- Email: [email protected]
Erythroderma in infancy: A sign of immunodeficiency?
694
3. 4. 5.
6.
7. 8.
9.
Correspondence
epithelial tumors of the skin. Acta Derm Venereol (Stockh) (suppl) 1975;74:163-6. Robinson TA, Kligman AM. Treatment of solar keratoses with retinoie acid and 5-fluorouracil. Br J Derrnatol 1975;92:703-6. Peck GL. Topical tretinoin in actil~c keratosis and basal cell carcinoma. J AM AChD DERA4ATOL1986;15:829-35. Sherertz EF, Sloan KB, McTieman RG. Use of theoretical partition coefficients determined from solubility parameters to predict permeability coefficients for 5-fluorouracil. J Invest Dermatol (in press). Schiltz JR, Lanigan J, Nabial W, Petty B, Birnbaum JE. Retinoic acid induces cyclic changes in epidermal thickness and den'nal collagen and glycosaminoglycan biosynthesis rates. J Invest Dermatol 1986;87:663-7. Zil JS. Vitamin A acid effects on epidermal mitotic activity, thickness and cellularity in the hairless mouse. J Invest Dermatol 1972;59:228-32. Eaglstein WH, Weinstein GD, Frost P. Fluorouracil: mechanism of action in human skin and actinic keratoses. I. Effect on DNA synthesis in vivo. Arch Dermatol 1970;101:132-9. Elias PM. Epidermal effects of retinoids: supramoleeular observations and clinical implications. J AM AChD DERMATOL 1986;15:798-809.
Intralesional injection of sodium stibogluconate by dermal pressure-jet instrument To the Editor: In a recent article (J AM ACAD DERMATOL 1986;15:620-2) I reported the successful treatment of cutaneous leishmaniasis with intralesional sodium stibogluconate (Pentostam). As a closing comment, the suggestion was made that injection by a dermal pressure-jet instrument might be of value for intralesional treatment of cutaneous leishmaniasis. This communication reports the failure of such treatment with a dermal pressure-jet instrument (Dermo-Jet, Robbins Instruments, Inc., Chatham, NJ) in two patients. The reasons for failure can be summarized in the following paragraphs. Inadequate dose of drug delivered into the skin. The inflamed, heavily infiltrated, firm, edematous tissues of the leishmanial nodule, packed with parasitized macrophages, lymphocytes, and histiocytes, left little or no space to receive the additional fluid volume of medication. Most of each pressure-jet injection splattered harmlessly across the skin surface, with little evidence that any of the injection had entered the leishmanial lesion. Certain areas (e.g., the tip of the nose, bony prominences such as the zygoma or angle of the jaw, and knuckles) were particularly unresponsive. There was no evidence of the expected clinical response after
Journal of the American Academy of Dermatology
six to eight injections with the dermal pressure-jet instrument. Impossible to determine the actual dose o f drug delivered into the leishmanial lesion. With much of each pressure-jet injection splattering across the skin surface, the actual dose of drug entering the skin was unknown and indeterminable. Pressure-jet injection more painful. Both patients described much more pain and discomfort with the pressure-jet instrument than with the subsequent use of a 27- or 30-gauge needle. Drug wastage. Most, if not all, of each pressure-jet injection was wasted, so the pressure-jet injection of sodium stibogluconate is not cost-effective. Both patients cleared promptly after eight to ten injections of alternate-day intralesional sodium stibogluconate delivered through a 27- or 30-gauge needle. In summary, the attempts to treat cutaneous leishmaniasis by dermal pressure-jet injection of sodium stibogluconate failed for several reasons: inadequate dose of drug delivered into the leishmanial nodule, inability to determine the actual dose of drug delivered, subjectively greater discomfort and pain from the pressure-jet injection, and poor cost-effectiveness due to drug wastage. Therefore, the treatment of cutaneous leishmaniasis by dermal pressure-jet injection of sodium stibogluconate (Pentostam) is not recommended, but the intralesional delivery of this medication through a 27- or 30-gauge needle can still be recommended with confidence.
