- Email: [email protected]
Response to July 1989 CME article
860
Correspondence
Reply To the Editor: Shore et al. have performed significant research and follow-up on a large series of patients who received radiation for benign disease. They were able to determine radiation doses delivered and dates of treatment and have provided accurate information on risk of subsequent skin cancer development. It would have been helpful if we had been able to identify their article before publication of our manuscript in the JOURNAL, but the article was not identified in our literature search. Shore et al. demonstrated that at the fringe of the scalp skin cancer developed about four times more frequently per square centimeter as skin on the scalp itself. Therefore protection from ultraviolet radiation will decrease the risk of skin cancer following radiation for benign disease. Patients with the lowest sun-reactive skin types who received radiation for benign disease are at the highest risk for developing skin cancer. These patients should be doubly warned of the need for sun protection when exposure to the sun cannot be avoided. Mary Margaret Davis, MD, and C. William Hanke, MD, FACP Indiana University School of Medicine Indianapolis. IN 46223
Bullous pemphigoid and multiple sclerosis To the Editor: In response to the report of Masouye et al. (J AM ACAD DERMATOL 1989;21:63-8), I would like to add an additional case that strengthens their contention that the concurrence of these diseases is more than coincidental. Case report. During September 1986 a 43-year-old white man with a 19-year history of multiple sclerosis had blisters and crusting on the anterior aspect of the right thigh. For 4 years he had been taking baclofen, a muscle relaxant, and for 3 weeks tetracycline, 250 mg twice daily for a folliculitis of the shoulders and the upper part of the back. The eruption on the thigh was diagnosedas impetigo, and he was treated with dicloxacillin, 250 mg four times daily, and a topical antibacterial agent (Polysporin); tetracycline was discontinued. The patient returned 14 days later with a pruritic bullous eruption of both legs,the neck, the right forearm, and trunk. The blisters were tense and showed no peripheral extension with pressure, and many were now on an urticarial base. Iris lesions and mucosal lesions were not present. A biopsy specimen showed a discrete subepidermal blister filled with eosinophils. The dermis showed an active inflammatory infiltrate of eosinophils, ncutrophils, and rnonocytes in a perivascular arrangement. These changes were interpreted as compatible with bullous pemphigoid, a dermal form oferythema
Journal of the American Academy of Dermatology
multiforme, or a cell-rich variant of epidermolysis bulJosa acquisita (EBA). Bacterial culture of aspirated blister fluid wassterile, and direct immunofluorescence of peribullous tissue showed linear deposition of IgG and C3 at the dcrmoepidermal junction. Indirect immunofluorescence was not done. A diagnosis of bullous pemphigoidor a pemphigoid-like reaction to baclofen was made, although this reaction to baclofen has never been reported. Prednisonetreatment, 30mg everymorning, wasstarted, and the patient's neurologist gradually reduced the dosage of baclofen. Baclofen was discontinued in I week, and by 2 weeks the blisters were inactive and prednisone taper was initiated. Despitethe initial response to prednisone,while the patient was not receiving baclofen, mildflareswereobservedduring the next 6 months at prednisone dosages of 15 and \ 2.5 rng/day, In March \ 987 sustained-release tripelennamine, 100 mg twice daily, wasadded as a steroid-sparingagent and prednisonetaper was slowly resumed. Steroid therapy was discontinued in December \ 987, and the patient remained clear with tripelennamine treatment from January to December 1988 when this agent was also stopped. During the period of steroid taper, baclofen was cautiously reintroduced without adverse effect. From December 1988 to April \989 the patient remained clear while receiving baclofen and noother medication. In April 1989pruritic urticarial lesions recurred on both hips. These cleared promptly with tripelennamine treatment, and the patient is currently receiving that medication and has no symptoms. Discussion. Although available lab tests do not absolutely exclude EBA, the clinical course and prompt response to prednisone were most consistent with bullous pemphigoid. EBA is notoriously refractory to most therapy, whereas my patient successfully discontinued steroids and remains clear with a low dosage of tripelennamine and at times no medication. This patient is similar to those reported by Masouye et aI. by having long-standing advanced multiple sclerosis and relatively youthful onset of bullous pemphigoid, which was of moderate severity and responded promptly to treatment. I also agree with Masouye et al. that baclofen was not the cause of the bullous pemphigoid.
