Cutaneous B-cell lymphoma associated with follicular mucinosis

BRIEF COMMUNICATIONS

Cutaneous B-cell lymphoma associated with follicular mucinosis

H. Bench&hi, MD,a J. Wechsler, MD,b L. Rethers, MD,b F. Aubry, MD,a A. Bouzouita, MD,a J. P. Farcet, MD, PhD,b J. Revuz, MD,” and M. Bagot, MD, PhDa Cn?teiZ, France

Follicular mucinosis is defined by the accumula- tion of a mutinous material in hair follicles. It was initially described by Pink& in 1957, under the ti- tle “alopecia mucinosa.” Association of follicular mucinosis with a cutaneous lymphoma is well known, but in all reported cases it was a cutaneous T-cell lymphoma. 2-9 We report the first case of cu- taneous B-cell lymphoma associated with follicular mucinosis.

CASE REPORT

A 73-year-old woman had a l-year history of slightly prnritic lesions of the face and limbs. She had sharply de- marcated infiltrated plaques with prominent follicular or- ifices on the face and upper and lower extremities (Fig.

1). Alopecia of the right eyebrow was present. She also had numerous papules and nodules on the sides of the neck and arms. No adenopathy was found, the liver and spleen were of normal size.

Laboratory findings showed a leukocyte count of 730044 lymphocyte count of 1600/& and hemoglobin of 11.9 gm/lOO ml. Findings of computed tomographic scans of the tmnk and abdomen were normal. HIV and HTLV-I serologies were negative.

Biopsy specimens from the face and arms showed dif- fuse lymphoid infiltration within the reticular dermis, with a few cells in the papillary dermis and epidermis. The in- filtrate was composed predominantly of atypical small lymphocytes (Fig. 2, A). Some lymphoid infiltrates were seen around hair follicles. The pilar sheaths were disso- ciated by clear spaces that stained with alcian blue (Fig.

2, B).

Immunostaining analysis showed a mixed B- and T-cell infiltration. The B cells predominated in the deep part of the infiltration, whereas the T-cell population was

From the Departments of Dermatologya and Pathology, H6pital Henri Mondor.

Reprint requests: M. Bagot, H6pital Henri Mondor, 51 av du Markhal de Lattre de Tassigny, 94010 CEteil, France.

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Fig. 1. Erythematosquamous infiltrated plaques in cen- trofacial region and orbital arches.

predominant beneath the epidermis and within sebaceous follicles. T cells showed no lack of antigens. B cells ex- pressed CD20 and both light chains, with

^K

predomi- nance.

Gene rearrangement studies by polymerase chain reaction demonstrated a clonal rearrangement of immu- noglobulin heavy chain genes, clearly showing a B-cell clone. The T-cell receptor y gene was in a germinal po- sition. A bone marrow specimen showed a B-cell CD20+

lymphoid infiltration. In the blood, immnnophenotyping showed a predominance of K-positive lymphocytes. A B-cell clone was found by gene rearrangement analysis.

The patient was treated with oral cyclophosphamide (100 mg/day), which induced progressive clearing of the lesions.

DISCUSSION

We report the first case of follicular mucinosis associated with a cutaneous B-cell lymphoma. In our

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674 Brief communications

Journal of the American Academy of Dermatology October 1995

Fig. 2. A, Photomicrograph of cutaneous B-cell lymphoma; note nonepidertnotropic massive diffuse lymphoid infiltration. ^B, Follicular mucinosis. Staining with al&m blue in the pilar sheath. (A, x100; B, x200.)

patient, the clinical presentation resembled a cuta- neous T-cell lymphoma. However, this lymphoma was clearly a B-cell type.

Follicular mucinosis is defined by the accumula- tion of a mutinous material in the epithelial hair fol- licle sheath.lO> I1 Epithelial mucin secretion has been suggested to be induced by cytokines produced by T lymphocytes. l2 In the present case, the altered hair follicles were infiltrated by T cells. The possibility of coexistence of a cutaneous T-cell lymphoma was raised but excluded by the absence of a T-cell clone in the skin. Because reactive T cells are frequently found in primary cutaneous B-cell lymphoma, we consider this T-cell hair follicle infiltration to be re- active.t3

A cutaneous lymphoma is found in association with follicular mucinosis in 9% to 67% of the cases.3, 4, 6-9y l1 Most are T-cell lymphomas of the mycosis fungoides type and, exceptionally, Hodg- kin’s disease or chronic lymphocytic leukemia?, 6, * However, in most cases, only histologic studies have been performed. This suggests that the diagnosis of cutaneous B-cell lymphoma could have been missed.

For example, Mehregan et al? reported 33 cases of follicular muciuosis to be associated with mycosis fungoides. They performed gene rearrangement analysis in only two cases in which they found

a T-cell clone. Follicular mucinosis may be uncom- monly associated with lymphomas other than the T- cell type. In a study of 59 cases of follicular muci- nosis by Gibson et a1.,8 Hodgkin’s disease developed in two patients in the year after the diagnosis.

