail: bobys@libero.it
References
1 Zuckerman D, Seliem R, Hochberg E. Intravascular lymphoma: the
oncologist’s “great imitator”. Oncologist 2006; 11: 496–502.
2 Nakamura S, Ponzoni M, Campo E. Intravascular large B-cell lym-
phoma. In: Swerdlow SH, Campo E, Harris NL et al., eds. WHO classifi-
cation of tumours of haematopoietic and lymphoid tissues. IARC Press,
Lyon, France, 2008: 252–253.
3 Kong YY, Dai B, Sheng WQ et al. Intravascular large B-cell
lymphoma with cutaneous manifestations: a clinicopathologic,
immunophenotypic and molecular study of three cases. J Cutan
Pathol 2009; 36: 865–870.
4 Ferreri AJ, Campo E, Seymour JF et al. Intravascular lymphoma: clinical
presentation, natural history, management and prognostic factors in a
series of 38 cases, with special emphasis on the ‘cutaneous variant’. Br J
Haematol 2004; 127: 173–183.
5 Murase T, Yamaguchi M, Suzuki R et al. Intravascular large B-cell lym-
phoma (IVLBCL): a clinicopathologic study of 96 cases with special ref-
erence to the immuno phenotypic heterogeneity of CD5. Blood 2007;
109: 478–485.
6 Shimada K, Kinoshita T, Naoe T et al. Presentation and management of
intravascular large B-cell lymphoma. Lancet Oncol 2009; 10: 895–902.
DOI: 10.1111/jdv.13047
JEADV 2016, 30, 852–909
Letters to the Editor
Successful treatment of lichen
amyloidosus with oral alitretinoin
Editor
Lichen amyloidosus and macular amyloidosis are distinct mani-
festations of primary cutaneous amyloidosis. Skin lesions are
typically accompanied by severe itching. Amyloid deposits in the
papillary dermis are the typical histologic feature and diagnostic
of the disease.
No evidence-based standard treatment is available up to now.
A variety of different treatment options such as topical corticos-
teroids, dimethyl sulfoxide (DMSO), acitretin, dermabrasion or
ablative CO2 laser have been reported.1 Here, we describe a
marked and durable improvement of clinical symptoms in a
patient with long-lasting and recalcitrant lichen amyloidosus
upon treatment with oral alitretinoin.
A 66-year-old patient presented with pruritic papules on his
arms, back and legs. His skin was notably dry. Skin biopsies
taken during the course of the disease recurrently showed acan-
thosis with focal hypergranulosis and mild papillomatosis.
Eosinophilic material was found in the upper dermis, which
showed affinity to the pan-cytokeratin marker MNF-116. The
histologic analysis of active skin lesions confirmed the clinical
diagnosis of lichen amyloidosis (Fig. 1). A large spectrum of
treatments such as topical steroids, 0.1% tacrolimus ointment,
emollients, PUVA therapy and DMSO showed limited success.
We finally decided to try oral retinoids. Since we were concerned
that acitretin or etretinate would exacerbate the patient’s sebo-
stasis and xerosis, we decided to start oral alitretinoin at 30 mg
per day. The pruritus was effectively improved and the hyperker-
atotic papules flattened within one month on therapy (Fig. 2a
and b). Oral alitretinoin was initially given for six
subsequent months before the patient stopped the treatment
self-dependently. The disease remained stable over the next
4 months, but deteriorated in the follow-up. Re-implementation
of alitretinoin 30 mg per day proved to be effective in both
improving the pruritus and the skin lesions.
Cutaneous amyloidosis has been divided into two forms: mac-
ular amyloidosis and lichen amyloidosus, but also indeterminate
variants have been described. Macular amyloidosis occurs as hy-
perpigmented patches or plaques mainly on the back, whereas
lichen amyloidosis typically appears as firm or hyperkeratotic
papules on the extremities, specifically the legs. In both types,
lesions are extremely pruritic. The amyloid deposits consist
mainly of cytokeratin 5 originating from adjacent basal keratino-
cytes and are located predominantly in the papillary dermis.2
Several reports describe good responses to treatment with ret-
inoids such as acitretin and etretinate.3–5 Most retinoids, how-
ever, lead to dryness of the skin, which may eventually
deteriorate the itch. Therefore, we decided to use alitretinoin
(9-cis-retinoid acid). It is a retinoid receptor pan-agonist with
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor 885

(a) (b)

Figure 1 Amyloid deposits in the papillary

dermis. (a) Haematoxylin and eosin stain. (b)
Pan-cytokeratin staining with MNF-116,
which targets a variety of cytokeratins.

(a) (b)

Figure 2 The status of the skin of the

patient before and after treatment with
alitretinoin is shown.

the capacity to bind all six known retinoid receptors. In contrast
to isotretinoin or acitretin, it exerts strong anti-inflammatory
and immune-modulatory effects without suppressing the activity
of the sebaceous glands. It is used mainly for the treatment of
chronic hand eczema, leading to durable remissions. Additional
clinical efficacy has been reported in a variety of other cutaneous
diseases such as ichthyosis or Darier’s disease.6,7 In the case pre-
sented here, alitretinoin showed rapid and robust activity in
treating the papules without aggravating the xerosis.
Taken together, we identify alitretinoin as a novel effective
treatment option for cutaneous amyloidosis, specifically in
patients with strong pruritus and sebostatic skin conditions.
4 Choi JY, Sippe J, Lee S. Acitretin for lichen amyloidosus. Australas J
Dermatol 2008; 49: 109–113.
5 Ozcan A, Senol M, Aydin NE, Karaca S. Amyloidosis cutis dyschromica:
a case treated with acitretin. J Dermatol 2005; 32: 474–477.
6 Lynde C, Cambazard F, Ruzicka T et al. Extended treatment with oral
alitretinoin for patients with chronic hand eczema not fully responding
to initial treatment. Clin Exp Dermatol 2012; 37: 712–717.
7 Ruzicka T, Lynde CW, Jemec GB et al. Efficacy and safety of oral alitre-
tinoin (9-cis retinoic acid) in patients with severe chronic hand eczema
refractory to topical corticosteroids: results of a randomized, double-
blind, placebo-controlled, multicentre trial. Br J Dermatol 2008; 158:
808–817.
DOI: 10.1111/jdv.13048

J. K. Tietze,* M. V. Heppt, M. J. Flaig, P. Thomas

Department of Dermatology and Allergy, Ludwig-Maximilian University,
Munich, Germany
*Correspondence: J. K. Tietze. E-mail: julia.tietze@med.uni-muenchen.
de
An old lady with Pediculosis
pubis on the head hair
References
1 Schreml S, Szeimies RM, Landthaler M, Babilas P. Cutaneous amyloido-
Editor
sis. Hautarzt 2011; 62: 56–61. Three species of lice are specific ectoparasites for human, Pedicu-
2 Apaydin R, Gurbuz Y, Bayramgurler D, Muezzinoglu B, Bilen N. Cyto- lus humanus capitis (head lice), P. humanus corporis (body lice)
keratin expression in lichen amyloidosus and macular amyloidosis. J and Phtirus pubis (pubic lice). The head louse lives mainly on
Eur Acad Dermatol Venereol 2004; 18: 305–309.
3 Carlesimo M, Narcisi A, Orsini D et al. A case of lichen amyloidosus
the human scalp. It has never demonstrated a real vectorial
treated with acitretin. Clin Ter 2011; 162: e59–e61. role.1,2 The body louse is the primary vector of the bacterial

JEADV 2016, 30, 852–909 © 2015 European Academy of Dermatology and Venereology