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Ataxia-telangiectasia
686
Correspondence
Journal of the American Academy of Dermatology April 1995
Ataxia-telangiectasia
Cryosurgery for lentigo maligna
To the Editor: Giitz et al. recently described a 13-yearold girl with ataxia-telangiectasia and ulcerating lesions that by histopathologic examination showed palisading necrobiotic granulomas in the dermis (J AM ACAD DERMATOL 1994;3 1:124-6). The authors conclude that this patient has necrobiosis lipoidica in view of the clinical features of yellow-brown discoloration, atrophy, telangiectasia, and ulceration; the appearance of biopsy sections; and the known association of ataxia-tclangiectasia with glucose intolerance. In 199 1, we described eight patients with ataxia-telangiectasia and cutaneous granulomatous lesions, which were erythematous, atrophic lesions that frequently became ulcerated and crusted.’ Biopsy specimens of lesional skin showed a palisading arrangement of lymphocytes, histiocytes, and giant cells surrounding foci of degenerated collagen. Special stains and cultures of biopsy specimens in these immunocompromised patients revealed no organisms. Before our report, Fleck et a1.2 noted no,ninfectious cutaneous and synovial granulomas in a 13-yearold girl with ataxia-telangiectasia, thought to be “sarcoidosis.” In all cases,the granulomas were persistent, often recurring after surgical “excision.” We consider theclinical and histopathologic features of the lesions in the patient described by Giitz et al. to be a distinct granulomatous entity that is not necrobiosis lipoidica but that resembles the lesions of the nine previously described patients with ataxia-telangiectasia and atrophic, often ulcerated cutaneous plaques. Because similar granulomatous lesions have been described in patients with other primary immunodeficiency disorders, including chronic granulomatous disease,common variable immunodeficiency, X-linked hypogammaglobulinemia, and combined immunodeficiency, it is possible that these cutaneous granulomas represent an attempt to localize antigen in the face of a dysfunctional immune system.
To the Editor: Kuflik and Gage’s article on the use of cryosurgery for lentigo maligna listed various treatments of that entity (J AM ACAD DERMATOL 199+31:75-g). Those options mentioned included “surgical excision * . . electrodesiccation, laser therapy, and radiotherapy,” with the authors believing that “cryosurgery is superior to all others as an alternative to conventional surgery.” (p. 77) This may be true, but certainly dermabrasion should have been mentioned as an alternative form of therapy on facial lesions where cosmesis was important and the technique otherwise equal in potential for curative intervention.l> 2 The various entities treated with dermabrasion include “superficial basal cell carcinoma, multiple pigmented nevi, chloasma, congenital pigmented nevi, verrucous nevus, linear epidermal nevus, and lentigines,“3 as well as multiple actinic keratoses4 various forms of scarring, and a variety of seborrheic keratoses. Many cosmetic indications are also referenced.t’ 5 Even recurrent basal cell carcinoma of the nasal tip was selectively dermabraded, with an excellent 1-year postoperative status.5 I have lectured and presented photographs many times regarding carefully selected and specifically informed patients with facial lentigo maligna who elected dermabrasion and close observation to potentially deforming surgical excision. I did not personally offer electrodesiccation, radiotherapy, or cryoprobe-controlled super& cial spray technique, but some occasional lesions seen by me were indeed successfully treated with open-spray application with a double or triple freeze-thaw technique. I have no statistics to offer, but those many hundreds of dermatologists capable of harkening back to my dermabrasion presentations will recall the validity of the contention that dermabrasion may well represent a most appropriate and perhaps the most cosmetically elegant approach to certain superficial lentigines of early melanomatous type.
Amy S. Paller, MD Division-of Dermatology Departments of Pediatrics and Dermatology The Children’s Memorial Hospital Northwestern University Medical School Chicago, Illinois Michael Swift, MD Division of Human Genetics Department of Pediatrics New York Medical College Valhalla, New York REFERENCES 1. Paller AS, Massey RB, Curtis MA, et al. Cutaneous granulomatous lesions in patients with ataxia-telangiectasia. J Pediatr 1991;119:917-22. 2. Fleck RM, Myers LK, Wasserman RL, et al. Ataxia-telangiectasia associated with sarcoidosis. Pediatr Dermatol 1986;3:339-43.
Lawrence M. Field, MD 700 Promontory Point Lane, Suite I1 03 Foster City, CA 94404 REPERENCES 1. Burks J. Dermabrasion and chemical peeling. Springfield, Ilk Charles C Thomas, 1979:210. 2. Farber G. Verbal communications, 1976-1994. 3. Roenigk H Jr. Dermabrasion. In: Roenigk R, Roenigk H. Dermatologic surgery: principles and practice. New York: Marcel Dekker, 1989:959-78. 4. Field L. On the value of dermabrasion in the management of actinic keratoses. In: Epstein E, ed. Controversies in dermatology. Philadelphia: WB Saunders, 1984:96-102. 5. Roenigk H Jr. Dermabrasion for miscellaneous cutaneous lesions (exclusive of scarring from acne). J Dermatol Surg Oncol 1977;3:322-8.