Robert E. Kellum, M.D. King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Erythroderma in infancy: A sign of immunodeficiency? To the Editor: Erythroderma in infancy causes great diagnostic difficulties for the dermatologist. Especially during the first few months of life it often raises management probIems because it is potentially lifethreatening, i
Case report. We report the case of a 4-month-old infant, the third child of healthy unrelated parents. Two other siblings were unaffected. Her birth was uneventful at full term after a normal pregnancy. In the second week of life she had hypocalcemic convulsions (calcium level, 4.4 mg/dl). On examination the patient showed a slightly dysmorphic face, low-implanted ears, and a protruded tongue. Congenital
Volume 17 Number 4 October 1987
anomalies of heart, blood vessels, esophagus, and urinary tract were excluded. Dermatitis with marked erythema and scaling appeared in the second month of life; initially it was localized to the scalp and inguinal flexures and then spread over the entire body. Histologic examination of abdominal skin revealed a thin epidermis with focal parakeratosis and slight spongiosis with mononuclear cell exocytosis. The papillary dermis showed edema and a mild chronic inflammatory infiltrate with scattered plasma cells. Microscopic and cultural examinations of scales revealed Candida albicans. The cutaneous lesions were treated with emollients and antimicrobial solutions. The dermatosis continued for 4 weeks after our observation; then a spontaneous recovery occurred. Extensive laboratory studies showed the following abnormalities: there was sideropenic anemia; total white count was 15,700/ram 3, with 45% neutrophils, 45% lymphocytes, 4% eosinophils, and 6% monocytes. Total serum proteins were slightly decreased, but immunoglobulin levels were normal. Serum parathyroid hormone and calcitonin levels were low normal, and caleiuria was detected. There was moderate thymic hypoplasia on chest x-ray examination. The absolute number of lymphocyte subpopulations was the following: OKTll + (pan T ceils) cells were 2330/mm 3 (n.v., 2784-9379); OKT4 + (helper-inducer cells) cells were 430/mm 3 (n.y., 1932-6345); OKT8 + (suppressor-cytotoxic cells) cells were 1500/ram 3 (n.v., 966-3440); Sm Ig + (pan B cells) cells were 716/ram 3 (n.v., 197-1029). The OKT4/OKT8 ratio was 0.28 (n.v., 1.60 ± 0.9). There was a markedly decreased proliferative response to mitogenic lectins such as phytohemagglutinin, coneanavalin A, and pokeweed mitogen. Phagocytosis of the patient's neutrophils and opsonic activity of the patient's serum were assessed by means of chemiluminescence assays within the normal range for age. Bone marrow aspirate showed a normal maturation and differentiation ofmyeloid precursor cells, with slight increase of megakaryocytes and megakaryoblasts. In the following months the patient developed a bullous impetiginous eruption of the face and abdomen and recurrent infections, such as pneumonias and purulent otitis media, which required almost continuous treatment with parenteral antibiotics. She had additional hypocalcemic seizures. Repeated evaluations of lymphocyte subpopulations were consistently abnormal.
