Acquired Cutis Laxa of Face with Multiple Myeloma
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Letters to the Editor 6. Takayama K, Satoh T, Hayashi M, Yokozeki H. Psoriatic skin Serum β2‑microglobulin was increased. Bone marrow lesions induced by BCG vaccination. Acta Derm Venereol examination revealed 14% plasma cells. Based on 2008;88:621‑2. 7. Monacelli M. Koebner reaction in psoriasis. In: Farber EM, these findings, a diagnosis of early multiple myeloma Cox AJ, editors. Psoriasis: Proceedings of the International with nephrotic syndrome was made. She was started Symposium. Vol. 99. Stanford: Stanford University press; 1971. p. 104‑11. on a chemotherapy regimen consisting of thalidomide 8. Rambukkana A, Das PK, Witkamp L, Yong S, Meinardi MM, (100 mg/day), oral cyclophosphamide (150 mg/day) Bos JD. Antibodies to mycobacterial 65‑kDa heat shock protein both for the first five days in every month with weekly and other immunodominantantigens in patients with psoriasis. J Invest Dermatol 1993;100:87‑92. intravenous dexamethasone (40 mg/week) along with loop diuretics. In addition, the patient was prescribed Access this article online bortezomib which was discontinued by the patient after two months. Following one year of treatment, the Quick Response Code: Website: www.ijdvl.com anasarca subsided leaving behind loose wrinkled skin all over the body. Over the next one year, the skin over the DOI: 10.4103/0378-6323.140309 extremities and the abdomen reverted back to normal; however, loosening of skin persisted over the face. PMID: ***** Examination revealed a healthy‑appearing woman, who appeared older than her stated age [Figure 1]. She Acquired cutis laxa of face with had loose, sagging skin on the face and neck giving her a hound‑dog facies [Figure 2]. Skin over the ear multiple myeloma lobules was lax. On stretching of the skin over face, no elastic recoil was seen. Her systemic examination was Sir, also normal. Hematoxylin and eosin (H and E) stained Cutis laxa is a rare dermatosis characterized by sections from a skin biopsy from the face showed a diffuse laxity of skin resulting in a prematurely aged mixed cell infiltrate of lymphocytes and occasional appearance. It is most commonly an inherited condition. neutrophils [Figure 3]. A special stain to demonstrate The acquired form is rare and has been associated elastic tissue (Verhoff van Gieson) showed fragmented with various conditions, including multiple myeloma, and clumped elastic fibers [Figure 4]. monoclonal gammopathy of undetermined significance, and heavy chain deposition disease. Cutis laxa usually Based on her clinical features, a diagnosis of acquired follows a progressive course and there is no definitive cutis laxa localized to the face following multiple treatment available.[1] We describe a 44‑year‑old female myeloma was made and she was referred to a plastic with multiple myeloma and cutis laxa localized to surgeon for surgical correction of redundant folds. the face. She developed anasarca, which subsided following treatment for multiple myeloma leaving lax Cutis laxa is a rare condition characterized clinically by and wrinkled skin all over the body. Lax skin improved lax, pendulous skin and histologically by loss of elastic spontaneously elsewhere but persisted on the face. tissue in the dermis. It is a heterogeneous condition, which may be inherited as a dominant, recessive or A 44‑year‑old female presented to us with a two‑year X‑linked recessive disease, or it may be acquired. The history of loosening of skin over the face. Two years face and neck are often affected, which produces a back, she had developed severe anaemia with associated ‘bloodhound’ appearance of premature aging. Mild anasarca and exertional dyspnea. She also had heavy trauma can lead to purpura due to skin fragility and proteinuria, hypoproteinemia and progressive renal this may lead to formation of fibrotic nodules over the failure. A kidney biopsy was performed which revealed bony prominences. There may be widespread, massive mesangial cell proliferation and lobular accentuation folds of lax skin, or the changes may be mild and of glomerular tufts with marked mesangial matrix confined to a limited area. Such cases may be confused deposition and eosinophilic periodic acid Schiff (PAS) as having anetoderma. The acquired cephalic form of positive nodules. Immunofixation electrophoresis on localized cutis laxa presents characteristically as large, a 24‑hour urine sample was positive for kappa light hanging loose ear lobes, blepharochalasis and lax chains. Serum immunofixation electrophoresis (IFE) nasolabial folds. Organs other than the skin may also be revealed a stable monoclonal IgG‑λ M‑spike (0.5 g/dL). involved. Internal organ abnormalities like emphysema, 454 Indian Journal of Dermatology, Venereology, and Leprology | September-October 2014 | Vol 80 | Issue 5 Letters to the Editor Figure 1: Loose sagging folds of skin over the face Figure 2: Loose folds of skin over the neck Figure 3: A sparse infiltrate of lymphocytes and few histiocytes Figure 4: VVG stained sections showing clumped and fragmented are seen in the dermis seen on H