Daniel J. Trozak, MD 1444 Florida Ave., Suite 201 Modesto, CA 95350
Response to July 1989 CME article To the Editor: I would like to add some historical remarks on Miller's CME article "Nutritional Deficiency and the Skin" (J AM ACAD DERMATOL 1989;21:1-30). The part
Volume 22 Number 5, Part I May]990
Correspondence 861
dealing with zinc deficiency has especially caught my interest and attention. As in Miller's paper, acrodermatitis enteropathica is often cited to have been first described by Danbolt and Closs in 1942. But in fact it was the Swedish dermatologist Thore Brandt I who 6 years earlier gave an extensive description with pictures of the condition. He did not name it, because others simply regarded the disease as a variety of Hallopeau's acrodermatitis or of epidermolysis bullosa. Danbolt and Closs, as mentioned in the article, confirmed the findings and coined the term. Acquired zinc deficiency, zinc depletion syndrome resulting from parenteral nutrition devoid of zinc, was first described by Kay et a1,2 in 1976. The patient depicted in Fig. 17 of Miller's article is well known to me because it was one of two patients first reported from Denmark. The case history, including the clinical picture, had been reported earlier.'
REFERENCES l . Brandt T. Dermatitis in children with disturbances of the general condition and the absorption of food elements. Acta Derm Venereal (Stockh) 1936;17:513-46. 2. Kay RG, Tasman-Jones C, Pybus J, et a!. A syndrome of acute zinc deficiency during total parenteral alimentation in man. Ann Surg 1976;]83:331-40. 3. Weismann K, Hjorth N, Fischer A. Zinc depletion syndrome with acrodermatitis during long-term intravenous feeding. Clin Exp Derrnatol 1976;1:237-42.
Reply To the Editor: I thank Dr. Weismann for providing interesting additional history of the early description of acrodermatitis enteropathica and offer my sincere apologies for unknowingly publishing the fine clinical photograph without appropriate acknowledgement.
Kaare Weismann, MD Department of Dermatology Bispebjerg Hospital Copenhagen, Denmark
Stanley J. Miller San Diego, California
ABSTRACTS Hermansky-Pudlak syndrome
The pathogenesis of lichen planus
Schallreuter KU. Hautarzt 1989;40:130-3 (German)
Rochen M. Z Hautkr 1988;63:911-4 (German)
Hermansky-Pudlak syndrome is a hereditary diseasewithan autosomal recessivemodeof inheritance, characterized by the triad of tyrosinase-positiveoculocutancousalbinism, a hemorrhagic diathesis resulting from storage pool-deficient platelets, and accumulationof ceroid/ lipofuscin-like material in various cells and tissues. The basis defect remains unknown. It is believed that the primary defect may involve membranes of the platelet-densebodiesand the melanosomes. Recently a defective calcium uptake system and low activities for membraneassociated thioredoxin reductase have been shown. Yehudi M. Felman, M D
Although lichen planus is still considered a disease of unknown origin, the authors developa synoptic pathogenetic concept based on the variousscientific studies in thisfield, which may supply a rational foundation for empirically established therapy. Genetic and metabolic factors predispose patients to the development of lichen planus, which can be viewed as a type IV reaction against immunologicallyaltered keratinocytcs, The papule is the result of exaggerated repair mechanisms, Yehudi M. Felman, MD HLA-DR antigens and alopecia arcata
Electronmicroscopic examination of Darter's keratosis follicularis
Offidani AM, Simonetti 0, Politi A, et al. G Ital Dermatol Venereal 1988;123:409-12 (Italian)
Geling 2M, Chistyakova lA. Vestn Dcrmatol Venerol 1989;1:14-7 (Russian)
A group of22 unrelated patients (9 ma.e and 13 female) with alopecia arcata and 100 normal control subjec:s were typed for HLA-DR specificities. Twelve patients had patchy alopecia arcata and 10 had alopecia totalis. In the entire group of patients, no significant association was found with any DR antigen. When patients were subgrouped accordingto skininvolvement, DR2 and DR3 were increased in patients with patchy alopecia arcata and alopecia totalis, respectively. These data suggest that alopecia arcata is genetically a heterogenous disease and that DRS characterizesthe most severe form (RR = 4.3,p < 0.05).