Discussion. This observation of erythroderma in an infant presenting features of partial Di George's syndrome (hypocalcemic convulsions and T lymphocyte defec0 is further evidence that skin diseases, such as erythrodermalike rashes, seborrheic dermatitis, and atopic dermatitis, may be early signs of defects of the immune system. Therefore we suggest that extensive immunologic investigations should be carried out in newborns with erythrodermas of uncertain etiology because erythroderma has been reported recently in as-
Correspondence
695
sociation with immunologic defects such as severe atopic dermatitis, ~ Wiskott-Aldrich syndrome, 2 Leinet's disease, 3 severe combined immunodeficiency, 3'4 isolated T lymphocyte defect, 5 and Omenn disease. 6
Annalisa Patrizi, M.D., Marco Masina, M.D., Paola Tassoni, M.D., Alessandra Cassio, M.D., and PaoIo Paolucci, M.D. University of Bologna, Bologna, ltaly REFERENCES 1. Beare JM, Rook A. Leiner's disease. In: Rook A, Wilkinson DS, Ebling FJG, eds. Textbook of dermatology. 3rd ed. Oxford: Blackwell, 1979:196. 2. Gammon WR. Wiskott-Aldrich syndrome. In: Mosehella SL, Hurley HJ, eds. Dermatology. 2nd ed. Philadelphia: WB Saunders, 1985: vol. 1, p. 215. 3. Weston WL. Pediatric (neonatal) dermatology. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF, eds. Dermatologyin general medicine. 2nd ed. New York: McGraw-Hill, 1986: vol. 2, p. 2636. 4. Moore EC, Cohen F. Skin manifestation of immunodefieiency. In: Stone J, ed. Dermatologic immunology and allergy. St. Louis: CV Mosby, 1985:306. 5. Goering HD, Fiering C, Scroeder I. The treatment of isolated cellular immune defect with transfer factor. Dermatol Monatssehr 1980;166:81-7. 6. Janier M, Enjolras O, Mensire A, et al. Reticulose d'Omenn. Ann Dermato] Venerol 1986;113:980-3.
Verruciform xanthoma of the lower extremitymreport of a case and review of literature
To the Editor: Verruciform xanthoma is a rare skin condition characterized histologically by uniform epithelial acanthosis without atypia and foam cells within elongated dermal papillae. The lesion is usually solitary and occurs most commonly on the oral mucosa. A few cases of verruciform xanthoma on the female and male genitalia have been reported, l-~ Verruciform xanthoma also occurs, rarely, as a secondary reaction in lesions with marked epidermal hyperplasia, such as epidermal nevus and inflammatory linear verrucous epidermal nevus .4-6 We present herein a patient with verrueiforrn xanthoma occurring on the toes o f a lymphedematous leg. This case is unusual for its extramucosal location, development o f the lesion in an area where there was no preceding epidermal hyperplasia, and the occurrence of the lesion in a lymphedematous extremity. Case report. A 36-year-old black woman came to the University of Chicago Hospitals and Clinics for a recurrent
3. 4. 5.
6.
7. 8.
9.
Correspondence
epithelial tumors of the skin. Acta Derm Venereol (Stockh) (suppl) 1975;74:163-6. Robinson TA, Kligman AM. Treatment of solar keratoses with retinoie acid and 5-fluorouracil. Br J Derrnatol 1975;92:703-6. Peck GL. Topical tretinoin in actil~c keratosis and basal cell carcinoma. J AM AChD DERA4ATOL1986;15:829-35. Sherertz EF, Sloan KB, McTieman RG. Use of theoretical partition coefficients determined from solubility parameters to predict permeability coefficients for 5-fluorouracil. J Invest Dermatol (in press). Schiltz JR, Lanigan J, Nabial W, Petty B, Birnbaum JE. Retinoic acid induces cyclic changes in epidermal thickness and den'nal collagen and glycosaminoglycan biosynthesis rates. J Invest Dermatol 1986;87:663-7. Zil JS. Vitamin A acid effects on epidermal mitotic activity, thickness and cellularity in the hairless mouse. J Invest Dermatol 1972;59:228-32. Eaglstein WH, Weinstein GD, Frost P. Fluorouracil: mechanism of action in human skin and actinic keratoses. I. Effect on DNA synthesis in vivo. Arch Dermatol 1970;101:132-9. Elias PM. Epidermal effects of retinoids: supramoleeular observations and clinical implications. J AM AChD DERMATOL 1986;15:798-809.
