456
B r i e f clinical and laboratory observations
REFERENCES 1. Cheek .lA Jr, and Staub GF: Nasojejunal alimentation for premature and full-term newborn infants, J PEDIATR 82:955, 1973.
Hypothyroidism and pityriasis rubra pilaris Response to thyroid hormone Waiter W. Tunnessen, Jr., M.D.,* Phillip I. Nieburg, M.D., and Mary L. Voorhess, M.D., Syracuse, N. Y.
~ITYRIASIS RUBRA PILARIS is an u n c o m m o n skin disorder affecting both adults and children. Although the condition has received some attention in the dermatologic literature, we could find only one brief report of three cases in pediatric journals in the last ten years? The purpose of this report is to (1) acquaint pediatricians with this uncommon, but not rare entity, and (2) to report a case in a 4-year-old girl with unrecognized hypothyroid-
The Journal of Pediatrics March 1976
2. Rhea JW, Ahmad MS, and Mange MS: Nasojejunal (transpyloric) feeding: A commentary, J PEDIATR86:451, 1975. 3. Ziegler EE, and Fomon SJ: Fluid intake, renal solute load, and water balance in infancy, J P~DIATR78:561, 1971.
of the trunk was covered with a less erythematous but more confluent rough dry rash. The scalp was covered with thick yellowish scales which were not greasy. The rash was mildly pruritic. There was no family history of similar rash. Hypothyroidism and PRP were suspected. Pertinent laboratory data included: Hgb 11.7 gm/~tl; mean corpusculai" volume 92c; T, radioimmunoassay less than 1.0 /Lg/dl (N 4.8-11.4 ,ug/dl); thyroid stimulating hormone 120 IU/ml (N less than 10 IU); serum vitamin A 43 IU/ml (N 65-275 IU/ml); serum carotene 54 mg/dl (N 50-300); cholesterol 250 mg/dl (N 120-240). The bone age was 42 months at a chronologic age of 54 months. A skin biopsy from the periumbilical area showed hyperkeratosis around follicular orifices, spotty parakeratosis, slight acanthosis, epidermal spongiosis, and a slight inflammatory infiltration in the dermis, all consistent with PRP. Four months after thyroid replacement therapy was begun the rash had cleared completely except for minor residual scaling of the scalp (Fig. 2). Physical activity increased, bowel movements became normal, and the abdomen less protuberant. Serum concentration of T~ was 6.9/~g/dl and serum vitamin A 208 IU/ ml at that time.
ism. CASE R E P O R T A 4 mA-year-oldgirl was referred for evaluation of a rash, short stature, delayed developmental milestones, and anemia. Linear growth had been constant along the third percentile until age 18 months, after which growth velocity began to decelerate to the present measurement 9 cm below the third percentile for age. Symptoms included chronic constipation and a normocytic, normochromic anemia. Small areas of desquamation began on the soles of the feet 2 % months prior to referral, followed by involvement of the palms and a spread to the extremities and trunk. The thickening of the palms and soles became fissured and painful. A diagnosis of psoriasis was entertained, prompting referral for evaluation and skin biopsy. Coarse facial features were striking. The abdomen was protuberant with a 2 cm umbilical hernia. There was no edema. Involvement of the skin was marked and widespread (Fig. 1). The thickening, erythema, and fissuring of the palms and soles were most impressive. The dorsum of the hands and feet had scattered papular lesions covered with whitish scales which cofild easily be removed without producing a bleeding point. Papular iesions extended up both extremities becoming confluent in some areas. Normal appearing islands of skin were interspersed. Two-thirds From the Department of Pediatrics, Upstate Medical Center. *Reprint address: 750 E. Adams St., Syracuse, N. Y. 13210.
