I realize that this is purely a single anecdote, but examples of Gunther's disease are rare. I tried to persuade the patient to submit to a blind trial, but she was so impressed with the protection afforded by beta carotene that she refused to take any control tablets. There has been no evidence that the improvement of the skin blistering has had any beneficial effect on her hemolysis. She has continued to require the same number of transfu¬ sions per year as before beginning the beta carotene regimen. I. B. Sneddon, FRCP case
Sheffield, England In Reply.\p=m-\Isuspect that Dr Sneddon is correct in his assumption that beta carotene is beneficial therapy for
with erythropoietic porphyria. One of my patients (patient 2 of the study reported in the Archives, who is now 22 years old) was given beta carotene, 120 mg each day, during the past two summers. During this time his occupation as a "traveling salesman" required extensive periods of time away from home during which he would occasionally deplete his supply of beta carotene. Last summer, beta carotene therapy was started in April. He was free of blisters until May, when he discontinued his medication and three new blisters subsequently developed. Medication was resumed in mid-May, and the patient remained free of blisters for the remainder of the summer. Beta carotene treatment was discontinued in the fall, and in December, two new blisters formed on the hands of the patient. Beta carotene, along with prompt appropriate treatment of bacterial infections of the skin, has seemed to be beneficial for this patient. I would welcome communication from others that have had any experience with beta carotene therapy for erythro¬
patients
poietic porphyria.
George S. Stretcher, MD
Spartanburg, SC
Nonproprietary Name and Trademark of Drug Beta carotene—Solatene.
Hyphens
and En Dashes
To the Editor.\p=m-\"Anti-basal-cellanti-
body" is an improvement on the "antibasal cell antibody" that Dr Edward Shmunes says he originally wrote (Arch Dermatol 114:125, 1978), and it
is far better than "antibasal cell anti-
body," to which the term was changed by the manuscript editor.
Neither Dr Shmunes's version nor Dr Spatz's, however, is ideal: both are a little ambiguous. What is needed is
to attach "anti" to "basal-cell," not just to "basal." This can be done, not by using a hyphen, but by using the slightly longer (and unhappily, largely forgotten) en dash. The result looks like this: anti-basal-cell antibody. And
that it means an antibody against basal cells. The en dash is also needed, and almost never used, to connect a two\x=req-\ word expression with another of eiany reader
can see
one or two words, eg, periodic acid-Schiff, which is a mysterious
ther
first not proto be printed
expression\p=m-\especiallyif word (pair\m=.\eye\m=.\o\m=.\dic)is
the
nounced properly, ought thus: periodic acid-Schiff. And incidentally, in reference to Dr Spatz's comments on p 126 of the same Archives, there is only a single "1" in both sequela and sequelae, according to the Oxford English Dictionary and both Gould's and Dorland's medical dictionaries. Harry L. Arnold, Jr, MD Honolulu
Photochemotherapy for Psoriasis To the Editor.\p=m-\The article in the December Archives (113:1667-1670, 1977) by Roenigk and Martin on photochemotherapy of psoriasis was most informative. The authors noted that their patients wore sunglasses that protect against ultraviolet-A (UV-A) for five hours after photochemotherapy is given in the office. I would like to point out that for the two hours prior to receiving the photochemotherapy, the patients should also wear the protective glasses since they do have substantial blood levels of methoxsalen and are receiving UV-A from sunlight. Since we all are concerned about the possibility of retinal and/or lens changes that might occur with this form of treatment, we should take every precaution to minimize the patient's exposure to UV-A while there are detectable blood levels of methoxsalen. Even these measures may not prevent ocular changes, since we do not know whether methoxsalen enters the human lens and, if so, how long it remains there or even whether it potentiates UV-A damage to the lens.