and E, (×100) elastic fibers (×400) gastric fibromas and tracheobronchomegaly have been Tulika A. Yadav, Atul M. Dongre, [2] reported. Uday S. Khopkar Department of Dermatology, Seth GS Medical College and King Histologically, the skin is of normal thickness, but Edward Memorial Hospital, Mumbai, Maharashtra, India the elastic fibers are sparse, short, fragmented and clumped, particularly in the upper dermis, and they Address for correspondence: Dr. Tulika A. Yadav, [3] Department of Dermatology, King Edward Memorial show granular degeneration. The elastic fibers Hospital, Parel, Mumbai, Maharashtra, India. are found to be deficient in elastin, though their E‑mail: [email protected] microfibrils appear normal. Similar changes in elastic REFERENCES fibers may occur in the lungs and aorta.[4,5] There are occasional case reports of multiple myeloma and 1. Ferrándiz‑Pulido C, Serra M, Bel S, Ferrer B, Repiso T, other plasma cell dyscrasias associated with cutis Garcia‑Patos V. Multiple myeloma‑associated amyloidosis laxa.[6] presenting with acrolocalized acquired cutis laxa. Arch Dermatol 2010;146:1433‑4. 2. Wanderer AA, Ellis EF, Goltz RW, Cotton EK. It is likely that paraproteinemia resulting in the Tracheobronchomegaly and acquired cutis laxa in a child. deposition of immune complexes possibly leads to Physiologic and immunologic studies. Pediatrics 1969;44:709‑15. 3. Hashimoto K, Kanzaki T. Cutis laxa. Ultrastructural and the release of inflammatory cytokines and subsequent biochemical studies. Arch Dermatol 1975;111:861‑73. destruction of elastic fibers. Surgical repair seems 4. Marchase P, Holbrook K, Pinnell SR. A familial cutis laxa syndrome with ultrastructural abnormalities of collagen and to be the only therapeutic choice, but the results are elastin. J Invest Dermatol 1980;75:399‑403. variable and temporary. 5. Sephel GC, Byers PH, Holbrook KA, Davidson JM. Heterogeneity Indian Journal of Dermatology, Venereology, and Leprology | September-October 2014 | Vol 80 | Issue 5 455 Letters to the Editor of elastin expression in cutis laxa fibroblast strains. J Invest 0.05% cream, applied daily for the first month Dermatol 1989;93:147‑53. 6. New HD, Callen JP. Generalized acquired cutis laxa associated and then on alternate days. After 5 months, since with multiple myeloma with biphenotypic IgG‑ λ and IgA‑κ the patient no longer wished to continue with the gammopathy following treatment of a nodal plasmacytoma. cream, administration of monthly triamcinolone Arch Dermatol 2011;147:323‑8. acetonide (40 mg/mL) injections into the nodular lesions was started. Three months later, the plaques Access this article online had slightly regressed and the papulonodular lesions Quick Response Code: Website: were smaller [Figure 3]. The patient continued to www.ijdvl.com take pentoxyphylline tablets for another 10 months. DOI: Meanwhile, she was referred to a dietician and a 10.4103/0378-6323.140310 low‑calorie diet was recommended; however, she PMID: only lost 3‑4 kg. After 10 months of therapy, there ***** was no significant regression of the lesions so further treatment was stopped; the patient is still following up Obesity‑associated with us. lymphedematous mucinosis Mucin accumulation on the legs is usually considered an indicator of pretibial myxedema.[3] In 1993, Somach et al. reported that pretibial myxedema in euthyroid Sir, patients may be histologically different from the Obesity‑associated lymphedematous mucinosis is pretibial myxedema of hyperthyroidism.[2] Then in a newly recognised entity with only a few patients 2006, Tokuda et al. reported three cases of “chronic reported to date. Though this condition can clinically obesity lymphedematous mucinosis,”[4] where mucin mimic pretibial myxedema, it is differentiated from accumulation on the legs accompanying lymphedema the latter by microscopic findings and a lack of thyroid had histological findings similar to those reported by disease.[1,2] Somach et al. Most recently, in 2009, Rongioletti et al. reported five cases of obesity‑associated lymphedema A 71‑year‑old female presented to us with swelling, with mucin accumulation on the legs. They renamed erythema, and discoloration on both her legs; these this entity “obesity‑associated lymphedematous symptoms had been present for 1 year. She had been mucinosis.”[3] The pathogenesis of this condition overweight for 30 years (height 160 cm, weight 95 kg, is unclear but a lymphatic drainage defect may be and body mass index 37.1). Dermatologic examination a cause,[5] leading to excessive high‑protein fluid revealed pink‑red plaques on both legs, especially collecting in the interstitium. This in turn could the left leg. Several circular, semi‑translucent, cause thickening of the legs and a peau d’orange papulo‑nodular lesions of 1‑2 cm diameter were noted appearance.[2] surmounting these plaques [Figure 1a and b]. Clinically, obesity‑associated mucinosis is Blood tests including total protein, immunoglobulin characterized by skin‑colored or brown‑red papules levels, and thyroid function tests were all normal. and/or nodules on an erythematous base on the No cardiac or renal failure was detected.