Confluent and reticulated papillomatosis:A clicical and histopathological, and electron microscopic study / 대한피부과학회지

ABSTRACT

BACKGROUND: Confluent and reticulated papillomatosis(CRP) is a rare but clinically distinct dermatosis of which the etiology is unknown. The pathomechanisms have been discussed from four different viewpoints 1) endocrine imbalance 2) abnormal host reaction to colonization by pityrosporum orbiculare 3) disorder of keratinization 4) variant of amyloidosis cutis. However recent reports have shown that defect in the keratinization has become the predominant pathomechanism of CRP. OBJECTIVE: Our purpose was to evaluate 25 cases of CRP clinically, histopathologically and electornimcroscopically, to reviewed the literature, and to commented on the pathogenesis. MEHTODS The 25 patients were examined clinically and pathologic specimens were obtained from lesions for routine light microdcopy and electron microscopy. RESULTS: 1. There were difference between male(68%) ad female(32%) patients. 2. Age distribution, at first visit were variable, ranging from 13 to 35, with the mean age of 20 years-old.3. Duration of symptoms were variable, ranging from 1 month to 5 years with wean duration of 1.5 years. 4. In the seasonal influence 16 cases showed aggrevation of lesions in the summer. 5. The distribution of lesions were abdomen(18 cases), chest(16 cases), back(12 cases), shoulder(7 cases), and buttock(5 were) 6. Three patients had a concomitant disease such as Graves' disease(1 case) or iron-deficiency anemia(2 cases). 7. There were no familial occurrence in any cases. 8. There were no hyphaes or sppores in KOH mount in any cases. 9. The histopathological finding of the affected tissues were characterized as follow All cases showed laminated hyperkeratosis, epidermal atrophy, acanthosis, and papillomatosis. 23 cases showed an increase of melanin pigment in the basal layer, minimal perivascular and/or perifollicular infiltration. 20 cases showed a decrease of the granular layer. 16 cases showed mild to moderate papillary edema, and dilated or congested upper dermal vessels. 10. In an electron microscopic studies the transitional cells between the stratum corneum and stratum granulosum contained less electron-dense tonofilament-keratohyaline aggregates with electron-lucent inclusions in their cytoplasm in a thick marginal band and degraded cell organelles. These cell layers were increased to 3 or 4 layers. The sratum corneum was thichened and showed a regular keratin pattern with intracellular and extracellular vacuoles. 11. 8 of the 10 patients treated with oral etretinate for 2 months showed some improvement. 7 of the 12 patients treated with topical scalicylic acid oint and selenium sulfide solution for 2 months showed some improvement. 1 patients with oral minocyclin for 2 months showed a marked improvement. 2 patients treated with oral ketoconazole did not show any improve-showed a marked improvement. CONCLUSION: In an electron microscopic study we found that transitional cell layers between stratum corneum and stratum granulosum were increased to 3 or 4 layers. Therefore we think that CRP may be a disease of defect in the keratinization. Although this abnormal keratinization is distinctive, it is uncertain whether it is primary or secondary.