Ross
Hensley, MD
Lawton, Okla
In Reply.\p=m-\You are absolutely correct concerning the use of protective
sunglasses for the two hours preceding photochemotherapy, in addition to
the five hours after treatment. Although the peak blood levels of methoxsalen are at two hours, there is a substantial amount present during the two hours prior to this peak as well. Harry H. Roenigk, Jr, MD
Chicago
Ichthyosiform Dermatosis and Deafness
To the Editor.\p=m-\Ibelieve that there are reported cases of the syndrome of ichthyosiform dermatosis and deafness as discussed by Baden and Alper more
(Archives 113:1701-1704, 1977). Very recently, Senter and colleagues' added another case; they included in their
summary a similar case of3 Salamon et al2 and three cases of Tay who clearly lack some of the predominant features. Still another patient who was described in 1975 by Pincus et al4 seems to belong to this group. In addition to the ichthyosiform skin disorder and neurosensory deafness, her patient demonstrates several of the other features common in this syndrome: loss of vision owing to progressive vascularization of the corneas; abnormalities of the hair, teeth, and nails; postnatal growth deficiency; and neuromuscular defects and recurrent skin infections. The patient in Pincus's case report is now 8 years old and has had hyperkeratotic and erythematous skin from birth. A skin biopsy specimen showed
papillomatosis, hyperkeratosis, irregular hypergranulosis, focal parakeratosis, and areas of dermal histiocytic
infiltration in the presence of normal skin appendages. Hearing was thought to be deficient at the age of 10 months when recur¬ rent otitis media and externa were noted. At age 3, pronounced sensorineural hearing loss was demonstrated; the patient's best ear had some sensi¬ tivity to a live voice at 90 dB. Bilateral interstitial corneal opaci¬ ties were apparent at 10 months of age. Recurrent bacterial conjunctivi¬ tis and progressive vascularization of the corneas, even after corneal trans¬ plantation, have resulted in near blindness. Hair was present at birth, but scar¬ ring alopecia of the scalp, along with loss of eyebrows and eyelashes, have followed recurrent skin infections. Primary teeth developed normally, while slow root development delayed adult dentition. Subsequent gingival infections have necessitated the ex¬ traction of ten teeth.
Downloaded From: http://archderm.jamanetwork.com/ by a University of Michigan User on 05/18/2015
Sheffield, England In Reply.\p=m-\Isuspect that Dr Sneddon is correct in his assumption that beta carotene is beneficial therapy for
with erythropoietic porphyria. One of my patients (patient 2 of the study reported in the Archives, who is now 22 years old) was given beta carotene, 120 mg each day, during the past two summers. During this time his occupation as a "traveling salesman" required extensive periods of time away from home during which he would occasionally deplete his supply of beta carotene. Last summer, beta carotene therapy was started in April. He was free of blisters until May, when he discontinued his medication and three new blisters subsequently developed. Medication was resumed in mid-May, and the patient remained free of blisters for the remainder of the summer. Beta carotene treatment was discontinued in the fall, and in December, two new blisters formed on the hands of the patient. Beta carotene, along with prompt appropriate treatment of bacterial infections of the skin, has seemed to be beneficial for this patient. I would welcome communication from others that have had any experience with beta carotene therapy for erythro¬
patients
poietic porphyria.
George S. Stretcher, MD
Spartanburg, SC
Nonproprietary Name and Trademark of Drug Beta carotene—Solatene.
Hyphens
and En Dashes
To the Editor.\p=m-\"Anti-basal-cellanti-
body" is an improvement on the "antibasal cell antibody" that Dr Edward Shmunes says he originally wrote (Arch Dermatol 114:125, 1978), and it
is far better than "antibasal cell anti-
body," to which the term was changed by the manuscript editor.