I
Abbreviation used PRP: pityriasis rubra pilaris
DISCUSSION Pityriasis rubra pilaris is generally misdiagnosed as either psoriasis or seborrheic dermatitis. The lesions of PRP are papular and demonstrate keratotic follicular plugging. They may become confluent, especially over the trunk, and are dry and rough to the touch, described as a nutmeg-grater feel. The involved areas vary from flesh colored to rosy yellow, pink, or red. Erythematous plaques may be found most often on the dorsum of the phalanges, wrists, forearms, and anterior axillary folds. Islands of normal skin within the plaques are characteristic. Frequently a fine desquamation of the skin o f the trunk may occur. Yellowish scaling of the scalp and face may mimic seborrhea; however, the greasy feel is lacking. The hyperkeratosis of the palms and soles with intense erythema sharply demarcated from the dorsal surfaces is one of the diagnostic features. Nail pitting and striations are common. Pruritis may occur in 10% to 20% of patients. The etiology of PRP remains unknown. 2 ~ Generally PRP has been divided into two types: (1) a familial form with onset in childhood which often seems to be trans-
Volume 88 Number 3
Brief clinical and laboratory observations
457
Fig. 1. Full body, A, and feet, B, before treatment.
Fig. 2. Full body, A, and feet, B, after treatment. mitted as an autosomal dominant and (2) an acquired form which is more insiduous in onset and occurs mainly in adults. A variety Of conditions ranging from thermal injuries to infection were considered significant in precipitating PRP in 14 of 173 patients reviewed by Gross and associates. 3 An isolated instance of hypothyroidism was
mentioned in this report without further discussion. The diagnosis of PRP is mainly a clinical one. Histologic examination of a skin biopsy may be helpful in solidifying the diagnosis. No other laboratory examinations have been found to be consistently abnormal or helpful. Various therapeutic regimens have been tried to
458
Brief clinical and laboratory observations
control PRP including large doses of vitamin A (50,000 to 200,000 IU/day) because of a histologic similarity to vitamin A deficiency. Remissions have been reported with vitamin A administered either Orally or topically? ~ Thyroid hormone is necessary to convert ingested carotenes to vitamin A, and in hypothyroidism this conversion is nearly completely blocked? Only small amounts of untransformed carotenoids are absorbed in man? Almost paradoxically, however, the occurrence of carotenemia in hypothyroidism has long been known. O n e explanation is that this is related to increases in concentrations of serum beta-lipoproteins found in hypothyroidism.7 It is also known that hypercarotenemia is found only in patients with hypothyroidism who ingest food containing carotene? We did not measure lipoproteins in our patient, but in retrospect found that she did not ingest vegetables in any form. The dietary lack of carotene may explain the low normal value of serum carotene in our patient. The relationship between thyroid hormone and carotene conversion to vitamin A noted above was instrumental in our decision to withhold high-dose vitamin A therapy while replacing thyroid hormone. Thyroid hormone replacement resulted in normalization of the serum vitamin A level. Coincident with this was a dramatic clearing of the patient's rash. Despite the fact that most reported cases of PRP have not had low serum vitamin A levels, many of the patients have responded to high dose vitamin A therapy. Underwood and associates 9 have reported that plasma levels of vitamin A do not
Overwhelming sepsis following splenectomy for trauma John R. Balfanz, M.D.,* Mark E. Neshit, Jr., M.D., C. Jarvis, M.D., and William
The Journal of Pediatrics March 1976
necessarily reflect the adequacy of liver stores of, the vitamin. This experience in addition to our patient suggests that vitamin A metabolism, including absorption and carotene conversion, should be evaluated in patients with PRP. The authors thank Dr. James J. Leyden, Department of Dermatology, Hospital of the University of Pennsylvania, for reviewing the photographs of the rash and the histologic specimens of this patient.
