Numerous asymptomatic papulo-nodules and plaques in a young male :[PAUTHORS], Indian Journal of Dermatology

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Year : 2015 | Volume : 60 | Issue : 1 | Page : 99--100

Numerous asymptomatic papulo-nodules and plaques in a young male

Dipti Das, Anupam Das, Abanti Saha, Swapan Sardar, Kaushik Shome, Ramesh C Gharami
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India

Correspondence Address:
Anupam Das
�DQ�Prerana�DQ�, 19 Phoolbagan, Kolkata - 700 086, West Bengal
India

How to cite this article:
Das D, Das A, Saha A, Sardar S, Shome K, Gharami RC. Numerous asymptomatic papulo-nodules and plaques in a young male.Indian J Dermatol 2015;60:99-100

How to cite this URL:
Das D, Das A, Saha A, Sardar S, Shome K, Gharami RC. Numerous asymptomatic papulo-nodules and plaques in a young male. Indian J Dermatol [serial online] 2015 [cited 2023 Jun 3 ];60:99-100
Available from: https://www.e-ijd.org/text.asp?2015/60/1/99/147823

Full Text

A 16-year-old boy born of non-consanguineous marriage presented with numerous solid elevated lesions over his body. To start with, the lesions were asymptomatic flesh-colored papules over the face 1 year back and gradually, they progressed to attain the present status. Cutaneous examination revealed multiple brown-colored papules, discrete as well as conﬂuent, yellow to brown-colored, well-circumscribed and smooth-surfaced nodules and plaques of varied sizes over face, more concentrated over the eyelids and chin, ears, neck, axillae, elbows, popliteal fossae, scrotum and oral mucosa [Figure 1], [Figure 2], [Figure 3]. Hairs, nails and teeth did not reveal any abnormality. There was no history of seizures, dysphagia, dyspnea, hoarseness of voice, blindness, increased urinary frequency, bone pain or any gastrointestinal complaints. Family history was non-contributory. Systemic examinations including ophthalmologic, oropharyngeal and otorhinolaryngological referral were done and nothing was found to be abnormal. Laboratory investigations including complete blood count, biochemistry panel, lipid profile, thyroid function tests, urinalysis, ECG, chest X-ray, and ultrasonographic studies of the abdomen were within normal limits. A skin biopsy was done from multiple sites. Histopathological examination in all the specimens showed diffuse proliferation of histiocytes, admixed with Touton giant cells, foam cells, scalloped macrophages, lymphocytes and eosinophils in the dermis [Figure 4], [Figure 5], [Figure 6]. Immunohistochemical staining for CD1a was found to be negative, whereas CD 68 and factor XIIIa were positive.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

Question

What is the diagnosis?

View Answer

Answer

Xanthoma disseminatum.

Discussion

Since its frst description by Montgomery and Osterberg in 1938, nearly 100 cases have been reported in the world literature. Xanthoma disseminatum (XD) is a condition in which lipid deposition occurs secondary to a histiocytic proliferation. [1] Age at onset of XD can range from 8 months to 85 years with a defnite gender predilection (male to female ratio of 2:1). Its pathogenesis remains unclear; the non‑neoplastic but pathological non‑X histiocytic cell proliferation reaction pattern of macrophage/monocyte origin is perhaps triggered by some superantigens. [2]

Depending upon its evolution and prognosis, it is grouped into three forms: (1) a self‑healing form with spontaneous resolution; (2) a persistent form (commonest) in which lesions may never resolve; and (3) a very rare progressive form with organ dysfunction and central nervous system involvement. [3] The disorder can be differentiated from eruptive xanthomas, malignant histiocytosis, and Langerhans cell histiocytosis through age at onset,

serum lipoprotein profles, histopathologic fndings, and immunohistochemical results. An early lesion is basically a histiocytic proliferation and the mature lesion is composed of an admixture of histiocytes, foam cells, Touton giant cells, and plenty of infammatory cells within the dermis. This histiocytic proliferation goes in favor of a histiocytic disorder, with Touton giant cells representing an accentuated xanthomatoid reaction, and immunohistochemistry staining positive for CD 68 and factor XIIIa support it being a disorder of histiocytes/ macrophages, whereas negative staining for S‑100, CD1a and Birbeck granules reliably excludes Langerhans cell histiocytosis. [4] In early lesions, scalloped macrophages dominate the histologic picture. In contrast, papular xanthoma and diffuse plane xanthoma show only a minority of nonfoamy cells. More developed lesions may still show scalloped cells, but xanthomatization occurs in most cases. [4,5] In our case, lesions both in early and late stages were present. A table showing the salient points of difference between papular xanthoma, progressive nodular histiocytosis, generalized eruptive histiocytoma and XD has been provided [Table 1].{Table 1}