Mehregan et al? reported one case of Hodgkin’s disease, one case of chronic lymphocytic leukemia, and a third case of an undefined lymphoproliferative disorder. Ramon et al.14 reported two cases of Hodg- kin’s disease in association with follicular mucino- sis. A case of follicular mucinosis has also been re- ported in association with a Grawitz tumor.15 REFERENCES

1. Pinkus H. Alopecia mucinosa: inflammatory plaques with alopecia characterized by root-sheath mucinosis. Arch Dermatol 1957;76:419-26.

2. Braun-Falco 0. Mucophanerosis intrafollicularis et se- boglandularis. Dermatol Wochenschr 1954;136:1289.

3. Pinkus H. The relationship of alopecia muclnosa to malig- nant lymphoma. Dermatologica 1964;129:266-70.

4. Degos R, Beuve-Mery M. La mu&rose folliculaire et ses rapports avec les hemoreticulopathies malignes. G Ital Derm 1966;107:663-74.

5. Plomick H, Abrecht M. Alopecia mucinosa and lym- phoma. Arch Dermatol 1965;92: 137-41.

6. Emmerson RW. Follicular mucinosis: a study of 47 patients. Br J Dermatol 1969;81:395-414.

7. Kanno S, Niia K, Machida S, et al. Follicular mucino-

sis developing into cutaneous lymphoma. Acta Dermatol

Venereol (Stockh) 1984;64:86-8.

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8. Gibson LE, Muller SA, Leiferman KM, et al. Follicular mucinosis: clinical and histopathologic study. J AM ACAD DE%MATOL 1989;20:441-6.

9. Mehregan AD, Gibson EL, Muller AS. Follicular mucino- sis: histopathologic review of 33 cases. Mayo Clin Pmc 1991; 66:387-90.

10. Rongioletti F, Rebora A. Les mucinoses cutankes. Ann Dermatol Venereol 1993;120:7S-87.

11. HaberH.Follicularmucinosis.BrJDermatol1961;73:313- 22.

12. Sabourin JC, Wechsler J, Bagot M, et al. La mucinose fol- liculaire: entiti dermatologique frkquemment associke Bun lymphome T. Ann Path01 1993;13:29-31.

13. Ramsay DA, Smith WJ, Isaacson GP. T-cell-rich B-cell lymphoma. Am J Surg Path01 1988;12:433-43.

14. Ramon D, Jorda E, Molina I. Follicular mucinosis and Hodgkin’s disease. Iot J Dermatol 1992;31:791-2.

15. Derancourt C, Blanc D, Agache P. Follicular mucinosis associated with a Grawitz tumor. Clim Exp Dermatoll993;

l&391.

Lichenoid eruption associated with ethambutol

Marc E. Grossman, MD, Kelly Warren, BA, Attila Mady, MD, and Karin H. Satra, MD New York, New York

Ethambutol is generally well tolerated, adverse reactions have been reported in fewer than 2% of patients. The most common side effect is dose- related optic neuritis, which is usually reversible af- ter discontinuation of the medication.’ Cutaneous side effects are unusual, and there is only one report of a lichenoid drug eruption (LDE) caused by ethambutol.2 We report an additional case of etham- butol-induced LDE restricted to light-exposed areas that resolved after withdrawal of the drug.

CASE REPORT

A 67-year-old white man with a previous medical bis- tory of childhood tuberculosis, a long smoking history with severe emphysema, and multiple episodes of pneu- monia was found to have acid-fast bacilli in his sputum.

He was initially treated with isoniazid and rifampin, but cultures of his sputum continued to be positive for acid- fast bacilli. Fyrazinamide and ethambutol were added.

Fig. 1. Lichenoid eruption on patient’s forearm.

obscuring the dermoepidermal border, and an infiitrate around the deep vessels. Eosinopbils were not observed (Fig. 2).

Three months later, nearly confluent erytbematous to violaceous lichenoid pap&s developed on the forearms and hands (Fig. 1). The eruption was in an apparent pho- todistribution. A skin biopsy specimen was consistent with a lichenoid drug eruption and demonstrated scattered necrotic keratinocytes in the epidermis, a superficial bandlike infiltrate of predominantly mononuclear cells

From the Department of Dermatology, College of Physicians and Sur- geons of Columbia University.

Reprint requests: Marc E. Grossman, MD, FACT, Department of Der- matology, Columbia-Presbyterian Medical Center, 630 W. 168th St., New York, NY 10032.

J AM ACAD DERMATOL 1995;33:675-6.

Copyright 0 1995 by the American Academy of Dermatology, Inc.

0190-9622J95 $5.00 + 0 16/5'4/65618

The patient’s medications included etbambutol, pyra- zinamide, rifampin, isoniazid, vitamin Bg, verapamil, digoxin, prednisone, tbeophylline, heparin, diphenby- dramine hydrochloride, ranitidine, Kaopectate, haloperi- dol, thiamine, nystatin, docusate sodium (Colace), al- buterol inhaler, propine and pilocarpine eyedrops, acet- aminophen, and diazepam. Only the etbambutol was discontinued; the patient’s skin lesions rapidly healed and healing was complete within 3 weeks. He was not chal- lenged with etbambutol.

DISCUSSION

LDE is a condition that is well characterized both

clinically and histopathologically.3 In our patient,

distribution of the lesions on the back of the hands