Intralesional injection of sodium stibogluconate by dermal pressure-jet instrument To the Editor: In a recent article (J AM ACAD DERMATOL 1986;15:620-2) I reported the successful treatment of cutaneous leishmaniasis with intralesional sodium stibogluconate (Pentostam). As a closing comment, the suggestion was made that injection by a dermal pressure-jet instrument might be of value for intralesional treatment of cutaneous leishmaniasis. This communication reports the failure of such treatment with a dermal pressure-jet instrument (Dermo-Jet, Robbins Instruments, Inc., Chatham, NJ) in two patients. The reasons for failure can be summarized in the following paragraphs. Inadequate dose of drug delivered into the skin. The inflamed, heavily infiltrated, firm, edematous tissues of the leishmanial nodule, packed with parasitized macrophages, lymphocytes, and histiocytes, left little or no space to receive the additional fluid volume of medication. Most of each pressure-jet injection splattered harmlessly across the skin surface, with little evidence that any of the injection had entered the leishmanial lesion. Certain areas (e.g., the tip of the nose, bony prominences such as the zygoma or angle of the jaw, and knuckles) were particularly unresponsive. There was no evidence of the expected clinical response after
Journal of the American Academy of Dermatology
six to eight injections with the dermal pressure-jet instrument. Impossible to determine the actual dose o f drug delivered into the leishmanial lesion. With much of each pressure-jet injection splattering across the skin surface, the actual dose of drug entering the skin was unknown and indeterminable. Pressure-jet injection more painful. Both patients described much more pain and discomfort with the pressure-jet instrument than with the subsequent use of a 27- or 30-gauge needle. Drug wastage. Most, if not all, of each pressure-jet injection was wasted, so the pressure-jet injection of sodium stibogluconate is not cost-effective. Both patients cleared promptly after eight to ten injections of alternate-day intralesional sodium stibogluconate delivered through a 27- or 30-gauge needle. In summary, the attempts to treat cutaneous leishmaniasis by dermal pressure-jet injection of sodium stibogluconate failed for several reasons: inadequate dose of drug delivered into the leishmanial nodule, inability to determine the actual dose of drug delivered, subjectively greater discomfort and pain from the pressure-jet injection, and poor cost-effectiveness due to drug wastage. Therefore, the treatment of cutaneous leishmaniasis by dermal pressure-jet injection of sodium stibogluconate (Pentostam) is not recommended, but the intralesional delivery of this medication through a 27- or 30-gauge needle can still be recommended with confidence.
Robert E. Kellum, M.D. King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Erythroderma in infancy: A sign of immunodeficiency? To the Editor: Erythroderma in infancy causes great diagnostic difficulties for the dermatologist. Especially during the first few months of life it often raises management probIems because it is potentially lifethreatening, i
Case report. We report the case of a 4-month-old infant, the third child of healthy unrelated parents. Two other siblings were unaffected. Her birth was uneventful at full term after a normal pregnancy. In the second week of life she had hypocalcemic convulsions (calcium level, 4.4 mg/dl). On examination the patient showed a slightly dysmorphic face, low-implanted ears, and a protruded tongue. Congenital
Volume 17 Number 4 October 1987
anomalies of heart, blood vessels, esophagus, and urinary tract were excluded. Dermatitis with marked erythema and scaling appeared in the second month of life; initially it was localized to the scalp and inguinal flexures and then spread over the entire body. Histologic examination of abdominal skin revealed a thin epidermis with focal parakeratosis and slight spongiosis with mononuclear cell exocytosis. The papillary dermis showed edema and a mild chronic inflammatory infiltrate with scattered plasma cells. Microscopic and cultural examinations of scales revealed Candida albicans. The cutaneous lesions were treated with emollients and antimicrobial solutions. The dermatosis continued for 4 weeks after our observation; then a spontaneous recovery occurred. Extensive laboratory studies showed the following abnormalities: there was sideropenic anemia; total white count was 15,700/ram 3, with 45% neutrophils, 45% lymphocytes, 4% eosinophils, and 6% monocytes. Total serum proteins were slightly decreased, but immunoglobulin levels were normal. Serum parathyroid hormone and calcitonin levels were low normal, and caleiuria was detected. There was moderate thymic hypoplasia on chest x-ray examination. The absolute number of lymphocyte subpopulations was the following: OKTll + (pan T ceils) cells were 2330/mm 3 (n.v., 2784-9379); OKT4 + (helper-inducer cells) cells were 430/mm 3 (n.y., 1932-6345); OKT8 + (suppressor-cytotoxic cells) cells were 1500/ram 3 (n.v., 966-3440); Sm Ig + (pan B cells) cells were 716/ram 3 (n.v., 197-1029). The OKT4/OKT8 ratio was 0.28 (n.v., 1.60 ± 0.9). There was a markedly decreased proliferative response to mitogenic lectins such as phytohemagglutinin, coneanavalin A, and pokeweed mitogen. Phagocytosis of the patient's neutrophils and opsonic activity of the patient's serum were assessed by means of chemiluminescence assays within the normal range for age. Bone marrow aspirate showed a normal maturation and differentiation ofmyeloid precursor cells, with slight increase of megakaryocytes and megakaryoblasts. In the following months the patient developed a bullous impetiginous eruption of the face and abdomen and recurrent infections, such as pneumonias and purulent otitis media, which required almost continuous treatment with parenteral antibiotics. She had additional hypocalcemic seizures. Repeated evaluations of lymphocyte subpopulations were consistently abnormal.