REFERENCES 1. Huntly CC: Pityriasisrubra pilaris; Am J Dis Child 122:22, 1971. 2. Lamar LM, and Gaethe G: Pityriasis rubra pilaris, Arch Dermatol 89:515, 1964. 3. GrossDA, Landau JW, and Newcomer VD: Pityriasis rubra pilaris, Arch Dermatol 99:710, 1969. 4. Davidson CL, Winkelmann RK, and Kierland RR: Pityriasis rubra pilaris: A follow-up study of 57 patients, Arch Dermatol 100:175, 1969. 5. Jennings IW: Vitamins in endocrine metabolism, London, 1970, William Heinemann Medical Boods, Ltd, p 41. 6. Morton RA, editor: Fat soluble vitamins, Oxford, 1970, Pergamon Press, Inc, p 224. 7, Kimberg DV: Hypothyroidism: Gastrointestinal tract, in Werner SC and Ingbar SH, editors: The thyroid, ed 3, New York, 1971, Harper & Row, Publishers, p 739. 8. Josephs HW: The carotenemia of hypothyroidism, J PEDIATR41:784, 1952. 9. Underwood BA, Siegel H, Weisall RC, and Dolinski M: Liver stores of vitamin A in a normal population dying suddenly or rapidly from unnatural causes in New York City, Am J. Clin Nutr 23:1037, 1970,
THE ENTITY of overwhelming postsplenectomy infection is characterized by abrupt onset, unusual exuberance of bacterial growth, high incidence of disseminated intravascular coagulation, death occurring within hours of onset, and a mortality rate as high as 80%.1 Diplococcus Abbreviation used OPSI: overwhelming postsplenectomy infection
Krlvit, M.D., Ph.D., Minneapolis, Minn.
From the Department of Pediatrics, University of Minnesota Medical School. Supported by grantsfrom the United States Public Health Service, Nos. CA07306, CAl1996, CA08832, and the Minnesota Medical Foundation. *Reprint address: Box 284, Mayo Hospital, Universityof Minnesota, Minneapolis,Minn. 55455.
pneumoniae has been isolated in the majority of instances, but other encapsulated organisms (i.e., Haemophilus influenzae, Neisseria meningitidis, and Escherichia coli) have been reported. The classical OPSI syndrome occurs in young patients ( < 4 years) within two to three years of splenectomy, and has been thought to occur only when splenectomy was done for an underlying hematologic or malignant condition. Failure tO observe the OPSI syndrome following splen-
B r i e f clinical and laboratory observations
REFERENCES 1. Cheek .lA Jr, and Staub GF: Nasojejunal alimentation for premature and full-term newborn infants, J PEDIATR 82:955, 1973.
Hypothyroidism and pityriasis rubra pilaris Response to thyroid hormone Waiter W. Tunnessen, Jr., M.D.,* Phillip I. Nieburg, M.D., and Mary L. Voorhess, M.D., Syracuse, N. Y.
~ITYRIASIS RUBRA PILARIS is an u n c o m m o n skin disorder affecting both adults and children. Although the condition has received some attention in the dermatologic literature, we could find only one brief report of three cases in pediatric journals in the last ten years? The purpose of this report is to (1) acquaint pediatricians with this uncommon, but not rare entity, and (2) to report a case in a 4-year-old girl with unrecognized hypothyroid-
The Journal of Pediatrics March 1976
2. Rhea JW, Ahmad MS, and Mange MS: Nasojejunal (transpyloric) feeding: A commentary, J PEDIATR86:451, 1975. 3. Ziegler EE, and Fomon SJ: Fluid intake, renal solute load, and water balance in infancy, J P~DIATR78:561, 1971.
of the trunk was covered with a less erythematous but more confluent rough dry rash. The scalp was covered with thick yellowish scales which were not greasy. The rash was mildly pruritic. There was no family history of similar rash. Hypothyroidism and PRP were suspected. Pertinent laboratory data included: Hgb 11.7 gm/~tl; mean corpusculai" volume 92c; T, radioimmunoassay less than 1.0 /Lg/dl (N 4.8-11.4 ,ug/dl); thyroid stimulating hormone 120 IU/ml (N less than 10 IU); serum vitamin A 43 IU/ml (N 65-275 IU/ml); serum carotene 54 mg/dl (N 50-300); cholesterol 250 mg/dl (N 120-240). The bone age was 42 months at a chronologic age of 54 months. A skin biopsy from the periumbilical area showed hyperkeratosis around follicular orifices, spotty parakeratosis, slight acanthosis, epidermal spongiosis, and a slight inflammatory infiltration in the dermis, all consistent with PRP. Four months after thyroid replacement therapy was begun the rash had cleared completely except for minor residual scaling of the scalp (Fig. 2). Physical activity increased, bowel movements became normal, and the abdomen less protuberant. Serum concentration of T~ was 6.9/~g/dl and serum vitamin A 208 IU/ ml at that time.