The treatment modalities have shown variable results. Eisendle et al. reported that oral prednisolone and azathioprine showed no improvement but a combination of lipid‑lowering agents or azathioprine and cyclophosphamide was found to be useful. [6] Kang et al. reported the successful use of a combination of oral steroids, clofbrate, and chemotherapy. [7] Bone marrow transplantation has been used successfully in a case of life‑threatening xanthogranuloma disseminatum in neurofbromatosis type‑1 in a recent report by SavaΊan et al. [8] In 2011, Khezri et al. reported a case series in which 2‑chlorodeoxyadenosine therapy was found useful in maintaining remission and long‑term control of cutaneous lesions, wherein among eight cases of XD, a positive response to treatment with 2‑chlorodeoxyadenosine was seen in fve cases. [9]

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Shraddha Uprety, PGIMER, ChandigarhResham Vasani, Anand Polyclinic, Matunga, MumbaiJimish Bagadia, K.J.Somaiya hospital, Mumbai

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Sarina Jain, Ankan Gupta, Sumit Gupta, Anuj Tenani, Sahil Mrigpuri, Ishad Aggarwal, Gitesh Sawatkar, Sonal Tibrewal, Anup Kumar Tiwary, Dr. Anuradha, Pallavi Jha, Prameela Talakola, Poonam Puri, Sonali Bhat, Swetha Jain, Akhilesh Behra, Manisha thapa , Sourabh Jain, Tirthankar Gayen, Rakesh Kumar Patel, Jaydip Tank, Shruti Alhad Jadhav, Koyani Pooja, Anisha Sethi, Surajit Gorai, Samkit Shah, Vishal Chugh, Somodyuti Chandra, Sumit Sethi, Anirban Das, Riti Bhatia, Vishal Gupta, Katrodiya Nilesh, Ashish Jagati, Mugdha Jhamwar, Sanjay Bosamiya, Dr Anupama, Suneil Pravin Gandhi, Geeti Khullar, Angoori Gnaneshwar Rao, Shamma Aboobacker, Keerthi Subramaniam, Sudhir Nayak U.K., M.Pavan kumar, C.Vijay Krishna, Shiti bose, Priyanka Jain, Suman Patra, Pooja Agarwal, Jasveen Kaur, Anuradha Kakkanatt Babu, Kusuma M R, Vinitha Gopalakrishnan, S.Chidambara Murthy, Khushboo Gupta, Alice eleanor.Y, V.Ramesh

References

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3	Kim JY, Jung HD, Choe YS, Lee WJ, Lee SJ, Kim do W, et al. A case of xanthoma disseminatum accentuating over the eyelids. Ann Dermatol 2010;22:353‑7.
4	Burgdorf WH, Zelger B. The histiocytoses. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Lever's Histopathology of the Skin. 10 ^th ed. Philadelphia: Lippincott Williams and Wilkins; 2009. p. 675.
5	James WD, Berger TG, Elston DM, editors. Andrew's Diseases of the Skin: Clinical Dermatology. 11 ^th ed. Philadelphia, PA: Saunders Elsevier Publications; 2011. p. 710‑11.
6	Eisendle K, Linder D, Ratzinger G, Zelger B, Philipp W, Piza H, et al. Infammation and lipid accumulation in xanthoma disseminatum: Therapeutic considerations. J Am Acad Dermatol 2008;58 Suppl 2:S47‑9.
7	Kang TW, Kim SC. A case of xanthoma disseminatum presenting as pedunculating nodules and plaques. Korean J Dermatol 2007;45:290‑3.
8	Savaºan S, Smith L, Scheer C, Dansey R, Abella E. Successful bone marrow transplantation for life threatening xanthogranuloma disseminatum in neurofbromatosis type‑1. Pediatr Transplant 2005;9:534‑6.
9	Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: Effective therapy with 2‑chlorodeoxyadenosine in a case series. Arch Dermatol 2011;147:459‑64.