Discussion. This observation of erythroderma in an infant presenting features of partial Di George's syndrome (hypocalcemic convulsions and T lymphocyte defec0 is further evidence that skin diseases, such as erythrodermalike rashes, seborrheic dermatitis, and atopic dermatitis, may be early signs of defects of the immune system. Therefore we suggest that extensive immunologic investigations should be carried out in newborns with erythrodermas of uncertain etiology because erythroderma has been reported recently in as-
Correspondence
695
sociation with immunologic defects such as severe atopic dermatitis, ~ Wiskott-Aldrich syndrome, 2 Leinet's disease, 3 severe combined immunodeficiency, 3'4 isolated T lymphocyte defect, 5 and Omenn disease. 6
Annalisa Patrizi, M.D., Marco Masina, M.D., Paola Tassoni, M.D., Alessandra Cassio, M.D., and PaoIo Paolucci, M.D. University of Bologna, Bologna, ltaly REFERENCES 1. Beare JM, Rook A. Leiner's disease. In: Rook A, Wilkinson DS, Ebling FJG, eds. Textbook of dermatology. 3rd ed. Oxford: Blackwell, 1979:196. 2. Gammon WR. Wiskott-Aldrich syndrome. In: Mosehella SL, Hurley HJ, eds. Dermatology. 2nd ed. Philadelphia: WB Saunders, 1985: vol. 1, p. 215. 3. Weston WL. Pediatric (neonatal) dermatology. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF, eds. Dermatologyin general medicine. 2nd ed. New York: McGraw-Hill, 1986: vol. 2, p. 2636. 4. Moore EC, Cohen F. Skin manifestation of immunodefieiency. In: Stone J, ed. Dermatologic immunology and allergy. St. Louis: CV Mosby, 1985:306. 5. Goering HD, Fiering C, Scroeder I. The treatment of isolated cellular immune defect with transfer factor. Dermatol Monatssehr 1980;166:81-7. 6. Janier M, Enjolras O, Mensire A, et al. Reticulose d'Omenn. Ann Dermato] Venerol 1986;113:980-3.
Verruciform xanthoma of the lower extremitymreport of a case and review of literature
To the Editor: Verruciform xanthoma is a rare skin condition characterized histologically by uniform epithelial acanthosis without atypia and foam cells within elongated dermal papillae. The lesion is usually solitary and occurs most commonly on the oral mucosa. A few cases of verruciform xanthoma on the female and male genitalia have been reported, l-~ Verruciform xanthoma also occurs, rarely, as a secondary reaction in lesions with marked epidermal hyperplasia, such as epidermal nevus and inflammatory linear verrucous epidermal nevus .4-6 We present herein a patient with verrueiforrn xanthoma occurring on the toes o f a lymphedematous leg. This case is unusual for its extramucosal location, development o f the lesion in an area where there was no preceding epidermal hyperplasia, and the occurrence of the lesion in a lymphedematous extremity. Case report. A 36-year-old black woman came to the University of Chicago Hospitals and Clinics for a recurrent