Ultrastructure of the skin biopsyspecimensof 10patients with Darier's keratosis follicularis (DKF), six of them with the vesicularformof the dermatosis, is reported. The study revealed dyskeratosis with tonofilament homogenization and well-developed keratohyalin granules, round bodies and granules, and acantholysis less apparent than in chronic benign familial pemphigus. Ultrastructural featuresof the skin in vesicular DKF are acantholysis and dyskeratosis more prominent than in typical DKF. Yehudi M. Felman, MD
Yehudi M. Fe/mall, /lfD
Correspondence
Reply To the Editor: Shore et al. have performed significant research and follow-up on a large series of patients who received radiation for benign disease. They were able to determine radiation doses delivered and dates of treatment and have provided accurate information on risk of subsequent skin cancer development. It would have been helpful if we had been able to identify their article before publication of our manuscript in the JOURNAL, but the article was not identified in our literature search. Shore et al. demonstrated that at the fringe of the scalp skin cancer developed about four times more frequently per square centimeter as skin on the scalp itself. Therefore protection from ultraviolet radiation will decrease the risk of skin cancer following radiation for benign disease. Patients with the lowest sun-reactive skin types who received radiation for benign disease are at the highest risk for developing skin cancer. These patients should be doubly warned of the need for sun protection when exposure to the sun cannot be avoided. Mary Margaret Davis, MD, and C. William Hanke, MD, FACP Indiana University School of Medicine Indianapolis. IN 46223
Bullous pemphigoid and multiple sclerosis To the Editor: In response to the report of Masouye et al. (J AM ACAD DERMATOL 1989;21:63-8), I would like to add an additional case that strengthens their contention that the concurrence of these diseases is more than coincidental. Case report. During September 1986 a 43-year-old white man with a 19-year history of multiple sclerosis had blisters and crusting on the anterior aspect of the right thigh. For 4 years he had been taking baclofen, a muscle relaxant, and for 3 weeks tetracycline, 250 mg twice daily for a folliculitis of the shoulders and the upper part of the back. The eruption on the thigh was diagnosedas impetigo, and he was treated with dicloxacillin, 250 mg four times daily, and a topical antibacterial agent (Polysporin); tetracycline was discontinued. The patient returned 14 days later with a pruritic bullous eruption of both legs,the neck, the right forearm, and trunk. The blisters were tense and showed no peripheral extension with pressure, and many were now on an urticarial base. Iris lesions and mucosal lesions were not present. A biopsy specimen showed a discrete subepidermal blister filled with eosinophils. The dermis showed an active inflammatory infiltrate of eosinophils, ncutrophils, and rnonocytes in a perivascular arrangement. These changes were interpreted as compatible with bullous pemphigoid, a dermal form oferythema
Journal of the American Academy of Dermatology
multiforme, or a cell-rich variant of epidermolysis bulJosa acquisita (EBA). Bacterial culture of aspirated blister fluid wassterile, and direct immunofluorescence of peribullous tissue showed linear deposition of IgG and C3 at the dcrmoepidermal junction. Indirect immunofluorescence was not done. A diagnosis of bullous pemphigoidor a pemphigoid-like reaction to baclofen was made, although this reaction to baclofen has never been reported. Prednisonetreatment, 30mg everymorning, wasstarted, and the patient's neurologist gradually reduced the dosage of baclofen. Baclofen was discontinued in I week, and by 2 weeks the blisters were inactive and prednisone taper was initiated. Despitethe initial response to prednisone,while the patient was not receiving baclofen, mildflareswereobservedduring the next 6 months at prednisone dosages of 15 and \ 2.5 rng/day, In March \ 987 sustained-release tripelennamine, 100 mg twice daily, wasadded as a steroid-sparingagent and prednisonetaper was slowly resumed. Steroid therapy was discontinued in December \ 987, and the patient remained clear with tripelennamine treatment from January to December 1988 when this agent was also stopped. During the period of steroid taper, baclofen was cautiously reintroduced without adverse effect. From December 1988 to April \989 the patient remained clear while receiving baclofen and noother medication. In April 1989pruritic urticarial lesions recurred on both hips. These cleared promptly with tripelennamine treatment, and the patient is currently receiving that medication and has no symptoms. Discussion. Although available lab tests do not absolutely exclude EBA, the clinical course and prompt response to prednisone were most consistent with bullous pemphigoid. EBA is notoriously refractory to most therapy, whereas my patient successfully discontinued steroids and remains clear with a low dosage of tripelennamine and at times no medication. This patient is similar to those reported by Masouye et aI. by having long-standing advanced multiple sclerosis and relatively youthful onset of bullous pemphigoid, which was of moderate severity and responded promptly to treatment. I also agree with Masouye et al. that baclofen was not the cause of the bullous pemphigoid.