Neither Dr Shmunes's version nor Dr Spatz's, however, is ideal: both are a little ambiguous. What is needed is
to attach "anti" to "basal-cell," not just to "basal." This can be done, not by using a hyphen, but by using the slightly longer (and unhappily, largely forgotten) en dash. The result looks like this: anti-basal-cell antibody. And
that it means an antibody against basal cells. The en dash is also needed, and almost never used, to connect a two\x=req-\ word expression with another of eiany reader
can see
one or two words, eg, periodic acid-Schiff, which is a mysterious
ther
first not proto be printed
expression\p=m-\especiallyif word (pair\m=.\eye\m=.\o\m=.\dic)is
the
nounced properly, ought thus: periodic acid-Schiff. And incidentally, in reference to Dr Spatz's comments on p 126 of the same Archives, there is only a single "1" in both sequela and sequelae, according to the Oxford English Dictionary and both Gould's and Dorland's medical dictionaries. Harry L. Arnold, Jr, MD Honolulu
Photochemotherapy for Psoriasis To the Editor.\p=m-\The article in the December Archives (113:1667-1670, 1977) by Roenigk and Martin on photochemotherapy of psoriasis was most informative. The authors noted that their patients wore sunglasses that protect against ultraviolet-A (UV-A) for five hours after photochemotherapy is given in the office. I would like to point out that for the two hours prior to receiving the photochemotherapy, the patients should also wear the protective glasses since they do have substantial blood levels of methoxsalen and are receiving UV-A from sunlight. Since we all are concerned about the possibility of retinal and/or lens changes that might occur with this form of treatment, we should take every precaution to minimize the patient's exposure to UV-A while there are detectable blood levels of methoxsalen. Even these measures may not prevent ocular changes, since we do not know whether methoxsalen enters the human lens and, if so, how long it remains there or even whether it potentiates UV-A damage to the lens.
Ross
Hensley, MD
Lawton, Okla
In Reply.\p=m-\You are absolutely correct concerning the use of protective
sunglasses for the two hours preceding photochemotherapy, in addition to
the five hours after treatment. Although the peak blood levels of methoxsalen are at two hours, there is a substantial amount present during the two hours prior to this peak as well. Harry H. Roenigk, Jr, MD
Chicago
Ichthyosiform Dermatosis and Deafness
To the Editor.\p=m-\Ibelieve that there are reported cases of the syndrome of ichthyosiform dermatosis and deafness as discussed by Baden and Alper more
(Archives 113:1701-1704, 1977). Very recently, Senter and colleagues' added another case; they included in their
summary a similar case of3 Salamon et al2 and three cases of Tay who clearly lack some of the predominant features. Still another patient who was described in 1975 by Pincus et al4 seems to belong to this group. In addition to the ichthyosiform skin disorder and neurosensory deafness, her patient demonstrates several of the other features common in this syndrome: loss of vision owing to progressive vascularization of the corneas; abnormalities of the hair, teeth, and nails; postnatal growth deficiency; and neuromuscular defects and recurrent skin infections. The patient in Pincus's case report is now 8 years old and has had hyperkeratotic and erythematous skin from birth. A skin biopsy specimen showed
papillomatosis, hyperkeratosis, irregular hypergranulosis, focal parakeratosis, and areas of dermal histiocytic
infiltration in the presence of normal skin appendages. Hearing was thought to be deficient at the age of 10 months when recur¬ rent otitis media and externa were noted. At age 3, pronounced sensorineural hearing loss was demonstrated; the patient's best ear had some sensi¬ tivity to a live voice at 90 dB. Bilateral interstitial corneal opaci¬ ties were apparent at 10 months of age. Recurrent bacterial conjunctivi¬ tis and progressive vascularization of the corneas, even after corneal trans¬ plantation, have resulted in near blindness. Hair was present at birth, but scar¬ ring alopecia of the scalp, along with loss of eyebrows and eyelashes, have followed recurrent skin infections. Primary teeth developed normally, while slow root development delayed adult dentition. Subsequent gingival infections have necessitated the ex¬ traction of ten teeth.
Downloaded From: http://archderm.jamanetwork.com/ by a University of Michigan User on 05/18/2015