ism. CASE R E P O R T A 4 mA-year-oldgirl was referred for evaluation of a rash, short stature, delayed developmental milestones, and anemia. Linear growth had been constant along the third percentile until age 18 months, after which growth velocity began to decelerate to the present measurement 9 cm below the third percentile for age. Symptoms included chronic constipation and a normocytic, normochromic anemia. Small areas of desquamation began on the soles of the feet 2 % months prior to referral, followed by involvement of the palms and a spread to the extremities and trunk. The thickening of the palms and soles became fissured and painful. A diagnosis of psoriasis was entertained, prompting referral for evaluation and skin biopsy. Coarse facial features were striking. The abdomen was protuberant with a 2 cm umbilical hernia. There was no edema. Involvement of the skin was marked and widespread (Fig. 1). The thickening, erythema, and fissuring of the palms and soles were most impressive. The dorsum of the hands and feet had scattered papular lesions covered with whitish scales which cofild easily be removed without producing a bleeding point. Papular iesions extended up both extremities becoming confluent in some areas. Normal appearing islands of skin were interspersed. Two-thirds From the Department of Pediatrics, Upstate Medical Center. *Reprint address: 750 E. Adams St., Syracuse, N. Y. 13210.
I
Abbreviation used PRP: pityriasis rubra pilaris
DISCUSSION Pityriasis rubra pilaris is generally misdiagnosed as either psoriasis or seborrheic dermatitis. The lesions of PRP are papular and demonstrate keratotic follicular plugging. They may become confluent, especially over the trunk, and are dry and rough to the touch, described as a nutmeg-grater feel. The involved areas vary from flesh colored to rosy yellow, pink, or red. Erythematous plaques may be found most often on the dorsum of the phalanges, wrists, forearms, and anterior axillary folds. Islands of normal skin within the plaques are characteristic. Frequently a fine desquamation of the skin o f the trunk may occur. Yellowish scaling of the scalp and face may mimic seborrhea; however, the greasy feel is lacking. The hyperkeratosis of the palms and soles with intense erythema sharply demarcated from the dorsal surfaces is one of the diagnostic features. Nail pitting and striations are common. Pruritis may occur in 10% to 20% of patients. The etiology of PRP remains unknown. 2 ~ Generally PRP has been divided into two types: (1) a familial form with onset in childhood which often seems to be trans-
Volume 88 Number 3
Brief clinical and laboratory observations
457
Fig. 1. Full body, A, and feet, B, before treatment.