Daniel J. Trozak, MD 1444 Florida Ave., Suite 201 Modesto, CA 95350
Response to July 1989 CME article To the Editor: I would like to add some historical remarks on Miller's CME article "Nutritional Deficiency and the Skin" (J AM ACAD DERMATOL 1989;21:1-30). The part
Volume 22 Number 5, Part I May]990
Correspondence 861
dealing with zinc deficiency has especially caught my interest and attention. As in Miller's paper, acrodermatitis enteropathica is often cited to have been first described by Danbolt and Closs in 1942. But in fact it was the Swedish dermatologist Thore Brandt I who 6 years earlier gave an extensive description with pictures of the condition. He did not name it, because others simply regarded the disease as a variety of Hallopeau's acrodermatitis or of epidermolysis bullosa. Danbolt and Closs, as mentioned in the article, confirmed the findings and coined the term. Acquired zinc deficiency, zinc depletion syndrome resulting from parenteral nutrition devoid of zinc, was first described by Kay et a1,2 in 1976. The patient depicted in Fig. 17 of Miller's article is well known to me because it was one of two patients first reported from Denmark. The case history, including the clinical picture, had been reported earlier.'
REFERENCES l . Brandt T. Dermatitis in children with disturbances of the general condition and the absorption of food elements. Acta Derm Venereal (Stockh) 1936;17:513-46. 2. Kay RG, Tasman-Jones C, Pybus J, et a!. A syndrome of acute zinc deficiency during total parenteral alimentation in man. Ann Surg 1976;]83:331-40. 3. Weismann K, Hjorth N, Fischer A. Zinc depletion syndrome with acrodermatitis during long-term intravenous feeding. Clin Exp Derrnatol 1976;1:237-42.
Reply To the Editor: I thank Dr. Weismann for providing interesting additional history of the early description of acrodermatitis enteropathica and offer my sincere apologies for unknowingly publishing the fine clinical photograph without appropriate acknowledgement.
Kaare Weismann, MD Department of Dermatology Bispebjerg Hospital Copenhagen, Denmark
Stanley J. Miller San Diego, California
ABSTRACTS Hermansky-Pudlak syndrome
The pathogenesis of lichen planus
Schallreuter KU. Hautarzt 1989;40:130-3 (German)
Rochen M. Z Hautkr 1988;63:911-4 (German)
Hermansky-Pudlak syndrome is a hereditary diseasewithan autosomal recessivemodeof inheritance, characterized by the triad of tyrosinase-positiveoculocutancousalbinism, a hemorrhagic diathesis resulting from storage pool-deficient platelets, and accumulationof ceroid/ lipofuscin-like material in various cells and tissues. The basis defect remains unknown. It is believed that the primary defect may involve membranes of the platelet-densebodiesand the melanosomes. Recently a defective calcium uptake system and low activities for membraneassociated thioredoxin reductase have been shown. Yehudi M. Felman, M D
Although lichen planus is still considered a disease of unknown origin, the authors developa synoptic pathogenetic concept based on the variousscientific studies in thisfield, which may supply a rational foundation for empirically established therapy. Genetic and metabolic factors predispose patients to the development of lichen planus, which can be viewed as a type IV reaction against immunologicallyaltered keratinocytcs, The papule is the result of exaggerated repair mechanisms, Yehudi M. Felman, MD HLA-DR antigens and alopecia arcata
Electronmicroscopic examination of Darter's keratosis follicularis
Offidani AM, Simonetti 0, Politi A, et al. G Ital Dermatol Venereal 1988;123:409-12 (Italian)
Geling 2M, Chistyakova lA. Vestn Dcrmatol Venerol 1989;1:14-7 (Russian)
A group of22 unrelated patients (9 ma.e and 13 female) with alopecia arcata and 100 normal control subjec:s were typed for HLA-DR specificities. Twelve patients had patchy alopecia arcata and 10 had alopecia totalis. In the entire group of patients, no significant association was found with any DR antigen. When patients were subgrouped accordingto skininvolvement, DR2 and DR3 were increased in patients with patchy alopecia arcata and alopecia totalis, respectively. These data suggest that alopecia arcata is genetically a heterogenous disease and that DRS characterizesthe most severe form (RR = 4.3,p < 0.05).
Ultrastructure of the skin biopsyspecimensof 10patients with Darier's keratosis follicularis (DKF), six of them with the vesicularformof the dermatosis, is reported. The study revealed dyskeratosis with tonofilament homogenization and well-developed keratohyalin granules, round bodies and granules, and acantholysis less apparent than in chronic benign familial pemphigus. Ultrastructural featuresof the skin in vesicular DKF are acantholysis and dyskeratosis more prominent than in typical DKF. Yehudi M. Felman, MD
Yehudi M. Fe/mall, /lfD