Correspondence
Journal of the American Academy of Dermatology April 1995
Ataxia-telangiectasia
Cryosurgery for lentigo maligna
To the Editor: Giitz et al. recently described a 13-yearold girl with ataxia-telangiectasia and ulcerating lesions that by histopathologic examination showed palisading necrobiotic granulomas in the dermis (J AM ACAD DERMATOL 1994;3 1:124-6). The authors conclude that this patient has necrobiosis lipoidica in view of the clinical features of yellow-brown discoloration, atrophy, telangiectasia, and ulceration; the appearance of biopsy sections; and the known association of ataxia-tclangiectasia with glucose intolerance. In 199 1, we described eight patients with ataxia-telangiectasia and cutaneous granulomatous lesions, which were erythematous, atrophic lesions that frequently became ulcerated and crusted.’ Biopsy specimens of lesional skin showed a palisading arrangement of lymphocytes, histiocytes, and giant cells surrounding foci of degenerated collagen. Special stains and cultures of biopsy specimens in these immunocompromised patients revealed no organisms. Before our report, Fleck et a1.2 noted no,ninfectious cutaneous and synovial granulomas in a 13-yearold girl with ataxia-telangiectasia, thought to be “sarcoidosis.” In all cases,the granulomas were persistent, often recurring after surgical “excision.” We consider theclinical and histopathologic features of the lesions in the patient described by Giitz et al. to be a distinct granulomatous entity that is not necrobiosis lipoidica but that resembles the lesions of the nine previously described patients with ataxia-telangiectasia and atrophic, often ulcerated cutaneous plaques. Because similar granulomatous lesions have been described in patients with other primary immunodeficiency disorders, including chronic granulomatous disease,common variable immunodeficiency, X-linked hypogammaglobulinemia, and combined immunodeficiency, it is possible that these cutaneous granulomas represent an attempt to localize antigen in the face of a dysfunctional immune system.
To the Editor: Kuflik and Gage’s article on the use of cryosurgery for lentigo maligna listed various treatments of that entity (J AM ACAD DERMATOL 199+31:75-g). Those options mentioned included “surgical excision * . . electrodesiccation, laser therapy, and radiotherapy,” with the authors believing that “cryosurgery is superior to all others as an alternative to conventional surgery.” (p. 77) This may be true, but certainly dermabrasion should have been mentioned as an alternative form of therapy on facial lesions where cosmesis was important and the technique otherwise equal in potential for curative intervention.l> 2 The various entities treated with dermabrasion include “superficial basal cell carcinoma, multiple pigmented nevi, chloasma, congenital pigmented nevi, verrucous nevus, linear epidermal nevus, and lentigines,“3 as well as multiple actinic keratoses4 various forms of scarring, and a variety of seborrheic keratoses. Many cosmetic indications are also referenced.t’ 5 Even recurrent basal cell carcinoma of the nasal tip was selectively dermabraded, with an excellent 1-year postoperative status.5 I have lectured and presented photographs many times regarding carefully selected and specifically informed patients with facial lentigo maligna who elected dermabrasion and close observation to potentially deforming surgical excision. I did not personally offer electrodesiccation, radiotherapy, or cryoprobe-controlled super& cial spray technique, but some occasional lesions seen by me were indeed successfully treated with open-spray application with a double or triple freeze-thaw technique. I have no statistics to offer, but those many hundreds of dermatologists capable of harkening back to my dermabrasion presentations will recall the validity of the contention that dermabrasion may well represent a most appropriate and perhaps the most cosmetically elegant approach to certain superficial lentigines of early melanomatous type.
Amy S. Paller, MD Division-of Dermatology Departments of Pediatrics and Dermatology The Children’s Memorial Hospital Northwestern University Medical School Chicago, Illinois Michael Swift, MD Division of Human Genetics Department of Pediatrics New York Medical College Valhalla, New York REFERENCES 1. Paller AS, Massey RB, Curtis MA, et al. Cutaneous granulomatous lesions in patients with ataxia-telangiectasia. J Pediatr 1991;119:917-22. 2. Fleck RM, Myers LK, Wasserman RL, et al. Ataxia-telangiectasia associated with sarcoidosis. Pediatr Dermatol 1986;3:339-43.
Lawrence M. Field, MD 700 Promontory Point Lane, Suite I1 03 Foster City, CA 94404 REPERENCES 1. Burks J. Dermabrasion and chemical peeling. Springfield, Ilk Charles C Thomas, 1979:210. 2. Farber G. Verbal communications, 1976-1994. 3. Roenigk H Jr. Dermabrasion. In: Roenigk R, Roenigk H. Dermatologic surgery: principles and practice. New York: Marcel Dekker, 1989:959-78. 4. Field L. On the value of dermabrasion in the management of actinic keratoses. In: Epstein E, ed. Controversies in dermatology. Philadelphia: WB Saunders, 1984:96-102. 5. Roenigk H Jr. Dermabrasion for miscellaneous cutaneous lesions (exclusive of scarring from acne). J Dermatol Surg Oncol 1977;3:322-8.