Fig. 2. Full body, A, and feet, B, after treatment. mitted as an autosomal dominant and (2) an acquired form which is more insiduous in onset and occurs mainly in adults. A variety Of conditions ranging from thermal injuries to infection were considered significant in precipitating PRP in 14 of 173 patients reviewed by Gross and associates. 3 An isolated instance of hypothyroidism was
mentioned in this report without further discussion. The diagnosis of PRP is mainly a clinical one. Histologic examination of a skin biopsy may be helpful in solidifying the diagnosis. No other laboratory examinations have been found to be consistently abnormal or helpful. Various therapeutic regimens have been tried to
458
Brief clinical and laboratory observations
control PRP including large doses of vitamin A (50,000 to 200,000 IU/day) because of a histologic similarity to vitamin A deficiency. Remissions have been reported with vitamin A administered either Orally or topically? ~ Thyroid hormone is necessary to convert ingested carotenes to vitamin A, and in hypothyroidism this conversion is nearly completely blocked? Only small amounts of untransformed carotenoids are absorbed in man? Almost paradoxically, however, the occurrence of carotenemia in hypothyroidism has long been known. O n e explanation is that this is related to increases in concentrations of serum beta-lipoproteins found in hypothyroidism.7 It is also known that hypercarotenemia is found only in patients with hypothyroidism who ingest food containing carotene? We did not measure lipoproteins in our patient, but in retrospect found that she did not ingest vegetables in any form. The dietary lack of carotene may explain the low normal value of serum carotene in our patient. The relationship between thyroid hormone and carotene conversion to vitamin A noted above was instrumental in our decision to withhold high-dose vitamin A therapy while replacing thyroid hormone. Thyroid hormone replacement resulted in normalization of the serum vitamin A level. Coincident with this was a dramatic clearing of the patient's rash. Despite the fact that most reported cases of PRP have not had low serum vitamin A levels, many of the patients have responded to high dose vitamin A therapy. Underwood and associates 9 have reported that plasma levels of vitamin A do not
Overwhelming sepsis following splenectomy for trauma John R. Balfanz, M.D.,* Mark E. Neshit, Jr., M.D., C. Jarvis, M.D., and William
The Journal of Pediatrics March 1976
necessarily reflect the adequacy of liver stores of, the vitamin. This experience in addition to our patient suggests that vitamin A metabolism, including absorption and carotene conversion, should be evaluated in patients with PRP. The authors thank Dr. James J. Leyden, Department of Dermatology, Hospital of the University of Pennsylvania, for reviewing the photographs of the rash and the histologic specimens of this patient.
REFERENCES 1. Huntly CC: Pityriasisrubra pilaris; Am J Dis Child 122:22, 1971. 2. Lamar LM, and Gaethe G: Pityriasis rubra pilaris, Arch Dermatol 89:515, 1964. 3. GrossDA, Landau JW, and Newcomer VD: Pityriasis rubra pilaris, Arch Dermatol 99:710, 1969. 4. Davidson CL, Winkelmann RK, and Kierland RR: Pityriasis rubra pilaris: A follow-up study of 57 patients, Arch Dermatol 100:175, 1969. 5. Jennings IW: Vitamins in endocrine metabolism, London, 1970, William Heinemann Medical Boods, Ltd, p 41. 6. Morton RA, editor: Fat soluble vitamins, Oxford, 1970, Pergamon Press, Inc, p 224. 7, Kimberg DV: Hypothyroidism: Gastrointestinal tract, in Werner SC and Ingbar SH, editors: The thyroid, ed 3, New York, 1971, Harper & Row, Publishers, p 739. 8. Josephs HW: The carotenemia of hypothyroidism, J PEDIATR41:784, 1952. 9. Underwood BA, Siegel H, Weisall RC, and Dolinski M: Liver stores of vitamin A in a normal population dying suddenly or rapidly from unnatural causes in New York City, Am J. Clin Nutr 23:1037, 1970,
THE ENTITY of overwhelming postsplenectomy infection is characterized by abrupt onset, unusual exuberance of bacterial growth, high incidence of disseminated intravascular coagulation, death occurring within hours of onset, and a mortality rate as high as 80%.1 Diplococcus Abbreviation used OPSI: overwhelming postsplenectomy infection
Krlvit, M.D., Ph.D., Minneapolis, Minn.
From the Department of Pediatrics, University of Minnesota Medical School. Supported by grantsfrom the United States Public Health Service, Nos. CA07306, CAl1996, CA08832, and the Minnesota Medical Foundation. *Reprint address: Box 284, Mayo Hospital, Universityof Minnesota, Minneapolis,Minn. 55455.
pneumoniae has been isolated in the majority of instances, but other encapsulated organisms (i.e., Haemophilus influenzae, Neisseria meningitidis, and Escherichia coli) have been reported. The classical OPSI syndrome occurs in young patients ( < 4 years) within two to three years of splenectomy, and has been thought to occur only when splenectomy was done for an underlying hematologic or malignant condition. Failure tO observe the OPSI syndrome following splen-
